Past names/synonyms: many reference sources refer to progressive systemic sclerosis as scleroderma (1).

Definition: PSS is a multisystem disorder characterized chiefly by fibrosis of blood vessels, skin, musculoskeletal system, and visceral organs (1).

Pathophysiology: pathogenesis of progressive systemic sclerosis involves microvascular insult and a secondary overproduction and accumulation of collagen, activation of immunologic mechanisms, and increased fibroblast activation and proliferation (1).

Variants: two major forms of this disease are limited cutaneous systemic sclerosis (formerly CREST syndrome [calcinosis, raynaud disease, esophageal motility disorders, sclerodactyly, and telangiectasis] (2)) and diffuse cutaneous systemic sclerosis (1).

Epidemiology (1):

• Prevalence: 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 occuring in the third to fifth decade of life.

Symptoms (1):

• Raynaud's phenomenon is usually the initial presenting symptom and commonly precedes skin changes by <=1 year in cases of diffuse, cutaneous progressive systemic sclerosis.
• Dermal thickening can result in flexion contractures, ischemic ulcerations, and resorption of distal phalanges.
• Esophageal mucosal thinning occurs, with fibrotic thickening in the remaining layers. There may be extensive mucosal ulceration and marked esophageal dilation.
• 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 progressive systemic sclerosis. 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.
• Pregancy is associated with disease progression in 50% of patients with progressive systemic sclerosis (3).

Measurements (1): anesthetic considerations:

• Raynaud's phenomenon -> peripheral vasoconstriction.
• Dermal thickening, calcifications, contractures -> peripheral iv difficulties:
• Skin tightening, microstomia, decreased neck flexibility -> difficult airway management.
• Telangiectasias -> oral or nasal bleeding.
• Esophageal dilatation, decreased lower esophageal sphincter tone -> aspiration: any information regarding dysphagia, regurgitation, weight loss, and digital pallor must be noted.
• Intestinal malabsorption -> decreased vitamin K-dependent clotting factors: for patients with signs of malnutrition or malabsorption, a complete blood count, prothrombin time, partial thromboplastin time, and albumin levels should be assessed.
• Restrictive pulmonary disease -> increased positive airway pressure, increased oxygen concentration, extubation delays: pulmonary involvement should be evaluated with an electrocardiogram, 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 <1 liter typically prove difficult to wean and extubate secondary to inadequate ventilatory effort.
• Myocardial fibrosis -> ventricular hypertrophy, diastolic dysfunction, conduction defects, coronary vasospasm: patients with cardiac involvement or potential pulmonary hypertension should have an electrocardiogram, chest radiograph, and evaluation of blood urea nitrogen and creatinine.
• Renal disease -> hypertension, decreased renal clearance: for patients with a history of renal disease, measurement of electrolytes, blood urea nitrogen, and creatinine levels are appropriate.

Treatment (1):

• 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.
• Anesthetic considerations:
• 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 lower esophageal sphincter tone renders the patient vulnerable to regurgitation and increases the risk of aspiration. Metoclopramide has the dual benefit of increasing gastric motility and increasing lower esophageal sphincter tone.
• Operating room temperature should be maintained above 21° C, and intravenous fluids should be warmed before administration so as to minimize peripheral vasoconstriction (4).
• 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 (4). Patients with progressive systemic sclerosis 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 (5).
• Increased positive airway pressure and higher than 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 vasoconstrictions.