The Spectrum of Rheumatic Diseases

Chapter 1

The Spectrum of Rheumatic Diseases Estefanı´a Calle* and Jose´ A. Go´mez-Puertax, 1

*Universidad de Antioquia, Medellı´n, Colombia; xHospital Clı´nic, Barcelona, Spain 1 Corresponding author

1. INTRODUCTION Rheumatic diseases include a wide spectrum of disorders that can involve many tissues, with a predominant involvement of the musculoskeletal system. Rheumatic diseases can be classified into different ways, but a simple form of classification is based on their nature. Rheumatic diseases are divided into autoimmune, autoinflammatory, and degenerative/metabolic disorders. Table 1.1 summarizes the main rheumatic disorders according to their origin. TABLE 1.1 General Classification of Rheumatic Diseases Autoimmune Systemic lupus erythematosus Rheumatoid arthritis Systemic sclerosis Idiopathic inflammatory myopathies Antiphospholipid syndrome Sjo¨gren’s syndrome Primary vasculitis

Large vessel vasculitis

Takayasu arteritis, giant cell arteritis

Medium vessel vasculitis

Polyarteritis nodosa, Kawasaki disease Continued

Surgery in Rheumatic and Musculoskeletal Disease. https://doi.org/10.1016/B978-0-444-63887-8.00001-3 Copyright © 2018 Elsevier B.V. All rights reserved.

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TABLE 1.1 General Classification of Rheumatic Diseasesdcont’d Small vessel vasculitis

ANCA associated, immune complex mediated

Variable vessel vasculitis

Behcet’s disease, Cogan syndrome

Single-organ vasculitis Autoinflammatory Familial Mediterranean fever TRAPS Mevalonate kinase deficiencies MuckleeWells syndrome Familial cold urticaria NOMID syndrome NLRP12-associated syndrome PAPA syndrome Chronic recurrent multifocal osteomyelitis Adult-onset Still disease Metabolic Osteoporosis Parathyroid disorders Paget’s disease Calcium and phosphorus disorders Vitamin D deficiency/osteomalacia Renal osteodystrophy Calcium deposition disease (CPPD, gout, hydroxyapatite) Hemochromatosis Ochronosis Degenerative Osteoarthritis CPPD, calcium pyrophosphate deposition disease; PAPA, pyogenic arthritis, pyoderma grangrenosum, and acne.

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Autoimmune diseases include a range of disorders from organ-specific (e.g., Hashimoto’s thyroiditis, vitiligo, type 1 diabetes, among others) to systemic disorders with multiorgan involvement. Autoimmune rheumatic diseases affect mainly, but not exclusively, muscles and joints and include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), inflammatory myopathies, systemic sclerosis (SSc), and systemic vasculitides, among others [1]. In systemic autoimmune diseases there is an impairment in the balance between avoidance of self-attack and recognition of pathogens, and the immune system is continuously activated in the absence of any infection [2].

2. SYSTEMIC AUTOIMMUNE DISEASES 2.1 Systemic Lupus Erythematosus SLE is a chronic, multisystemic disease that mainly affects young women. SLE has a protean manifestation that can vary from mild to severe, and the diagnosis is based on symptoms, laboratory tests, and antibodies [3]. Almost any organ can be involved, including joints, lungs, kidneys, skin, and central nervous system, among others. There are some differences among races and ethnicities, with more severe forms in Afro-Americans and Mestizo patients. Recognition of flares is not always an easy task for clinicians, especially for those cases with concomitant infections. SLE treatment is selected according to the different manifestations and is based on antimalarial agents, which should be used unless a contraindication exists, glucocorticoids and immunosuppressive agents such as mycophenolate mofetil, cyclophosphamide, azathioprine, and methotrexate (MTX), as well as targeted immunotherapy with belimumab or rituximab (anti-CD20 monoclonal antibody) [4]. Severe organ involvement by SLE usually requires large-dose steroids, which might lead to related postoperative complications. SLE patients also are prone to have preoperative coexisting medical conditions, such as hypertension, congestive heart failure, coronary disease, osteoporosis, chronic renal disease, and hypothyroidism, and there has been demonstrated that they show higher rates of 30-day postoperative complications, including infections, acute renal failure, and pulmonary embolism [5]. Thromboembolic risk is elevated independent of the presence of antiphospholipid (aPL) antibodies just because of the endothelial dysfunction secondary to the persistent inflammatory state [6].

2.2 Rheumatoid Arthritis RA affects 0.5%e1.0% of adults in developed countries, its prevalence rising with age is three times more frequent in women than men. RA results from

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different genetic risks and environmental exposures, mainly smoking. It is characterized by systemic inflammation, persistent synovitis, and autoantibodies, particularly to citrullinated peptides (anti-CCPs) and rheumatoid factor [7]. The typical presentation is characterized by tender and swollen joints of recent onset, morning joint stiffness, and elevation acute-phase reactants (erythrocyte sedimentation rate and/or C-reactive protein). However, RA can also involve extraarticular tissues in the form of interstitial lung disease and vasculitis, leading to complications related with chronic inflammation, including a higher risk for cardiovascular disease [8]. The cervical spine is involved in up to 86% of patients with RA, especially in those with more severe forms of the disease or with inadequate treatment. The most common site of involvement is the atlantoaxial region, with loss of ligamentous restriction, leading to atlantoaxial instability. Simple X-rays are recommended for screening cervical instabilities, and in some cases, CT scan or MRI should be performed when abnormalities are not clearly detected on plain X-rays [9]. It is an important fact to take into consideration in the preoperative evaluation and cervical spine assessment to prevent spinal cord injuries during orotracheal intubation, not only in RA patients but also in some other rheumatic disorders, such as ankylosing spondylitis and psoriatic arthritis, in which the cervical spine is also vulnerable [10]. Regarding treatment, MTX should be part of the first strategy unless contraindications are observed, and short-term glucocorticoids should be considered when initiating conventional disease-modifying antirheumatic drugs (DMARDs). If the treatment target is not achieved (low disease activity or remission) with the first strategy, another DMARD, either conventional or biological therapy or small molecules (Janus Kinase (JAKs) inhibitors), could be added [11]. Biologics are known to impair the defense system and have been reported to cause serious adverse events, including infections. Given the higher risk of infections in patients receiving biologic therapies, treatment withdrawal is recommended some weeks before surgery, according to drug’s half-life [12]. The surgery should be scheduled at the end of the dosing cycle (see Table 1.2) and medication resumed at minimum 2 weeks after the procedure.

2.3 Systemic Sclerosis SSc is an immune-mediated disease characterized by fibrosis of the skin, internal organs, and vasculopathy and has greater mortality than other rheumatic diseases. It is classified based on the extent of skin involvement into limited skin fibrosis, where it is restricted to the distal extremities and face, or diffuse cutaneous, with the trunk and proximal extremities also affected [13,14]. There is a small group called sine scleroderma in which there are characteristic clinical features and antibodies but no skin involvement [13].

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TABLE 1.2 Dosing Interval for Biologic Agents [53] Biologic

Interval

Adalimumab

Every 2 weeks (SQ)

Abatacept

Weekly (SQ) or monthly (IV)

Certolizumab

Every 2 or 4 weeks

Etanercept

Weekly or twice weekly

Golimumab

Every 4 weeks (SQ) or 8 weeks (IV)

Infliximab

Every 4, 6, or 8 weeks

Rituximab

2 doses 2 weeks apart every 6 months

Tocilizumab

Every week (SQ) or every 4 weeks (IV)

The American College of Rheumatology/European League Against Rheumatism criteria for the classification of SSc include skin thickening of the fingers proximal to the metacarpophalangeal joints, puffy fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, pulmonary arterial hypertension or interstitial lung disease, Raynaud’s phenomenon, and scleroderma-related antibodies (anticentromere, anti-Scl-70, or anti-RNA polymerase-3) [15]. As a result of clinical manifestations, a meticulous preanesthesia check-up is essential. Owing to the difficulty of a peripheral venous access because of vasoconstriction, flexion contractures, and skin thickening, it is often necessitated to get a central one. Orotracheal intubation may be made difficult by the limitation in oral opening and also leads to profuse bleeding because of mucosal telangiectasia. In addition, there is increased risk of aspiration due to lower esophageal sphincter incompetence and esophageal dysmotility [16]. Intravenous fluids should be warmed before administration to minimize peripheral vasoconstriction [17]. Pulmonary hypertension is not uncommon in patients suffering from SSc. Several complications might occur in patients with pulmonary hypertension during surgery, including hypotension, respiratory insufficiency, or right ventricular failure, either intraoperatively or postoperatively. Additionally, factors contributing to pulmonary hypertension in the perioperative period include hypoxia, fluid overload, positive pressure ventilation, and increased sympathetic tone due to pain and airway instrumentation [18].

2.4 Idiopathic Inflammatory Myopathies Idiopathic inflammatory myopathies are rare diseases characterized by proximal muscle weakness, elevated levels of enzymes derived from skeletal

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muscle, nonsuppurative inflammation in muscle biopsy, and myopathic pattern on electrophysiological studies. They include dermatomyositis, juvenile dermatomyositis, polymyositis, inclusion body myositis, and necrotizing myopathy [19]. Dermatomyositis presents with a photosensitive erythematous rash involving photo-exposed areas and extensor surfaces of the extremities, Gottron papules, heliotrope rash, periorbital edema, and periungual telangiectasias [20]. Beside muscular compromise, there can also be vascular, pulmonary, gastrointestinal, cardiac system affection and an increased risk of malignancy within 0e5 years of disease onset [20]. Most patients show at least a partial response to high-dose corticosteroid therapy, often associated to other immunosuppressive agents such as azathioprine or MTX, but there are refractory cases in which other drugs such as mycophenolate mofetil, tacrolimus, intravenous immunoglobulins, and rituximab have to be used [21]. In the perioperative setting, there are some manifestations that should be taken into account. Interstitial lung disease occurs in 10%e20% of patients with dermatomyositis and is characterized by reduced diffusing capacity of lungs for carbon monoxide and forced vital capacity [22], and there are reports of spontaneous pneumomediastinum as a rare complication of dermatomyositis and polymyositis [23]. There is also a risk of cardiomyopathy, manifested as congestive heart failure or arrhythmias, and involvement of the skeletal and smooth muscles of the gastrointestinal tract can lead to delayed gastric emptying and aspiration [22].

2.5 Antiphospholipid Syndrome Antiphospholipid syndrome (APS) is defined by the coexistence of obstetric morbidity (mainly pregnancy losses) and/or vascular thrombosis (venous or arterial) with the presence of aPL, namely lupus anticoagulant, anticardiolipin, or anti-b2-glycoprotein I antibodies. It can be primary or secondary if associated with other autoimmune disorders, mainly SLE. Treatment consists of long-term oral anticoagulation or the combination of aspirin and heparin, depending on whether there are thrombotic or obstetric manifestations, respectively [24], and adjustments should be made in these drugs in the patient who will be taken to surgery. Vitamin K antagonist (VKA) must be stopped 3e4 days before surgery. Unfractionated heparin (UFH) or low-molecularweight heparin (LMWH) are recommended during admission. An uncommon, but often lethal, complication of APS is called catastrophic APS, which is characterized by multiple vascular occlusive events, with a mortality rate around 40%e50%. Catastrophic APS is often triggered by situations such as infections, anticoagulation withdrawal, or surgical procedures [25].

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There has been demonstrated that after a first episode of thrombosis, patients with APS have a higher risk of recurrent thrombosis. They are also considered at high risk for perioperative thrombosis and because of this they require bridging therapy defined as the administration of a short-acting anticoagulant, such as intravenous UFH or subcutaneous LMWH during the perioperative period until resumption of oral anticoagulants. It should not be forgotten, however, that in some cases the risk of bleeding associated with bridging therapy might exceed the risk of thrombosis [26]. In patients who require temporary interruption of a VKA before surgery, VKAs should be stopped 5 days before surgery to allow an INR < 1.5 [27] and bridging therapy should start 36 h after the last warfarin dose, usually around 3 days before surgery. The last dose of LMWH is administered 24 h before the procedure using half the normal daily dose. UFH may be of particular use in severe renal involvement, in whom LMWHs should be avoided. The better moment to restart bridging therapy depends on procedural bleeding risk. VKAs could be restarted 12e24 h after surgery if there is adequate hemostasis and bridging with LMWH or UFH within 24 h of a minor procedure or 48e72 after a major surgery that confers high risk of bleeding [27,28].

3. AUTOINFLAMMATORY DISEASES Autoinflammatory diseases are a group of diseases characterized by unprovoked episodes of inflammation, without antigen-specific T cells or high-titre autoantibodies, that are caused by dysregulation of innate immunity. They are manifested by recurrent episodes of fever, skin rashes, abdominal or chest pain, lymphadenopathy, or arthritis [29]. They include familial Mediterranean fever, Tumor necrosis factor receptorassociated periodic syndrome (TRAPS), mevalonate kinase deficiencies, MuckleeWells syndrome, familial cold urticaria, Neonatal onset multisystem inflammatory disease (NOMID) syndrome, NLRP12-associated syndrome, and the deficiency of the interleukin (IL)-1ereceptor antagonist. There have also been described diseases with a Mendelian pattern of inheritance but lacking the intermittent character of the symptoms, including PAPA syndrome (pyogenic arthritis, pyoderma grangrenosum and acne) and chronic recurrent multifocal osteomyelitis associated with anaemia, and there is a third group of inflammatory diseases that lacks Mendelian inheritance, like adult-onset Still disease [30]. These syndromes present mainly in young children, with recurrent unexplained fever associated with episodic symptoms in eyes, skin, thorax, gastrointestinal, musculoskeletal, and central nervous systems, or even in asymptomatic patients with unexplained increases in laboratory indices of inflammation [31]. The treatment is usually based on colchicine, glucocorticoids, and IL-1 inhibitors [31].

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4. METABOLIC DISEASES Bone mineral metabolism disturbances may end in decreasing bone mineral density leading to osteoporosis, and articular and periarticular deposits of calcium pyrophosphate, hydroxyapatite, and monosodium urate are a known cause of arthropathy and tendinopathy.

4.1 Osteoporosis Osteoporosis is a metabolic bone disorder that is characterized by low bone mass and microarchitectural deterioration of bone tissue that led to increased risk of fracture, mainly affecting hip, wrist, and spine and is considered a public health problem because of its high prevalence and relation with millions of fractures worldwide each year [32]. Osteoporosis is defined as a T-score (the number of standard deviation below the young adult mean value for women) of 2.5 or lower in the lumbar spine femoral neck or, independent of mineral bone density, in the presence of a fragility fracture [33]. Pharmacologic treatment is recommended for those who meet this definition or when the T-score is between 1.0 and 2.5 if the FRAX 10-year probability for major osteoporotic fracture or hip fracture is 20% or 3%, respectively [33]. The importance of treatment lies in the fact that fractures are a major source of morbidity and mortality: 24% of hip fracture patients die within 1 year of the event, and 20% will suffer an additional osteoporotic fracture in the 2 years following the event [34]. There are many treatment options, among which are bisphosphonates. The primary that suppresses bone resorption by inhibiting osteoclast formation and deposited at the site of an acute fracture has raised the question of whether bisphosphonates might affect fracture healing [34]. To clarify this question, several studies have been carried out. A systematic review found that bisphosphonates significantly prolong union times of distal radius fractures but not femoral fractures [35]. Colo´n-Emeric et al. found that zoledronic acid had a similar incidence of delayed hip fracture healing compared with placebo when the drug was administered early (within 2 weeks, between 2 and 4 weeks, between 4 and 6 weeks, and after 6 weeks from the surgical repair) [34]. Health Outcomes and Reduced Incidence with Zoledronic acid once (HORIZON) Recurrent Fracture Trial used zoledronic acid or placebo within the first 90 days after the surgical repair of a hip fracture, finding that zoledronic acid was associated with a smaller percentage of new clinical fractures and less mortality in a follow-up of 5 years [36]. Eriksen et al. examined whether timing of the first infusion of zoledronic acid study drug after hip fracture repair influenced the antifracture efficacy and mortality benefit, finding that administration of zoledronic acid to patients suffering a low-trauma hip fracture 2 weeks or later after surgical repair

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increased hip bone mineral density, induced reductions in the risk of subsequent clinical vertebral, nonvertebral, and hip fractures, and reduced mortality [37]. In accordance with the above, there seems to be no contraindication for the early initiation of zoledronic acid days after the surgical repair of a hip fracture and on the contrary, this approach seems to have beneficial effects in relation to the risk of recurrent fracture and mortality.

4.2 Crystal Deposition Disease 4.2.1 Calcium Pyrophosphate Disease (CPPD) CPPD or pseudogout crystal deposits in articular and periarticular tissues [38] occurs mainly in the elderly and has been related to predisposing factors, including hyperparathyroidism, haemochromatosis, and hypomagnesaemia [39]. It may be asymptomatic, presenting as incidental finding of chondrocalcinosis on X-ray, or with acute or chronic arthritis, resembling other arthropathies [40]. In acute CPPD patients present with oligoarticular or monoarticular arthritis with swelling, erythema, and warmth in and around the joint, and with elevated ESR and CRP, resembling septic or gouty arthritis [41], and the most commonly affected joints are knees, wrists, and shoulders [40]. Chronic forms present mainly with polyarticular involvement that differentiates from primary osteoarthritis (OA) by serious articular damage involving joints not often affected by primary OA, and episodic flares with inflammatory signs [41]. It may also present as symmetrical polyarthritis frequently affecting metacarpophalangeal joints and wrists, making it challenging to differentiate it from RA [38]. Although the diagnosis of CPPD may be suspected based on clinical and radiographic features, it should be confirmed by identification of CPPD crystals in synovial fluid or biopsied tissue [39].

4.3 Gout Gout is due to chronic deposition of monosodium urate crystals, which form in the presence of high serum urate concentrations (6.8 mg/dL) resulting from overproduction from cell turnover and hepatic metabolism or by renal or extrarenal under excretion [42]. It is the most common form of inflammatory arthritis [43] and develops in conjunction with chronic diseases, mainly cardiovascular and renal [44]. Gout may present as chronic or acute arthritis, the latter characterized by swelling, tenderness, intense pain, and erythema in a peripheral joint or bursa, mainly affecting the first metatarsophalangeal joint. These symptoms typically come quickly and reach their highest intensity within the first 24 h [43]. Flares may be triggered by dehydration, alcohol, or purine-rich food intake or medical or surgical illness [42]. Hospitalization has a fourfold increase in adjusted odds of gout attacks [44], and there has been reported that after surgery 15% of patients with gout can develop a gout attack, with a mean interval to develop it in 4.1 days, and with predilection for lower

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extremities, frequently affecting more than one joint [45]. These acute attacks should be diagnosed and treated in a timely manner, otherwise they can be mistaken for an infectious condition, delay early ambulation, and prolong hospital stay [45]. Acute flares treatment consists mainly of colchicine, NSAID, or corticosteroids. Combination therapy, such as colchicine and corticosteroids or colchicine and NSAID, is an option when there are multiple joints involved in the flare. Urate-lowering therapy (ULT) is indicated when there are tophi, urate arthropathy, renal stones, or two or more flares in a year and could be considered near to the first attack if the patient is 40 years or younger, has a uric acid level of more than 8 mg/dL, or has comorbidities such as hypertension, heart failure, ischemic heart disease, or renal impairment [46]. The optimal time to start ULT has been a matter of debate; traditionally a 2-week interval has been considered appropriate, but recently two trials found that ULT initiation during an acute gout attack did not prolong the acute, treated attack and caused no significant difference in daily pain, recurrent flares, or inflammatory markers [47,48].

5. DEGENERATIVE DISEASES 5.1 Osteoarthritis OA is the most common form of arthritis worldwide, with incidence rates for symptomatic hip, hand, and knee OA estimated to be 88, 100, and 240 cases per 100,000 person-years, respectively, with rising rates related to ageing and obesity. Owing to its predilection for lower extremity joints, it is the leading cause of lower extremity disability in the elderly [49] and is the fourth leading cause of total years lost due to disease [50]. The disease occurs when the mechanical loads exceed those that can be tolerated by the joint tissues, leading to an imbalance between the breakdown and repair mechanisms. Local mechanical factors, such as abnormal joint congruity or alteration in the joint environment, can facilitate progression of joint damage. OA is typically defined according to radiographic characteristics, including osteophytes, joint space narrowing, and subchondral sclerosis [50], but a modest correlation between pain intensity and the degree of joint degeneration has been reported [51]. The existing pharmacologic agents have not been shown to have a convincing disease-modifying effect [52], and for the moment the treatment is based on the control of pain.

6. CONCLUSIONS Rheumatic disorders include a wide spectrum of diseases, which compromise not only joints but also multiple organ systems, leading to significant morbidity and mortality. Both the diseases per se and their treatment have important implications that must be taken into account when planning and carrying out a surgical intervention.

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KEYPOINTS l

l

l

l

In patients suffering from systemic autoimmune disease, such as RA or SLE, it is important to assess current medications and comorbidities, to prevent postoperative complications. Perioperative management in APS patients undergoing surgical procedures is challenging because interrupting anticoagulation for a procedure transiently increases the risk of thromboembolism. Given their higher risk of infections in patients receiving biologic therapies, treatment withdrawal is recommended some weeks before surgery, according to drug’s half-life. Overall, bisphosphonates can prevent the appearance of hip fractures, reduce the mortality, and does not increase the overall complications in elderly patients with hip fracture.

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