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Scleroderma and related conditions
Scleroderma and related conditions
5.6
Tanya M Ball
What is “scleroderma”? Scleroderma is a generic term encompassing a spectrum of complex autoimmune conditions with similar, or overlapping clinical features. These can range from relatively localized disorders such as primary Raynaud’s phenomenon, to significantly more debilitating systemic diseases, including systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) (Denton & Black 2002; Assassi et al. 2007). This umbrella term encompasses SSc, SLE, mixed connective tissue disease (MCTD), Sjo¨gren’s disease (SjD), primary/secondary Raynaud’s phenomenon (PRP/ SRP), and rheumatoid arthritis (RA). Scleroderma denotes a condition presenting as thickening, hardening, and scarring of the skin. Systemic refers to an autoimmune condition that affects the internal organs as well as the skin and superficial (subcutaneous and “deep investing”) fascia layers. This chapter will focus on the systemic forms of the disease because of their wider significance for manual therapy (MT) intervention. Conditions classified under the “scleroderma umbrella” generally share the following characteristics: 1. All are chronic, inflammatory, autoimmune conditions. 2. Etiology is multifactorial, with potential genetic, environmental, physical, and emotional health predisposing factors, with no known cause. Excessive coincidence or accumulation of such factors in relation to the individual’s specific immune tolerance triggers a “mistaken identity” failure within this system. Healthy body cells are erroneously interpreted as foreign, Q 2012, Elsevier Ltd.
threatening entities to be destroyed by the creation of associated antibodies. Blood tests can identify disease-specific abnormal antibodies which predominantly target the skin and fasciae or connective tissues (CTs) (Denton & Black 2002). 3. Prevalence is eight times greater in women than men, with onset between the mid 20s and mid 50s. 4. While various interventions, including drugs, can help stabilize and manage these conditions, they have no known cure. Chronic inflammation of the affected tissues leads to debilitating consequences including severe pain, fatigue, further immune system overload, tissue and organ damage, loss of functional autonomy, and quality of life. Complications from systemic forms of scleroderma, usually associated with infection, critical cardiorespiratory or renal failure, can be fatal, though survival rates have risen significantly (Denton & Black 2002), with SLE survival rates improving from <50% at 5 years in the 1950s, to 85% at 10 years nowadays (Merrell & Shulman 1955; Urowitz et al. 1997; Bernatsky et al. 2006; Abu-Shakram 2008). 5. Pathological hardening, thickening, scarring of any tissue affects three essential systems of immediate and significant relevance to manual therapy (MT): The vascular system The immune system, including the lymphatic system; and The fascial system (Denton et al. 1996, Denton & Black 2002; Assassi et al. 2007; Abu-Shakram 2008).
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Fascia-Related Disorders
Clinical features of special relevance to MT The most significant consideration from a MT perspective is the impact of scleroderma on the CTs or fasciae. A sound understanding of how these are affected is critical to appropriate clinical reasoning, and an effective treatment and management plan. Martin’s (2009) case study of a diffuse systemic sclerosis (dSSc) patient summarizes the pathological processes involved, which are characterized by fibrotic changes associated with fibroblastic cell over-activity, leading to collagen overproduction within those tissues (Denton & Black 2002). Ensuing adverse tissue changes include thickening, shortening, hardening, and scarring, which in turn result in reduced range of motion (RoM), vascular, lymphatic, neural, joint, and visceral compression and constriction. Consequences include ischemic pain, with potential local tissue necrosis or infection, sensory, motor, and autonomic dysfunction, and further immune deregulation, thus completing the “downward spiral”(Adams et al. 2002; Denton & Black 2002). This intricately linked neurovascular–fascial pathology accounts for much of patients’ pain, functional impairment, and psychological distress (Findley & Schleip 2007; Martin 2009). It is important from a MT clinical perspective to appreciate the nature and process of damage to the varying fasciae of different structures and organs.
Features affecting the neurovascular and fascial systems Vasculitis Collagen overproduction within vascular walls (epi- and endothelium) leads to thickening, stiffening, narrowing of their internal diameter, and hence ischemia to the tissues and cells supplied. The very fine capillaries supplying oxygen at cellular level are the most susceptible. The most commonly involved tissues and organs include: the extremities – hands and feet, especially the fingers and/or toes, the gastrointestinal (GI) tract, lungs, heart, kidneys, as well as muscles and joints.
The myofascial system Fibrotic myofascial change can vary in severity both in terms of area(s) affected and degree of ensuing restriction and dysfunction. It is near-universal in 226
the systemic forms of the disease, and can result in unrelenting, debilitating myofascial pain. In the author’s experience, fibrotic shortening and restriction particularly occur in the flexor muscle groups. Increased secondary spinal curves at the lumbar and mid–upper cervical levels are common, forcing the head anteriorly in relation to the body’s center of gravity, with a posterior tilt (“chin poke”). Additional asymmetrical fascial changes occur both locally and over a distance, with postural and functional impact, compounded by pain, fatigue, and sometimes muscle weakness. The face, mouth, and jaw are commonly affected, which can lead to dental overcrowding, mouth opening restriction, and associated eating and other implications for daily life. Joint inflammation (particularly in SLE patients) presents as acute swelling, pain, and stiffness. The wrists, hands, fingers, or feet and toes can develop permanent deformities, usually associated with secondary Raynaud’s phenomenon and sclerodactyly.
Secondary Raynaud’s phenomenon (“Raynaud’s”) Most scleroderma sufferers experience abnormal, severe vasoconstriction to the small capillaries in their fingers and/or toes when exposed to cold, emotional stress, or immune system overload, for instance prolonged excessive working hours and inadequate sleep. Typically, the affected hands and particularly the fingers become chronically swollen, which further compromises vascular and lymphatic circulation. Fibrotic changes, coupled with collagen overproduction as described above, thicken and stiffen the capillary walls, thereby reducing or even occluding their inner diameter.
Sclerodactyly Aggressive fibrotic changes to the skin and subcutaneous fascia predominantly manifest in the hands, fingers, feet, and/or toes with swelling, tightening, hardening, and thinning of the skin, which takes on a waxy, shiny appearance. The tightness is frequently associated with shortening of underlying fasciae, resulting in claw-like finger deformities. Commonly, the wrist and metacarpophalangeal joints become locked in relative extension, while the proximal and distal interphalangeal joints (IPJ) become “locked” into mild to excessive flexion. The thumb saddle joint adducts, with IPJ hyperextension. When
Scleroderma and related conditions
distal limb sections are affected, patients exhibit semi-flexed elbows or knees at rest due to excessive contracture.
Pulmonary sclerosis/pulmonary hypertension (PHT) A thickening, hardening, and scarring of the alveolae and/or adjacent capillary epi-/endothelium can induce, through fibrosis, two related potentially life-threatening situations: (i) reduced oxygen uptake and carbon dioxide removal through the thicker, inelastic, fibrotic tissue layer, leading to excessive breathlessness; (ii) rise in pulmonary arterial pressure due to the same fibrotic narrowing or occlusion of the capillaries adjacent to the alveolae. Pleuritis inflammation of the pleura, the outer fascial lining of the lungs, can also occur, causing chest pain aggravated with coughing.
CHAPTER 5.6
bidirectional peristalsis. Poor peristalsis can lead to cyclical episodes of bacterial overgrowth; symptoms can include alternating bouts of constipation and diarrhea, nausea, sometimes with vomiting, abdominal bloating, swelling, severe pain, tenderness, and extreme exhaustion. 3. Lower GI tract extremity/anal canal: fibrotic damage to the neurovascular–fascial structures of the inner and/or outer anal sphincter(s) affects the sensory-motor supply to the pelvic floor musculature, inhibiting the normal closure reflex. This can cause loss of full bowel control and intermittent leaking or incontinence. GI tract involvement can have a particularly profound psychosocial impact on those affected and their families.
Other potential organ involvement Myo- and pericardiac damage More rarely, interstitial pulmonary fibrosis as described above can progress to severe lung damage, triggering an immunoinflammatory response in the heart. This presents as pericardial swelling, left ventricular (“pressure pump”) hypertrophy against reduced right ventricular (“volume pump”) input in response to pulmonary arterial hypertension, arrhythmia, and diastolic dysfunction. Fibrosis of the heart muscle can be a further serious risk factor (Stamenkovic 2008, Allanore 2010).
The same inflammatory/fibrotic process rarely affects the brain, liver, and/or kidneys. Reduced white blood cells (leukopenia) or a tendency to bruise or an increased risk of bleeding, due to a low platelet count (thrombocytopenia) may also occur. Peripheral neurological disturbance can present as tingling, loss of sensation, numbness, or weakness.
Types of scleroderma, and where SSc fits in
GI tract 1. Upper GI tract: the lower esophageal sphincter (LES) opens upon swallowing, to enable food to be propelled into the stomach, and closes in between swallowing to prevent reflux of stomach contents back into the esophageal tube in a coordinated way, and is an essential part of overall peristalsis (Gaumnitz & Fayyad 2009). Atrophy and fibrosis result in acid and general stomach contents reflux when reclining, bending forward, or even sitting after food or drink consumption. Second, it results in difficulty in swallowing, notably solid food, and particularly when time has elapsed since fluid intake and/or with low salivation. 2. Mid/lower GI tract: smooth muscle atrophy and fibrosis can result in distention of the intestinal diameter, leading to inefficient, sometimes
Scleroderma is categorized according to the extent of skin and organ involvement, which with childhood onset can lead to growth deformities.
Localized scleroderma (LSc) Morphea presents as: localized patches of thickened, sclerotic, usually discolored (darker or lighter) skin, most often oval or round in shape, which can appear anywhere on the body. These patches are normally painless but can be itchy. Linear scleroderma presents as: lines of hardened, sclerotic skin that can appear on the head, arms, or legs, and can involve the subcutaneous (areolar) fascia as well as underlying myofascia and even bone. 227
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Fascia-Related Disorders
Systemic sclerosis (SSc) Limited cutaneous systemic sclerosis presents with: calcinosis; Raynaud’s phenomenon; esophageal dysfunction; sclerodactyly; and telangiectasia (Denton & Black 2002), represented by the acronym CREST. Diffuse cutaneous systemic sclerosis: has the highest incidence of fascial and organ pathologies, and higher morbidity and mortality rates (Denton & Black 2002; Stamenkovic 2006); with renal failure a common complication.
Mixed connective tissue disease (MCTD) These patients have a mixture of local and diffuse tissue involvement.
Conventional medical management Reduction in scleroderma-related mortality has led to a priority for morbidity management strategies (Merrell & Shulman 1955; Urowitz et al. 1997; Denton & Black 2002; Bernatsky et al. 2006; AbuShakram 2008). Disease stabilization and patient management remain a challenging order, often with limited to moderate success. Patient education in what treatments and self-management options are available is an essential component, as is the careful correlation between intervention selection and disease subset and stage (Denton & Black 2002). Recent conventional medical prognostic developments include:
• Scleroderma “hallmark” antibody screening to identify patients at increased risk of complications. • Preventative patient screening for pulmonary hypertension. Conventional medical treatment and management aimed at stabilizing or even modifying the disease process broadly comprises three categories: • Strategies targeting the vascular system, and notably Raynaud’s symptoms. These include oral and intravenous (prostacyclin/iloprost) vasodilator and anticoagulant medication aimed at dilating capillary vessels and inhibiting clotting, with the view to maximizing blood flow through 228
the finest vessels in the extremities and/or organs. Other conventional interventions for hypertension are also used with variable success to control pulmonary HT (Wigley et al. 1992; Denton & Black 2002). • Immunosuppressive drugs, aimed at inhibiting autodestructive immune responses, have shown limited results for DCSSc patients, but remain largely ineffective for LCSSc sufferers. • Antifibrotic therapy, in the form of antagonist drugs aimed at inhibiting fibroblast proliferation and extracellular matrix synthesis, is summarized by Denton & Black (2002). In summary, while advances in medicine have reduced mortality and enhanced disease stabilization and patient (self-)management, these improvements remain limited, and elicit inconsistent responses from patient to patient, with frequent adverse medication side effects. This calls for the exploration of nonpharmaceutically based treatments to control, or even reverse symptoms, and notably tissue fibrosis.
Can MT help reduce or reverse scleroderma-related fibrotic changes? Clinical evidence to date Fascial release (FRT) and structural integration (SI) Walton (2007) reports briefer Raynaud’s attack episodes following a series of FRT treatments. Martin (2009) reports significant gains in chest expansion, temporomandibular (TMJ) mobility, and associated increase in mouth opening, marked wrist and finger joint RoM gains (of up to 100%), reduced digital ulcerations and overall Raynaud’s symptoms, recovery of nail growth, and most importantly perhaps, reduced or eliminated pain in all affected areas. Benefits were said gradually to decrease with prolonged periods of nonintervention, but were readily restored with intermittent treatment resumption. Increased parasympathetic activity, regulation of associated autonomic functions, and other related beneficial changes following manual therapy interventions including FRT have been recently reviewed by Moyer et al. (2004). The author’s first-hand clinical experience with some 20þ SSc patients in the UK and Europe shows
Scleroderma and related conditions
encouraging treatment effects of manual therapies, in conjunction with ongoing medical review and management, including via appropriate pharmaceutical drugs (Ball 2010), including enhancement of fascial “texture“, glide, and remodeling.
• Decreasing: pain; use of pain medication; episodes of exhaustion; duration and frequency of GI tract malfunction; hand and finger swelling, stiffness, tenderness. • Enhancing: functional mobility and RoM; hand and finger grip; mouth opening; functional autonomy; positive emotional state; and quality of life.
Rationale for potential MT efficacy FRT techniques, the SI process, and the KMIt model Through FRT manual manipulation of the connective tissues coupled with movement re-education, SI aims to restore length, symmetry, and balance to the body about its vertical skeletal axis in all planes (Myers 2009). In SI terminology, the aim is to restore optimal “tensegrity”, integrity of equal tension in all planes between the fascial tissues “pulling”, and hence applying compressive forces on, the skeletal structure. The Kinesis Myofascial Integration (KMI)t SI model developed by Thomas Myers (2009), which formed the basis of the author’s therapeutic approach, rests on “reading and treating the cohesive myofascial continuities of the Anatomy Trains”. The “Anatomy Trains” are a system of 12 key myofascial continuities, “myofascial meridians”, that drape the body from either head or neck to foot or toe, from trunk to hand or finger, along a variety of relatively straight, spiral, curvy, or “basket-weave” alignments (Myers 1997, 2009). The process therefore involves accurate “body reading” to identify areas of shortened, congested, CT, compensated by overstretched, lengthened fascia elsewhere. “Interactive” manual manipulative techniques usually involving active client movement and participation are then methodically applied according to a structured treatment strategy based on associated clinical reasoning.
Potential mechanism of effect SI/KMIt-attributed fascial changes among the general population, and specific clinical cases including scoliosis, have been well documented (Chaitow & DeLany 2003; LeBauer et al. 2008; Myers 2009).
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Similar interventions with scleroderma patients, possibly with greater repetition or frequency, using softening, releasing, spreading, and lengthening techniques, may generate tissue remodeling, over time leading to reversing fibrotic CT and skin changes (De Lany et al. 2002; Schleip 2003). These immediate and longer-term changes would in turn:
• Enhance joint RoM and overall mobility. • Relieve vascular, lymphatic, neural, joint, and visceral compression and constriction. Leading to: • Improved vascular and lymphatic circulation, and hence oxygen and nutrition supply to chronically ischemic, inflamed, or damaged tissues. • Removal of congested interstitial fluid, metabolic waste, etc. • Decreased pain and stiffness. • Enhanced neural conduction sensory, motor, and autonomic. • Enhanced visceral “position” and function. In turn, decreased vascular, neural, and visceral compression would lead to reduced ischemic pain, raised pain threshold due to nociceptor nerve ending decompression, more effective respiratory and GI tract function. The overall outcome from the above could reasonably be assumed to include: • Reduced fatigue and anxiety. • Enhanced tissue healing, functional mobility, autonomy, quality of life, emotional state, autonomic and immune function (Chaitow & DeLany 2000, 2003; Evans et al. 2000; Adams et al. 2002; Findley & Schleip 2007; Huijing et al. 2009; Martin 2009).
Scientific basis: potential therapeutic mechanisms Fascial changes such as softening, increased pliability, flexibility, and mobility These suggest a process of fascial tissue remodeling involving structural reorganization favoring greater flexibility, as proposed by Martin (2009). Pioneering research by Schleip (2003) and Wipff & Hinz (2009) 229
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Fascia-Related Disorders
on myofibroblast development, and which key factors determine whether or not they maintain their “contractile phenotype” on maturation, offers appealing, and in the author’s view promising supporting evidence regarding the possible mechanisms involved: according to the study, mechanical stress such as FRT therapy is one of two principal factors, the other being the ability to utilize transforming growth factor-beta (TGF-b1).
Restored independent sliding, gliding, and coordination between adjacent myofascial structures and associated enhanced areolar fascia “fluidity” (Fourie 2008) This may likewise be attributed in part to the above process. Myers (2009) proposes the vivid analogy of “rolling” a grapefruit, thereby gently breaking up the various membrane layers separating the juice cells to facilitate the subsequent task of juicing, and the effect of FRT input on the areolar fasciae, intermuscular septae, and epimysia.
Decreased myofascial pain This may reasonably be attributable to multiple factors. LeMoon (2008) proposes a convincing “fasciagenic” pain model where prolonged, unremitting fascial thickening and stiffening is deemed responsible for generating myofascial pain symptoms. Conversely, fascial relaxation interventions are proposed as a key strategy towards alleviating it.
Findings arising from the 2009 2nd International Fascia Congress Building on earlier studies inter alia by Rijkelijkhuizen et al. (2005) and Schleip ( Findley & Schleip 2007; Van der Wal 2009a, b) demonstrated how ligament tissue fulfills key functional roles in any and every joint position, because of the “in series” as opposed to “in parallel” organization of muscles, aponeuroses, ligaments, and periosteum, in an ever-continuous, uninterrupted body-wide pattern. The fresh cadaver dissections and in-vivo video clips testified to how the omnipresent interconnectivity of the fascial matrix ensured a trans-muscular tensile force transmission in series, involving muscle–aponeurosis–ligament 230
tissue–periosteum, with an unbroken continuum both around a given joint, and from one joint to the next throughout the body. This offers compelling anatomical and physiological evidence as to how FRT-type therapeutic intervention at a given fascial site and layer might impact on deeper, manually unreachable tissue layers. These could be intrathoracic or abdominal myofascial and visceral structures and organs, with ensuing enhanced respiratory, visceral, and autonomic regulation.
Psychoneuroimmunology (PNI) In light of the acknowledged correlation between scleroderma-type disease and sympathetic over-activity (Denton et al. 1996), it would seem reasonable to speculate that enhancing physical and psychological well-being in patients via SI intervention, including stimulating parasympathetic activity, might help regulate autoimmune response. Strategies could include placing greater focus on “end game” attention to the cervical and sacral areas, as well as consciously and sensitively addressing sympathetic responsive sites such as the thorax, shoulders, and abdomen.
Neuromuscular technique (NMT) and muscle energy technique (MET) Evidence of NMT and MET efficacy in addressing chronic musculoskeletal pain and dysfunction is robustly supported by Chaitow (2003) and Chaitow & DeLany (2000, 2003): not only do these approaches take into account both local and distant potential sources of pain and dysfunction, but they seek to identify and address self-perpetuating factors such as myofascial trigger points (MTPs), areas of “locked short” versus “locked long” tissues reflecting loss of tensegrity, detrimental posture, and hence movement patterns. That NMT aims specifically to alter the ground substance of connective tissue, to deactivate myofascial trigger points, and reduce ischemia, and to assess and beneficially modify postural alignment, musculoskeletal dysfunction, and neural entrapment Chaitow & DeLany 2003.
offers plausible rationale for efficacy in reversing scleroderma-related fascial fibrotic change. By the same token, the essence of the neurophysiological process underpinning MET techniques such as postisometric relaxation (PIR) and reciprocal
Scleroderma and related conditions
inhibition (RI) is inhibition, in the sense of enhanced relaxation enabling shortened myofascia to be restored to a more ‘normal’ resting length. Its clinical efficacy in musculoskeletal therapy is well documented (Chaitow & DeLany 2003); Mitchell & Mitchell (1995, 1998, 1999) once again provide reasonable grounds for anticipating beneficial outcomes in the context of scleroderma.
Manual lymphatic drainage (MLD) The efficacy of MLD in reducing acute and chronic edema and excessive interstitial fluid is likewise well documented (Chaitow & DeLany 2003; Willis 2004; Fourie 2009). Its acceptance in the UK as a useful modality to address lymphedema, including notably during pregnancy, following breast cancer
CHAPTER 5.6
surgery/reconstructive surgery, is gaining momentum, with encouraging early evidence of efficacy for rheumatoid arthritis and scleroderma (www. bmlda.org.co.uk).
Conclusion The current inadequacy of conventional medicine in alleviating, or reversing, scleroderma-related fascial change, anecdotal indications of MT efficacy in this respect, and the growing evidence-base supporting MT therapeutic mechanisms, jointly offer an exciting and wide-ranging opportunity for much-needed ‘clinical’ and ‘laboratory’ research in the field over the coming years.
References Abu-Shakram, 2008. Do improved survival rates of patients with systemic lupus erythematosus reflect a global trend? J. Rheumatol. 35, 1906–1908. Adams, M., Burton, K., Bogduk, N., et al., 2002. The biomechanics of back pain. Churchill Livingstone, Edinburgh. Allanore, Y., Meune, C., Vonk, M.C., et al., 2010. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. Ann Rheum Dis. 69 (1), 218–221. Assassi, S., Mayes, M.D., Gourh, P., et al., 2007. SSc and SLE have distinct gene expression profiles in the peripheral blood cells. ACR ASM Presentation. Ball, T.M., 2011. Structural Integrationbased fascial release efficacy in alleviating specific symptoms in systemic lupus erythematosus (SLE): Two case studies. J. Bodyw. Mov. Ther. 15 (2), 217–225. Bernatsky, S., Boivin, J.F., Joseph, L., et al., 2006. Mortality in systemic lupus erythematosus. Arthritis Rheum. 54, 2550–2557. Chaitow, L., 2003. Muscle energy techniques, third ed. Churchill Livingstone/Elsevier, Edinburgh. Chaitow, L., DeLany, J.W., 2000. Clinical application of neuromuscular
techniques, vol. I. Harcourt Publishers/Churchill Livingstone, London. Chaitow, L., DeLany, J., 2003. Neuromuscular techniques in orthopaedics. Techniques in Orthopaedics 18 (1), 74–86. DeLany, J., 2002. Connective tissue perspectives. J. Bodyw. Mov. Ther. 6 (4), 220–227. Denton, C.P., Black, C.M., Korn, J.H., et al., 1996. Systemic sclerosis: current pathogenetic concepts and future prospects for targeted therapy. Lancet 347 (9013), 1453–1458. Denton, C.P., Black, C.M., 2002. Systemic sclerosis and related disorders. Oxford Textbook of Medicine, 4th Ed. Evans, P., Hucklebridge, F., Clow, A., 2000. Mind, immunity, and health. Free Association Books, London. Findley, T.W., Schleip, R. (Eds.), 2007. Fascia research. Elsevier, Munich. Fourie, W.J., 2008. Fascia lata: merely a thigh stocking, or a coordinator of complex thigh muscular activity? J. Bodyw. Mov. Ther. 12 (3), 265–273. Fourie, W.J., 2009. Fasciae in recovery from cancer surgery. 2nd International Fascia Congress presentation, Amsterdam. Gaumnitz, E.A., Fayyad, A., 2009. Oesophageal motility disorders. Medscape. Accessed Oct 4th, 2011, from: http://emedicine.
medscape.com/article/174783overview. Huijing, P.A., Hollander, P., Findley, T.W., et al., (Eds.), 2009. Fascia research II. Elsevier, Munich. LeBauer, A., Brtalik, R., Stowe, K., 2008. The effect of MFR on an adult with idiopathic scoliosis. J. Bodyw. Mov. Ther. 12 (4), 356–363. LeMoon, K., 2008. Clinical Reasoning in Massage Therapy. International Journal of Therapeutic Bodywork and Massage 1 (1). (http://www.ijtmb.org/ index.php/ijtmb/article/view/2/20). Martin, M.M., 2009. Effects of myofascial release in diffuse systemic sclerosis. J. Bodyw. Mov. Ther. 13 (4), 320–327. Merrell, M., Shulman, L.E., 1955. Determination of prognosis in chronic disease, illustrated by SLE. J. Chron. Dis. 1, 12–32. Mitchell, F.L., Mitchell, P.K.G., 1995. The muscle energy manual Vol. 1: Concepts and mechanisms the musculoskeletal screen cervical region evaluation & treatment. MET Press, East Lansing, MI. Mitchell, F.L., Mitchell, P.K.G., 1998. The muscle energy manual Vol. 2: Evaluation & treatment of the thoracic spine, lumbar spine & rib cage. MET Press, East Lansing, MI. Mitchell, F.L., Mitchell, P.K.G., 1999. The muscle energy manual Vol. 3: Evaluation & treatment of the pelvis and sacrum. MET Press, East Lansing, MI.
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Moyer, C.A., Rounds, J., Hannum, J.W., 2004. A meta-analysis of massage therapy research. Psychol. Bull. 130 (1), 3–18. Myers, T.W., 1997. The Anatomy Trains. J. Bodyw. Mov. Ther. 1 (2), 91–101. Myers, T.W., 2009. Anatomy Trains, second ed. Churchill Livingstone, Edinburgh. Rijkelijkhuizen, J.M., Baan, G.C., de Haan, A., et al., 2005. Extramuscular myofascial force transmission for in situ rat medial gastrocnemius and plantaris muscles in progressive stages of dissection. J. Exp. Biol. 208, 129–140. Schleip, R., 2003. Fascial plasticity: a new neurobiological explanation. J. Bodyw. Mov. Ther. 7 (1), 11–19 (Part 1) & 7 (2), 104–116 (Part 2). Stamenkovic, B.N., 2008. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort
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of patients with systemic sclerosis (SSc). Ann. Rheum. Dis. 67 (1), 31–36. Urowitz, M.B., Gladman, D.D., Abu-Shakra, M., Farewell, V.T., 1997. Mortality studies in systemic lupus erythematosus. Results from a single center. III. Improved survival over 24 years. J. Rheumatol. 24, 1061–1065. Van der Wal, J., 2009a. The architecture of the connective tissue in the musculoskeletal system: an often overlooked functional parameter as to proprioception in the locomotor apparatus. Int. J. Ther. Massage Bodyw. Res. Educ. Pract. 2 (4), 9–23. Van der Wal, J., 2009b. The architecture of connective tissue as a functional substrate for proprioception in the locomotor system. In: 2nd International Fascia Congress, Amsterdam.
Walton, A., 2007. Efficacy of MFR in the treatment of primary Raynaud’s phenomenon. J. Bodyw. Mov. Ther. 12 (3), 274–280. Wigley, F.M., Seibold, J.R., Wise, R.A., et al., 1992. Intravenous iloprost treatment of Raynaud’s phenomenon and ischemic ulcers secondary to systemic sclerosis. J. Rheumatol. 19 (9), 1407–1414. Willis, A., 2004. Manual Lymphatic Drainage and its Therapeutic Benefits. Positive Health Online issue 104, (http://www.positivehealth. com/article/manual-lymphaticdrainage/manual-lymphatic-drainageand-its-therapeutic-benefits). Wipff, J.P., Hinz, B., 2009. Myofibroblast mini-review: Myofibroblasts work best under stress. J. Bodyw. Mov. Ther. 13 (2), 121–127.
5.6
Tanya M Ball
What is “scleroderma”? Scleroderma is a generic term encompassing a spectrum of complex autoimmune conditions with similar, or overlapping clinical features. These can range from relatively localized disorders such as primary Raynaud’s phenomenon, to significantly more debilitating systemic diseases, including systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) (Denton & Black 2002; Assassi et al. 2007). This umbrella term encompasses SSc, SLE, mixed connective tissue disease (MCTD), Sjo¨gren’s disease (SjD), primary/secondary Raynaud’s phenomenon (PRP/ SRP), and rheumatoid arthritis (RA). Scleroderma denotes a condition presenting as thickening, hardening, and scarring of the skin. Systemic refers to an autoimmune condition that affects the internal organs as well as the skin and superficial (subcutaneous and “deep investing”) fascia layers. This chapter will focus on the systemic forms of the disease because of their wider significance for manual therapy (MT) intervention. Conditions classified under the “scleroderma umbrella” generally share the following characteristics: 1. All are chronic, inflammatory, autoimmune conditions. 2. Etiology is multifactorial, with potential genetic, environmental, physical, and emotional health predisposing factors, with no known cause. Excessive coincidence or accumulation of such factors in relation to the individual’s specific immune tolerance triggers a “mistaken identity” failure within this system. Healthy body cells are erroneously interpreted as foreign, Q 2012, Elsevier Ltd.
threatening entities to be destroyed by the creation of associated antibodies. Blood tests can identify disease-specific abnormal antibodies which predominantly target the skin and fasciae or connective tissues (CTs) (Denton & Black 2002). 3. Prevalence is eight times greater in women than men, with onset between the mid 20s and mid 50s. 4. While various interventions, including drugs, can help stabilize and manage these conditions, they have no known cure. Chronic inflammation of the affected tissues leads to debilitating consequences including severe pain, fatigue, further immune system overload, tissue and organ damage, loss of functional autonomy, and quality of life. Complications from systemic forms of scleroderma, usually associated with infection, critical cardiorespiratory or renal failure, can be fatal, though survival rates have risen significantly (Denton & Black 2002), with SLE survival rates improving from <50% at 5 years in the 1950s, to 85% at 10 years nowadays (Merrell & Shulman 1955; Urowitz et al. 1997; Bernatsky et al. 2006; Abu-Shakram 2008). 5. Pathological hardening, thickening, scarring of any tissue affects three essential systems of immediate and significant relevance to manual therapy (MT): The vascular system The immune system, including the lymphatic system; and The fascial system (Denton et al. 1996, Denton & Black 2002; Assassi et al. 2007; Abu-Shakram 2008).
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Fascia-Related Disorders
Clinical features of special relevance to MT The most significant consideration from a MT perspective is the impact of scleroderma on the CTs or fasciae. A sound understanding of how these are affected is critical to appropriate clinical reasoning, and an effective treatment and management plan. Martin’s (2009) case study of a diffuse systemic sclerosis (dSSc) patient summarizes the pathological processes involved, which are characterized by fibrotic changes associated with fibroblastic cell over-activity, leading to collagen overproduction within those tissues (Denton & Black 2002). Ensuing adverse tissue changes include thickening, shortening, hardening, and scarring, which in turn result in reduced range of motion (RoM), vascular, lymphatic, neural, joint, and visceral compression and constriction. Consequences include ischemic pain, with potential local tissue necrosis or infection, sensory, motor, and autonomic dysfunction, and further immune deregulation, thus completing the “downward spiral”(Adams et al. 2002; Denton & Black 2002). This intricately linked neurovascular–fascial pathology accounts for much of patients’ pain, functional impairment, and psychological distress (Findley & Schleip 2007; Martin 2009). It is important from a MT clinical perspective to appreciate the nature and process of damage to the varying fasciae of different structures and organs.
Features affecting the neurovascular and fascial systems Vasculitis Collagen overproduction within vascular walls (epi- and endothelium) leads to thickening, stiffening, narrowing of their internal diameter, and hence ischemia to the tissues and cells supplied. The very fine capillaries supplying oxygen at cellular level are the most susceptible. The most commonly involved tissues and organs include: the extremities – hands and feet, especially the fingers and/or toes, the gastrointestinal (GI) tract, lungs, heart, kidneys, as well as muscles and joints.
The myofascial system Fibrotic myofascial change can vary in severity both in terms of area(s) affected and degree of ensuing restriction and dysfunction. It is near-universal in 226
the systemic forms of the disease, and can result in unrelenting, debilitating myofascial pain. In the author’s experience, fibrotic shortening and restriction particularly occur in the flexor muscle groups. Increased secondary spinal curves at the lumbar and mid–upper cervical levels are common, forcing the head anteriorly in relation to the body’s center of gravity, with a posterior tilt (“chin poke”). Additional asymmetrical fascial changes occur both locally and over a distance, with postural and functional impact, compounded by pain, fatigue, and sometimes muscle weakness. The face, mouth, and jaw are commonly affected, which can lead to dental overcrowding, mouth opening restriction, and associated eating and other implications for daily life. Joint inflammation (particularly in SLE patients) presents as acute swelling, pain, and stiffness. The wrists, hands, fingers, or feet and toes can develop permanent deformities, usually associated with secondary Raynaud’s phenomenon and sclerodactyly.
Secondary Raynaud’s phenomenon (“Raynaud’s”) Most scleroderma sufferers experience abnormal, severe vasoconstriction to the small capillaries in their fingers and/or toes when exposed to cold, emotional stress, or immune system overload, for instance prolonged excessive working hours and inadequate sleep. Typically, the affected hands and particularly the fingers become chronically swollen, which further compromises vascular and lymphatic circulation. Fibrotic changes, coupled with collagen overproduction as described above, thicken and stiffen the capillary walls, thereby reducing or even occluding their inner diameter.
Sclerodactyly Aggressive fibrotic changes to the skin and subcutaneous fascia predominantly manifest in the hands, fingers, feet, and/or toes with swelling, tightening, hardening, and thinning of the skin, which takes on a waxy, shiny appearance. The tightness is frequently associated with shortening of underlying fasciae, resulting in claw-like finger deformities. Commonly, the wrist and metacarpophalangeal joints become locked in relative extension, while the proximal and distal interphalangeal joints (IPJ) become “locked” into mild to excessive flexion. The thumb saddle joint adducts, with IPJ hyperextension. When
Scleroderma and related conditions
distal limb sections are affected, patients exhibit semi-flexed elbows or knees at rest due to excessive contracture.
Pulmonary sclerosis/pulmonary hypertension (PHT) A thickening, hardening, and scarring of the alveolae and/or adjacent capillary epi-/endothelium can induce, through fibrosis, two related potentially life-threatening situations: (i) reduced oxygen uptake and carbon dioxide removal through the thicker, inelastic, fibrotic tissue layer, leading to excessive breathlessness; (ii) rise in pulmonary arterial pressure due to the same fibrotic narrowing or occlusion of the capillaries adjacent to the alveolae. Pleuritis inflammation of the pleura, the outer fascial lining of the lungs, can also occur, causing chest pain aggravated with coughing.
CHAPTER 5.6
bidirectional peristalsis. Poor peristalsis can lead to cyclical episodes of bacterial overgrowth; symptoms can include alternating bouts of constipation and diarrhea, nausea, sometimes with vomiting, abdominal bloating, swelling, severe pain, tenderness, and extreme exhaustion. 3. Lower GI tract extremity/anal canal: fibrotic damage to the neurovascular–fascial structures of the inner and/or outer anal sphincter(s) affects the sensory-motor supply to the pelvic floor musculature, inhibiting the normal closure reflex. This can cause loss of full bowel control and intermittent leaking or incontinence. GI tract involvement can have a particularly profound psychosocial impact on those affected and their families.
Other potential organ involvement Myo- and pericardiac damage More rarely, interstitial pulmonary fibrosis as described above can progress to severe lung damage, triggering an immunoinflammatory response in the heart. This presents as pericardial swelling, left ventricular (“pressure pump”) hypertrophy against reduced right ventricular (“volume pump”) input in response to pulmonary arterial hypertension, arrhythmia, and diastolic dysfunction. Fibrosis of the heart muscle can be a further serious risk factor (Stamenkovic 2008, Allanore 2010).
The same inflammatory/fibrotic process rarely affects the brain, liver, and/or kidneys. Reduced white blood cells (leukopenia) or a tendency to bruise or an increased risk of bleeding, due to a low platelet count (thrombocytopenia) may also occur. Peripheral neurological disturbance can present as tingling, loss of sensation, numbness, or weakness.
Types of scleroderma, and where SSc fits in
GI tract 1. Upper GI tract: the lower esophageal sphincter (LES) opens upon swallowing, to enable food to be propelled into the stomach, and closes in between swallowing to prevent reflux of stomach contents back into the esophageal tube in a coordinated way, and is an essential part of overall peristalsis (Gaumnitz & Fayyad 2009). Atrophy and fibrosis result in acid and general stomach contents reflux when reclining, bending forward, or even sitting after food or drink consumption. Second, it results in difficulty in swallowing, notably solid food, and particularly when time has elapsed since fluid intake and/or with low salivation. 2. Mid/lower GI tract: smooth muscle atrophy and fibrosis can result in distention of the intestinal diameter, leading to inefficient, sometimes
Scleroderma is categorized according to the extent of skin and organ involvement, which with childhood onset can lead to growth deformities.
Localized scleroderma (LSc) Morphea presents as: localized patches of thickened, sclerotic, usually discolored (darker or lighter) skin, most often oval or round in shape, which can appear anywhere on the body. These patches are normally painless but can be itchy. Linear scleroderma presents as: lines of hardened, sclerotic skin that can appear on the head, arms, or legs, and can involve the subcutaneous (areolar) fascia as well as underlying myofascia and even bone. 227
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Systemic sclerosis (SSc) Limited cutaneous systemic sclerosis presents with: calcinosis; Raynaud’s phenomenon; esophageal dysfunction; sclerodactyly; and telangiectasia (Denton & Black 2002), represented by the acronym CREST. Diffuse cutaneous systemic sclerosis: has the highest incidence of fascial and organ pathologies, and higher morbidity and mortality rates (Denton & Black 2002; Stamenkovic 2006); with renal failure a common complication.
Mixed connective tissue disease (MCTD) These patients have a mixture of local and diffuse tissue involvement.
Conventional medical management Reduction in scleroderma-related mortality has led to a priority for morbidity management strategies (Merrell & Shulman 1955; Urowitz et al. 1997; Denton & Black 2002; Bernatsky et al. 2006; AbuShakram 2008). Disease stabilization and patient management remain a challenging order, often with limited to moderate success. Patient education in what treatments and self-management options are available is an essential component, as is the careful correlation between intervention selection and disease subset and stage (Denton & Black 2002). Recent conventional medical prognostic developments include:
• Scleroderma “hallmark” antibody screening to identify patients at increased risk of complications. • Preventative patient screening for pulmonary hypertension. Conventional medical treatment and management aimed at stabilizing or even modifying the disease process broadly comprises three categories: • Strategies targeting the vascular system, and notably Raynaud’s symptoms. These include oral and intravenous (prostacyclin/iloprost) vasodilator and anticoagulant medication aimed at dilating capillary vessels and inhibiting clotting, with the view to maximizing blood flow through 228
the finest vessels in the extremities and/or organs. Other conventional interventions for hypertension are also used with variable success to control pulmonary HT (Wigley et al. 1992; Denton & Black 2002). • Immunosuppressive drugs, aimed at inhibiting autodestructive immune responses, have shown limited results for DCSSc patients, but remain largely ineffective for LCSSc sufferers. • Antifibrotic therapy, in the form of antagonist drugs aimed at inhibiting fibroblast proliferation and extracellular matrix synthesis, is summarized by Denton & Black (2002). In summary, while advances in medicine have reduced mortality and enhanced disease stabilization and patient (self-)management, these improvements remain limited, and elicit inconsistent responses from patient to patient, with frequent adverse medication side effects. This calls for the exploration of nonpharmaceutically based treatments to control, or even reverse symptoms, and notably tissue fibrosis.
Can MT help reduce or reverse scleroderma-related fibrotic changes? Clinical evidence to date Fascial release (FRT) and structural integration (SI) Walton (2007) reports briefer Raynaud’s attack episodes following a series of FRT treatments. Martin (2009) reports significant gains in chest expansion, temporomandibular (TMJ) mobility, and associated increase in mouth opening, marked wrist and finger joint RoM gains (of up to 100%), reduced digital ulcerations and overall Raynaud’s symptoms, recovery of nail growth, and most importantly perhaps, reduced or eliminated pain in all affected areas. Benefits were said gradually to decrease with prolonged periods of nonintervention, but were readily restored with intermittent treatment resumption. Increased parasympathetic activity, regulation of associated autonomic functions, and other related beneficial changes following manual therapy interventions including FRT have been recently reviewed by Moyer et al. (2004). The author’s first-hand clinical experience with some 20þ SSc patients in the UK and Europe shows
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encouraging treatment effects of manual therapies, in conjunction with ongoing medical review and management, including via appropriate pharmaceutical drugs (Ball 2010), including enhancement of fascial “texture“, glide, and remodeling.
• Decreasing: pain; use of pain medication; episodes of exhaustion; duration and frequency of GI tract malfunction; hand and finger swelling, stiffness, tenderness. • Enhancing: functional mobility and RoM; hand and finger grip; mouth opening; functional autonomy; positive emotional state; and quality of life.
Rationale for potential MT efficacy FRT techniques, the SI process, and the KMIt model Through FRT manual manipulation of the connective tissues coupled with movement re-education, SI aims to restore length, symmetry, and balance to the body about its vertical skeletal axis in all planes (Myers 2009). In SI terminology, the aim is to restore optimal “tensegrity”, integrity of equal tension in all planes between the fascial tissues “pulling”, and hence applying compressive forces on, the skeletal structure. The Kinesis Myofascial Integration (KMI)t SI model developed by Thomas Myers (2009), which formed the basis of the author’s therapeutic approach, rests on “reading and treating the cohesive myofascial continuities of the Anatomy Trains”. The “Anatomy Trains” are a system of 12 key myofascial continuities, “myofascial meridians”, that drape the body from either head or neck to foot or toe, from trunk to hand or finger, along a variety of relatively straight, spiral, curvy, or “basket-weave” alignments (Myers 1997, 2009). The process therefore involves accurate “body reading” to identify areas of shortened, congested, CT, compensated by overstretched, lengthened fascia elsewhere. “Interactive” manual manipulative techniques usually involving active client movement and participation are then methodically applied according to a structured treatment strategy based on associated clinical reasoning.
Potential mechanism of effect SI/KMIt-attributed fascial changes among the general population, and specific clinical cases including scoliosis, have been well documented (Chaitow & DeLany 2003; LeBauer et al. 2008; Myers 2009).
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Similar interventions with scleroderma patients, possibly with greater repetition or frequency, using softening, releasing, spreading, and lengthening techniques, may generate tissue remodeling, over time leading to reversing fibrotic CT and skin changes (De Lany et al. 2002; Schleip 2003). These immediate and longer-term changes would in turn:
• Enhance joint RoM and overall mobility. • Relieve vascular, lymphatic, neural, joint, and visceral compression and constriction. Leading to: • Improved vascular and lymphatic circulation, and hence oxygen and nutrition supply to chronically ischemic, inflamed, or damaged tissues. • Removal of congested interstitial fluid, metabolic waste, etc. • Decreased pain and stiffness. • Enhanced neural conduction sensory, motor, and autonomic. • Enhanced visceral “position” and function. In turn, decreased vascular, neural, and visceral compression would lead to reduced ischemic pain, raised pain threshold due to nociceptor nerve ending decompression, more effective respiratory and GI tract function. The overall outcome from the above could reasonably be assumed to include: • Reduced fatigue and anxiety. • Enhanced tissue healing, functional mobility, autonomy, quality of life, emotional state, autonomic and immune function (Chaitow & DeLany 2000, 2003; Evans et al. 2000; Adams et al. 2002; Findley & Schleip 2007; Huijing et al. 2009; Martin 2009).
Scientific basis: potential therapeutic mechanisms Fascial changes such as softening, increased pliability, flexibility, and mobility These suggest a process of fascial tissue remodeling involving structural reorganization favoring greater flexibility, as proposed by Martin (2009). Pioneering research by Schleip (2003) and Wipff & Hinz (2009) 229
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on myofibroblast development, and which key factors determine whether or not they maintain their “contractile phenotype” on maturation, offers appealing, and in the author’s view promising supporting evidence regarding the possible mechanisms involved: according to the study, mechanical stress such as FRT therapy is one of two principal factors, the other being the ability to utilize transforming growth factor-beta (TGF-b1).
Restored independent sliding, gliding, and coordination between adjacent myofascial structures and associated enhanced areolar fascia “fluidity” (Fourie 2008) This may likewise be attributed in part to the above process. Myers (2009) proposes the vivid analogy of “rolling” a grapefruit, thereby gently breaking up the various membrane layers separating the juice cells to facilitate the subsequent task of juicing, and the effect of FRT input on the areolar fasciae, intermuscular septae, and epimysia.
Decreased myofascial pain This may reasonably be attributable to multiple factors. LeMoon (2008) proposes a convincing “fasciagenic” pain model where prolonged, unremitting fascial thickening and stiffening is deemed responsible for generating myofascial pain symptoms. Conversely, fascial relaxation interventions are proposed as a key strategy towards alleviating it.
Findings arising from the 2009 2nd International Fascia Congress Building on earlier studies inter alia by Rijkelijkhuizen et al. (2005) and Schleip ( Findley & Schleip 2007; Van der Wal 2009a, b) demonstrated how ligament tissue fulfills key functional roles in any and every joint position, because of the “in series” as opposed to “in parallel” organization of muscles, aponeuroses, ligaments, and periosteum, in an ever-continuous, uninterrupted body-wide pattern. The fresh cadaver dissections and in-vivo video clips testified to how the omnipresent interconnectivity of the fascial matrix ensured a trans-muscular tensile force transmission in series, involving muscle–aponeurosis–ligament 230
tissue–periosteum, with an unbroken continuum both around a given joint, and from one joint to the next throughout the body. This offers compelling anatomical and physiological evidence as to how FRT-type therapeutic intervention at a given fascial site and layer might impact on deeper, manually unreachable tissue layers. These could be intrathoracic or abdominal myofascial and visceral structures and organs, with ensuing enhanced respiratory, visceral, and autonomic regulation.
Psychoneuroimmunology (PNI) In light of the acknowledged correlation between scleroderma-type disease and sympathetic over-activity (Denton et al. 1996), it would seem reasonable to speculate that enhancing physical and psychological well-being in patients via SI intervention, including stimulating parasympathetic activity, might help regulate autoimmune response. Strategies could include placing greater focus on “end game” attention to the cervical and sacral areas, as well as consciously and sensitively addressing sympathetic responsive sites such as the thorax, shoulders, and abdomen.
Neuromuscular technique (NMT) and muscle energy technique (MET) Evidence of NMT and MET efficacy in addressing chronic musculoskeletal pain and dysfunction is robustly supported by Chaitow (2003) and Chaitow & DeLany (2000, 2003): not only do these approaches take into account both local and distant potential sources of pain and dysfunction, but they seek to identify and address self-perpetuating factors such as myofascial trigger points (MTPs), areas of “locked short” versus “locked long” tissues reflecting loss of tensegrity, detrimental posture, and hence movement patterns. That NMT aims specifically to alter the ground substance of connective tissue, to deactivate myofascial trigger points, and reduce ischemia, and to assess and beneficially modify postural alignment, musculoskeletal dysfunction, and neural entrapment Chaitow & DeLany 2003.
offers plausible rationale for efficacy in reversing scleroderma-related fascial fibrotic change. By the same token, the essence of the neurophysiological process underpinning MET techniques such as postisometric relaxation (PIR) and reciprocal
Scleroderma and related conditions
inhibition (RI) is inhibition, in the sense of enhanced relaxation enabling shortened myofascia to be restored to a more ‘normal’ resting length. Its clinical efficacy in musculoskeletal therapy is well documented (Chaitow & DeLany 2003); Mitchell & Mitchell (1995, 1998, 1999) once again provide reasonable grounds for anticipating beneficial outcomes in the context of scleroderma.
Manual lymphatic drainage (MLD) The efficacy of MLD in reducing acute and chronic edema and excessive interstitial fluid is likewise well documented (Chaitow & DeLany 2003; Willis 2004; Fourie 2009). Its acceptance in the UK as a useful modality to address lymphedema, including notably during pregnancy, following breast cancer
CHAPTER 5.6
surgery/reconstructive surgery, is gaining momentum, with encouraging early evidence of efficacy for rheumatoid arthritis and scleroderma (www. bmlda.org.co.uk).
Conclusion The current inadequacy of conventional medicine in alleviating, or reversing, scleroderma-related fascial change, anecdotal indications of MT efficacy in this respect, and the growing evidence-base supporting MT therapeutic mechanisms, jointly offer an exciting and wide-ranging opportunity for much-needed ‘clinical’ and ‘laboratory’ research in the field over the coming years.
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