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Progressive Systemic Sclerosis
Special Articles Progressive Systemic Sclerosis: Clinical Manifestations and Anesthetic Considerations James G. Roberts, MD,* Raj Sabar, MD,* John A. Gianoli, MD,* Alan D. Kaye, MD, PhD† Department of Anesthesiology, Texas Tech University, Lubbock, TX
Progressive systemic sclerosis is a complex disease that involves multiple organ systems. Patients with progressive systemic sclerosis can pose a significant challenge for the anesthesiologist. As the disease progresses, profound musculoskeletal, gastrointestinal, pulmonary, renal, and cardiac system changes occur. The anesthesiologist should understand these complex pathophysiological processes so as to minimize potential risks, including aspiration and other pulmonary complications. In addition, the potential for reduced renal function and intraoperative hypothermia-induced vasospasm must be considered. © 2002 by Elsevier Science Inc. Keywords: Airway, difficult; anesthesia; aspiration, pulmonary; systemic sclerosis, progressive.
Introduction
*Resident in Anesthesiology †Professor and Chairman, Department of Anesthesiology Address correspondence to Dr. Kaye at the Department of Anesthesiology, Texas Tech University Medical Center, 3601 4th St., Lubbock, TX 79430, USA. E-mail: aneadk@ ttuhsc.edu Received for publication March 7, 2002; revised manuscript accepted for publication March 14, 2002.
Progressive systemic sclerosis (PSS) is a multisystem disorder characterized chiefly by fibrosis of blood vessels, skin, musculoskeletal system, and visceral organs. Pathogenesis of PSS involves microvascular insult and a secondary overproduction and accumulation of collagen, activation of immunologic mechanisms, and increased fibroblast activation and proliferation. Two major forms of this disease are limited cutaneous systemic sclerosis (formerly CREST syndrome) and diffuse cutaneous systemic sclerosis. Many reference sources refer to PSS as scleroderma. Progressive systemic sclerosis is distributed worldwide involving all races; however, increased frequency and severity of this disease is observed among black females. Female to male ratio is approximately 3:1, with highest incidence occurring in the third to fifth decade of life. In the general population, the prevalence of the disease may range from 75 per 100,000 to 19 per 100,000. Highest prevalence currently known is found within full-blooded Choctaw tribes of Oklahoma, and may be as high as 469 per 100,000.1
Clinical Features The etiology of systemic sclerosis is not fully understood, although there is some evidence suggesting that this disease may be multifactorial. Human leukocyte antigens (HLA DR1, 2, 3, 5, and HLA DQA2), antinuclear antibodies, anticentromere, and antinucleolar antibodies, have all been implicated in the etiology
Journal of Clinical Anesthesia 14:474 – 477, 2002 © 2002 Elsevier Science Inc. All rights reserved. 655 Avenue of the Americas, New York, NY 10010
0952-8180/02/$–see front matter PII S0952-8180(02)00380-X
Progressive systemic sclerosis: Roberts et al.
of this disease. An increased incidence among miners, and those exposed to high cumulative levels of solvent exposure, silica dust, vinyl chloride, epoxy resins, or aromatic hydrocarbons are a few possible environmental etiologic factors.2,3 Studies linking PSS to augmentation mammoplasty have been inconclusive.4 An association with cytomegalovirus indicates a possible role in disease pathogenesis.5 Raynaud’s phenomenon is usually the initial presenting symptom and commonly precedes skin changes by less than 1 year in cases of diffuse, cutaneous PSS. Episodic vasoconstriction induced by exposure to cold, vibration, or emotional stress results in pallor of the fingers and toes and/or cyanosis, followed by rubor and pain. Although the exact mechanism of Raynaud’s phenomenon is not clearly understood, there is evidence suggesting sympathetic nervous system involvement, since pain caused by vasospasm is relieved by ipsilateral stellate ganglion blockade.6 – 8 Digital pallor is the most reliable symptom for the presence of Raynaud’s. Most patients with Raynaud’s phenomenon do not progress to PSS. Dermal thickening can result in flexion contractures, ischemic ulcerations, and resorption of distal phalanges. Cutaneous calcification occurs most commonly on the extensor surfaces of the forearms. Skin tightening can lead to microstomia. Musculoskeletal changes may include interstitial fibrosis and lymphocytic infiltration of skeletal muscle fibers, producing proximal muscle weakness. Synovia may become surrounded by a thick fibrin layer, whereas intrasynovial fibrosis, edema, and lymphocytic and plasma cell invasion produces a symmetric polyarthritis and joint crepitation. Teleangiectatic units develop on the fingers, face, tongue, lips, and buccal or nasal mucosa. In the gastrointestinal system, esophageal mucosal thinning occurs, with fibrotic thickening in the remaining layers. There may be extensive mucosal ulceration and marked esophageal dilation. In the small intestine, smooth muscle atrophy can lead to decreased absorption with diverticuli formation. Pulmonary manifestations secondary to interstitial and peribronchial fibrosis, bronchial epithelial proliferation with intimal thickening, and smooth muscle hypertrophy of the pulmonary blood vessels results in pulmonary hypertension. Pulmonary hypertension and right-side heart failure have become the leading cause of death in patients with PSS. Pulmonary function tests are consistent with restrictive changes, demonstrating decreased compliance, vital capacity, and diffusion capacity. Myocardial fibrosis is the chief cardiac manifestation, resulting in degeneration of healthy myocardial fibers, ventricular hypertrophy, diastolic dysfunction, and cardiac conduction defects. Coronary vessels become sclerotic and are subject to vasospasm. Pericarditis with effusion is very common, and myocarditis may lead to severe left ventricular dysfunction. Thickening of renal glomerular basement membrane, intimal hyperplasia of interlobular arteries, and glomerulosclerosis results in exacerbating hypertension and decreased renal clearance of drugs. Patients may experience
hematuria and proteinuria. Pregnancy is associated with disease progression in 50% of patients with PSS.9
Treatment No demonstration of disease suppression or reversal has been reported in any controlled, prospective study to date. Current treatment goals are to treat symptoms and maintain or improve organ function. Specific therapy for each individual depends on disease manifestations. The patient with Raynaud’s disease may be receiving prazosin or calcium channel blockers, such as nifedipine or felodipine. Gastrointestinal disease is commonly treated with cimetidine, metoclopramide, or proton pump inhibitors. Nifedipine may be prescribed for the patient with myocardial involvement, and pulmonary hypertension and fibrosis are typically treated with vasodilators, captopril, and immunosuppressants. Angiotensin-converting enzyme inhibitors, such as enalapril, are mandatory for the prevention of renal crisis, as well as for the added benefit of decreasing systemic hypertension. Cyclophosphamide, cyclosporine, or prednisone may be used for generalized immunosuppression, and penicillamine or interferon may be beneficial in decreasing collagen production.
Anesthesia Considerations Dermal thickening, cardiac involvement, restrictive pulmonary disease, diminished renal clearance of drugs, and lower esophageal sphincter (LES) dilation can contribute to many serious anesthesia-related complications (Table 1). Preoperative evaluation should focus on skin tightening of the face, neck, and oral aperture and the effect these changes will have on endotracheal intubation. Specifically, the patient’s ability to open his or her mouth and flex the neck should be closely assessed, and any information regarding dysphagia, regurgitation, weight loss, and digital pallor also must be noted. Late and severe disease presents with cardiac, pulmonary, and renal manifestations. The need for specific laboratory evaluations is a matter of clinical judgment and will likely vary for each individual patient based on their presentation. Patients with cardiac involvement or potential pulmonary hypertension should have an electrocardiogram (ECG), chest radiograph, and evaluation of blood urea nitrogen and creatinine. The need for further tests (i.e., exercise or stress ECG, radionucleotide scan, creatinine clearance, or echocardiogram) depends on the severity of disease. Pulmonary involvement should be evaluated with an ECG, chest radiograph, and arterial blood gas analysis. Pulmonary function tests may be useful in the evaluation of the presence, or severity, of a restrictive component of disease. Thus, in the immediate postoperative period, patients who demonstrate a vital capacity of less than 1 liter typically prove difficult to wean and extubate secondary to inadequate ventilatory effort. For patients with a history of renal disease, measurement of electrolytes, J. Clin. Anesth., vol. 14, September 2002
475
Special Article
Table 1. Common Features Associated with Progressive Systemic Sclerosis (PSS) PSS Manifestation
Anesthetic Considerations
Raynaud’s phenomenon Dermal thickening, calcifications, contractures Skin tightening, microstomia, decreased neck flexibility Telangiectasias Esophageal dilatation, decreased LES tone Intestinal malabsorption Restrictive pulmonary disease
Peripheral vasoconstriction Peripheral IV difficulties Difficult airway management Oral or nasal bleeding Aspiration Decreased Vit K-dependent clotting factors Increased positive airway pressure, increased oxygen concentration, extubation delays Ventricular hypertrophy, diastolic dysfunction, conduction defects, coronary vasospasm Hypertension, decreased renal clearance
Myocardial fibrosis Renal disease LES ⫽ lower esophageal sphincter.
blood urea nitrogen, and creatinine levels are appropriate. Finally, for patients with signs of malnutrition or malabsorption, a complete blood count, prothrombin time, partial thromboplastin time, and albumin levels should be assessed. Before arrival in the operating room, the patient’s gastric pH should be increased with an antacid or histamine blocker, because esophageal dysmotility and a decreased LES tone renders the patient vulnerable to regurgitation and increases the risk of aspiration. Metoclopramide has the dual benefit of increasing gastric motility and increasing LES tone. Operating room temperature should be maintained above 21°C, and intravenous fluids should be warmed before administration so as to minimize peripheral vasoconstriction. Intravenous access and noninvasive blood pressure monitoring may be difficult in areas involving dermal thickening, flexion contractures, and vasoconstriction. This situation may necessitate the need for ultrasonic blood pressure sensors or invasive monitoring and central venous access.10 –12 Patients with PSS may require monitoring of cardiac performance, including cardiac output, contractility, pulmonary arterial, and left ventricular filling pressures in the presence of myocardial fibrosis or pulmonary hypertension. Technical difficulties should be anticipated from the dermal thickening and the potential for vasoconstriction, as above.9 The potential of reduced mandibular mobility or oral narrowing (microstomia) may require fiber optic, blind nasal, or retrograde intubation techniques. An awake tracheostomy with local anesthesia also can be considered if the above techniques are unsuccessful, although sclerotic changes in the head and neck region may cause considerable technical difficulty.9 Trauma to oral or nasal telangiectasias should be avoided so as to prevent potentially massive bleeding. Some authors have recommended the use of regional anesthesia in these patients and, assuming that normal coagulation is present, a regional technique may be successfully utilized. For example, a carefully titrated continuous spinal anesthetic may be employed. Increased positive airway pressure and higher than 476
J. Clin. Anesth., vol. 14, September 2002
normal oxygen concentration are often required to ensure adequate ventilation and diffusion, respectively. Respiratory acidosis and arterial hypoxemia must be prevented to avoid any increase in pulmonary vascular resistance. Central venous pressure should be closely monitored during the administration of nitrous oxide, as increases in central venous pressure may indicate pulmonary artery vasoconstriction. Ventilatory depression may occur with the use of opioids and postoperative ventilatory support should be anticipated in the patient with severe coexisting pulmonary disease. There are no specific contraindications to the use of any type of anesthetic; however, intraoperative use of particular drugs should be determined on an individual basis for each patient. Further, the degree of disease severity and specific manifestations should be the primary determinants for which drugs can or cannot be used, and which drugs may be used with caution. For example, drugs requiring extensive renal clearance should be avoided, when feasible, in the patient with severe renal dysfunction. The potential for prolonged duration of action of drugs such as morphine and pancuronium must be considered. Inhalational drugs with minimal metabolism such as desflurane, and neuromuscular blockers that are metabolized via nonorgan dependent pathways (e.g., mivacurium or cisatracurium) should be considered so as to optimize patient outcome. Patients with contracted intravascular fluid volume are at risk for exaggerated hypotension with drug-induced vasodilatation.
Conclusion Progressive systemic sclerosis is a complex disease process with many different levels of involvement. By definition, however, the patient with even early PSS presents with vasoconstrictive changes (Raynaud’s disease), skin changes, and visceral manifestations, most commonly esophageal dysfunction. Late and severe disease may involve pulmonary, cardiac, or renal systems. Anesthesiologists managing patients intraoperatively must be knowledgeable about the pathogenesis, clinical manifestations, system involvement, and anesthetic considerations for the PSS surgical patient. Every aspect of
Progressive systemic sclerosis: Roberts et al.
anesthesia care in the patient with PSS may be altered or hindered by the pathogenesis of this disease. Peripheral IV access, endotracheal intubation, ventilator management, pharmacologic choices, fluid management, coagulation profiles, and postoperative extubation all may be affected. The anesthesiologist should anticipate technical difficulties in each phase of care and be prepared for complications unique to the patient with PSS.
5.
6.
7. 8.
References 1. Arnett FC, Howard RF, Tan F, et al: Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma. Association with an Amerindian HLA haplotype. Arthritis Rheum 1996;39:1362–70. 2. Nietert PJ, Sutherland SE, Silver RM, Pandey JP, Dosemeci M: Solvent oriented hobbies and the risk of systemic sclerosis. J Rheumatol 1999;26:2369 –72. 3. Englert H, Small-McMahon J, Davis K, O’Connor H, Chambers P, Brooks P: Male systemic sclerosis and occupational silica exposure– a population-based study. Aust N Z J Med 2000;30:215–20. 4. Silman AJ, Hochberg MC: Occupational and environmental
9.
10. 11.
12.
influences on scleroderma. Rheum Dis Clin North Am 1996;22:737– 49. Neidhart M, Kuchen S, Distler O, et al: Increased serum levels of antibodies against human cytomegalovirus and prevalence of autoantibodies in systemic sclerosis. Arthritis Rheum 1999;42:389 – 92. Peacock JH: Vasodilatation in the human hand: observations on primary Raynaud’s disease and acrocyanosis of the upper extremities. Clin Sci 1958;17:575– 86. Jamieson GG, Ludbrook J, Wilson A: Cold hypersensitivity in Raynaud’s phenomenon. Circulation 1971;44:254 –264. Omote K, Kawamata M, Namiki A: Adverse effects of stellate ganglion block on Raynaud’s phenomenon associated with progressive systemic sclerosis. Anesth Analg 1993;77:1057– 60. D’Angelo R, Miller R: Pregnancy complicated by severe preeclampsia and thrombocytopenia in a patient with scleroderma. Anesth Analg 1997;85:839 – 41. Black CM, Stevens WM: Scleroderma. Rheum Dis Clin North Am 1989;15:193–212. Baethge BA, Wolf RE: Successful pregnancy with scleroderma renal disease and pulmonary hypertension in a patient using angiotensin converting enzyme inhibitors. Ann Rheum Dis 1989; 48:776 – 8. Thompson J, Conklin KA: Anesthetic management of a patient with scleroderma. Anesthesiology 1983;59:69 –71.
J. Clin. Anesth., vol. 14, September 2002
477
Progressive systemic sclerosis is a complex disease that involves multiple organ systems. Patients with progressive systemic sclerosis can pose a significant challenge for the anesthesiologist. As the disease progresses, profound musculoskeletal, gastrointestinal, pulmonary, renal, and cardiac system changes occur. The anesthesiologist should understand these complex pathophysiological processes so as to minimize potential risks, including aspiration and other pulmonary complications. In addition, the potential for reduced renal function and intraoperative hypothermia-induced vasospasm must be considered. © 2002 by Elsevier Science Inc. Keywords: Airway, difficult; anesthesia; aspiration, pulmonary; systemic sclerosis, progressive.
Introduction
*Resident in Anesthesiology †Professor and Chairman, Department of Anesthesiology Address correspondence to Dr. Kaye at the Department of Anesthesiology, Texas Tech University Medical Center, 3601 4th St., Lubbock, TX 79430, USA. E-mail: aneadk@ ttuhsc.edu Received for publication March 7, 2002; revised manuscript accepted for publication March 14, 2002.
Progressive systemic sclerosis (PSS) is a multisystem disorder characterized chiefly by fibrosis of blood vessels, skin, musculoskeletal system, and visceral organs. Pathogenesis of PSS involves microvascular insult and a secondary overproduction and accumulation of collagen, activation of immunologic mechanisms, and increased fibroblast activation and proliferation. Two major forms of this disease are limited cutaneous systemic sclerosis (formerly CREST syndrome) and diffuse cutaneous systemic sclerosis. Many reference sources refer to PSS as scleroderma. Progressive systemic sclerosis is distributed worldwide involving all races; however, increased frequency and severity of this disease is observed among black females. Female to male ratio is approximately 3:1, with highest incidence occurring in the third to fifth decade of life. In the general population, the prevalence of the disease may range from 75 per 100,000 to 19 per 100,000. Highest prevalence currently known is found within full-blooded Choctaw tribes of Oklahoma, and may be as high as 469 per 100,000.1
Clinical Features The etiology of systemic sclerosis is not fully understood, although there is some evidence suggesting that this disease may be multifactorial. Human leukocyte antigens (HLA DR1, 2, 3, 5, and HLA DQA2), antinuclear antibodies, anticentromere, and antinucleolar antibodies, have all been implicated in the etiology
Journal of Clinical Anesthesia 14:474 – 477, 2002 © 2002 Elsevier Science Inc. All rights reserved. 655 Avenue of the Americas, New York, NY 10010
0952-8180/02/$–see front matter PII S0952-8180(02)00380-X
Progressive systemic sclerosis: Roberts et al.
of this disease. An increased incidence among miners, and those exposed to high cumulative levels of solvent exposure, silica dust, vinyl chloride, epoxy resins, or aromatic hydrocarbons are a few possible environmental etiologic factors.2,3 Studies linking PSS to augmentation mammoplasty have been inconclusive.4 An association with cytomegalovirus indicates a possible role in disease pathogenesis.5 Raynaud’s phenomenon is usually the initial presenting symptom and commonly precedes skin changes by less than 1 year in cases of diffuse, cutaneous PSS. Episodic vasoconstriction induced by exposure to cold, vibration, or emotional stress results in pallor of the fingers and toes and/or cyanosis, followed by rubor and pain. Although the exact mechanism of Raynaud’s phenomenon is not clearly understood, there is evidence suggesting sympathetic nervous system involvement, since pain caused by vasospasm is relieved by ipsilateral stellate ganglion blockade.6 – 8 Digital pallor is the most reliable symptom for the presence of Raynaud’s. Most patients with Raynaud’s phenomenon do not progress to PSS. Dermal thickening can result in flexion contractures, ischemic ulcerations, and resorption of distal phalanges. Cutaneous calcification occurs most commonly on the extensor surfaces of the forearms. Skin tightening can lead to microstomia. Musculoskeletal changes may include interstitial fibrosis and lymphocytic infiltration of skeletal muscle fibers, producing proximal muscle weakness. Synovia may become surrounded by a thick fibrin layer, whereas intrasynovial fibrosis, edema, and lymphocytic and plasma cell invasion produces a symmetric polyarthritis and joint crepitation. Teleangiectatic units develop on the fingers, face, tongue, lips, and buccal or nasal mucosa. In the gastrointestinal system, esophageal mucosal thinning occurs, with fibrotic thickening in the remaining layers. There may be extensive mucosal ulceration and marked esophageal dilation. In the small intestine, smooth muscle atrophy can lead to decreased absorption with diverticuli formation. Pulmonary manifestations secondary to interstitial and peribronchial fibrosis, bronchial epithelial proliferation with intimal thickening, and smooth muscle hypertrophy of the pulmonary blood vessels results in pulmonary hypertension. Pulmonary hypertension and right-side heart failure have become the leading cause of death in patients with PSS. Pulmonary function tests are consistent with restrictive changes, demonstrating decreased compliance, vital capacity, and diffusion capacity. Myocardial fibrosis is the chief cardiac manifestation, resulting in degeneration of healthy myocardial fibers, ventricular hypertrophy, diastolic dysfunction, and cardiac conduction defects. Coronary vessels become sclerotic and are subject to vasospasm. Pericarditis with effusion is very common, and myocarditis may lead to severe left ventricular dysfunction. Thickening of renal glomerular basement membrane, intimal hyperplasia of interlobular arteries, and glomerulosclerosis results in exacerbating hypertension and decreased renal clearance of drugs. Patients may experience
hematuria and proteinuria. Pregnancy is associated with disease progression in 50% of patients with PSS.9
Treatment No demonstration of disease suppression or reversal has been reported in any controlled, prospective study to date. Current treatment goals are to treat symptoms and maintain or improve organ function. Specific therapy for each individual depends on disease manifestations. The patient with Raynaud’s disease may be receiving prazosin or calcium channel blockers, such as nifedipine or felodipine. Gastrointestinal disease is commonly treated with cimetidine, metoclopramide, or proton pump inhibitors. Nifedipine may be prescribed for the patient with myocardial involvement, and pulmonary hypertension and fibrosis are typically treated with vasodilators, captopril, and immunosuppressants. Angiotensin-converting enzyme inhibitors, such as enalapril, are mandatory for the prevention of renal crisis, as well as for the added benefit of decreasing systemic hypertension. Cyclophosphamide, cyclosporine, or prednisone may be used for generalized immunosuppression, and penicillamine or interferon may be beneficial in decreasing collagen production.
Anesthesia Considerations Dermal thickening, cardiac involvement, restrictive pulmonary disease, diminished renal clearance of drugs, and lower esophageal sphincter (LES) dilation can contribute to many serious anesthesia-related complications (Table 1). Preoperative evaluation should focus on skin tightening of the face, neck, and oral aperture and the effect these changes will have on endotracheal intubation. Specifically, the patient’s ability to open his or her mouth and flex the neck should be closely assessed, and any information regarding dysphagia, regurgitation, weight loss, and digital pallor also must be noted. Late and severe disease presents with cardiac, pulmonary, and renal manifestations. The need for specific laboratory evaluations is a matter of clinical judgment and will likely vary for each individual patient based on their presentation. Patients with cardiac involvement or potential pulmonary hypertension should have an electrocardiogram (ECG), chest radiograph, and evaluation of blood urea nitrogen and creatinine. The need for further tests (i.e., exercise or stress ECG, radionucleotide scan, creatinine clearance, or echocardiogram) depends on the severity of disease. Pulmonary involvement should be evaluated with an ECG, chest radiograph, and arterial blood gas analysis. Pulmonary function tests may be useful in the evaluation of the presence, or severity, of a restrictive component of disease. Thus, in the immediate postoperative period, patients who demonstrate a vital capacity of less than 1 liter typically prove difficult to wean and extubate secondary to inadequate ventilatory effort. For patients with a history of renal disease, measurement of electrolytes, J. Clin. Anesth., vol. 14, September 2002
475
Special Article
Table 1. Common Features Associated with Progressive Systemic Sclerosis (PSS) PSS Manifestation
Anesthetic Considerations
Raynaud’s phenomenon Dermal thickening, calcifications, contractures Skin tightening, microstomia, decreased neck flexibility Telangiectasias Esophageal dilatation, decreased LES tone Intestinal malabsorption Restrictive pulmonary disease
Peripheral vasoconstriction Peripheral IV difficulties Difficult airway management Oral or nasal bleeding Aspiration Decreased Vit K-dependent clotting factors Increased positive airway pressure, increased oxygen concentration, extubation delays Ventricular hypertrophy, diastolic dysfunction, conduction defects, coronary vasospasm Hypertension, decreased renal clearance
Myocardial fibrosis Renal disease LES ⫽ lower esophageal sphincter.
blood urea nitrogen, and creatinine levels are appropriate. Finally, for patients with signs of malnutrition or malabsorption, a complete blood count, prothrombin time, partial thromboplastin time, and albumin levels should be assessed. Before arrival in the operating room, the patient’s gastric pH should be increased with an antacid or histamine blocker, because esophageal dysmotility and a decreased LES tone renders the patient vulnerable to regurgitation and increases the risk of aspiration. Metoclopramide has the dual benefit of increasing gastric motility and increasing LES tone. Operating room temperature should be maintained above 21°C, and intravenous fluids should be warmed before administration so as to minimize peripheral vasoconstriction. Intravenous access and noninvasive blood pressure monitoring may be difficult in areas involving dermal thickening, flexion contractures, and vasoconstriction. This situation may necessitate the need for ultrasonic blood pressure sensors or invasive monitoring and central venous access.10 –12 Patients with PSS may require monitoring of cardiac performance, including cardiac output, contractility, pulmonary arterial, and left ventricular filling pressures in the presence of myocardial fibrosis or pulmonary hypertension. Technical difficulties should be anticipated from the dermal thickening and the potential for vasoconstriction, as above.9 The potential of reduced mandibular mobility or oral narrowing (microstomia) may require fiber optic, blind nasal, or retrograde intubation techniques. An awake tracheostomy with local anesthesia also can be considered if the above techniques are unsuccessful, although sclerotic changes in the head and neck region may cause considerable technical difficulty.9 Trauma to oral or nasal telangiectasias should be avoided so as to prevent potentially massive bleeding. Some authors have recommended the use of regional anesthesia in these patients and, assuming that normal coagulation is present, a regional technique may be successfully utilized. For example, a carefully titrated continuous spinal anesthetic may be employed. Increased positive airway pressure and higher than 476
J. Clin. Anesth., vol. 14, September 2002
normal oxygen concentration are often required to ensure adequate ventilation and diffusion, respectively. Respiratory acidosis and arterial hypoxemia must be prevented to avoid any increase in pulmonary vascular resistance. Central venous pressure should be closely monitored during the administration of nitrous oxide, as increases in central venous pressure may indicate pulmonary artery vasoconstriction. Ventilatory depression may occur with the use of opioids and postoperative ventilatory support should be anticipated in the patient with severe coexisting pulmonary disease. There are no specific contraindications to the use of any type of anesthetic; however, intraoperative use of particular drugs should be determined on an individual basis for each patient. Further, the degree of disease severity and specific manifestations should be the primary determinants for which drugs can or cannot be used, and which drugs may be used with caution. For example, drugs requiring extensive renal clearance should be avoided, when feasible, in the patient with severe renal dysfunction. The potential for prolonged duration of action of drugs such as morphine and pancuronium must be considered. Inhalational drugs with minimal metabolism such as desflurane, and neuromuscular blockers that are metabolized via nonorgan dependent pathways (e.g., mivacurium or cisatracurium) should be considered so as to optimize patient outcome. Patients with contracted intravascular fluid volume are at risk for exaggerated hypotension with drug-induced vasodilatation.
Conclusion Progressive systemic sclerosis is a complex disease process with many different levels of involvement. By definition, however, the patient with even early PSS presents with vasoconstrictive changes (Raynaud’s disease), skin changes, and visceral manifestations, most commonly esophageal dysfunction. Late and severe disease may involve pulmonary, cardiac, or renal systems. Anesthesiologists managing patients intraoperatively must be knowledgeable about the pathogenesis, clinical manifestations, system involvement, and anesthetic considerations for the PSS surgical patient. Every aspect of
Progressive systemic sclerosis: Roberts et al.
anesthesia care in the patient with PSS may be altered or hindered by the pathogenesis of this disease. Peripheral IV access, endotracheal intubation, ventilator management, pharmacologic choices, fluid management, coagulation profiles, and postoperative extubation all may be affected. The anesthesiologist should anticipate technical difficulties in each phase of care and be prepared for complications unique to the patient with PSS.
5.
6.
7. 8.
References 1. Arnett FC, Howard RF, Tan F, et al: Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma. Association with an Amerindian HLA haplotype. Arthritis Rheum 1996;39:1362–70. 2. Nietert PJ, Sutherland SE, Silver RM, Pandey JP, Dosemeci M: Solvent oriented hobbies and the risk of systemic sclerosis. J Rheumatol 1999;26:2369 –72. 3. Englert H, Small-McMahon J, Davis K, O’Connor H, Chambers P, Brooks P: Male systemic sclerosis and occupational silica exposure– a population-based study. Aust N Z J Med 2000;30:215–20. 4. Silman AJ, Hochberg MC: Occupational and environmental
9.
10. 11.
12.
influences on scleroderma. Rheum Dis Clin North Am 1996;22:737– 49. Neidhart M, Kuchen S, Distler O, et al: Increased serum levels of antibodies against human cytomegalovirus and prevalence of autoantibodies in systemic sclerosis. Arthritis Rheum 1999;42:389 – 92. Peacock JH: Vasodilatation in the human hand: observations on primary Raynaud’s disease and acrocyanosis of the upper extremities. Clin Sci 1958;17:575– 86. Jamieson GG, Ludbrook J, Wilson A: Cold hypersensitivity in Raynaud’s phenomenon. Circulation 1971;44:254 –264. Omote K, Kawamata M, Namiki A: Adverse effects of stellate ganglion block on Raynaud’s phenomenon associated with progressive systemic sclerosis. Anesth Analg 1993;77:1057– 60. D’Angelo R, Miller R: Pregnancy complicated by severe preeclampsia and thrombocytopenia in a patient with scleroderma. Anesth Analg 1997;85:839 – 41. Black CM, Stevens WM: Scleroderma. Rheum Dis Clin North Am 1989;15:193–212. Baethge BA, Wolf RE: Successful pregnancy with scleroderma renal disease and pulmonary hypertension in a patient using angiotensin converting enzyme inhibitors. Ann Rheum Dis 1989; 48:776 – 8. Thompson J, Conklin KA: Anesthetic management of a patient with scleroderma. Anesthesiology 1983;59:69 –71.
J. Clin. Anesth., vol. 14, September 2002
477