Scleroderma is a disease related with body autoimmune, connective tissue disease. Scleroderma induces inflammation and thickening of the skin. In scleroderma patients, their tissues are diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs. Below your will find the most common symptoms and signs of Systemic Sclerosis Scleroderma:
Onset. Raynaud’s phenomenon heralds the onset of the disease in at least 90% of patients. It may precede the other manifestations by many years. Scleroderma frequently begins with skin changes, but in one third of patients polyarthralgias and poly-arthritis are the first manifestations. Initial visceral involvement without skin changes occurs rarely.
Skin abnormalitiesThe clinical evolution of scleroderma occurs in three stages. In the edematous phase, symmetric nonpitting edema is present in the hands and, rarely, in the feet. The edema can progress to the forearms, arms, upper anterior chest, abdomen, back, and face. In the sclerotic phase, the skin is tight, smooth, and waxy and seems bound down to underlying structures. Skin folds and wrinkles disappear. The hands are involved in most patients, with painful, slowly healing ulcerations of the fingertips in half of those cases. The face appears stretched and mask-like, with thin lips and a “pinched†nose. Pigmentary changes and telangiectases are frequent at this stage.
The skin changes may stabilize for prolonged periods and then either progress to the third (atrophic) stage or soften and return to normal. It should be emphasized that not all patients pass through all the stages. Subcutaneous calcifications, usually in the fingertips (calcinosis circumscripta), occur more often in women than in men. The calcifications vary in size from tiny deposits to large masses and may develop over bony prominences throughout the body.
Joints and muscles. Articular complaints are very common and may begin at any time during the course of the disease. The arthralgias, stiffness, and frank arthritis seen in progressive systemic sclerosis may be difficult to distinguish from those of rheumatoid arthritis, particularly in the early stages of the epidemiology diseases. Involved joints include the metacarpophalangeals, proximal interphalangeals, wrists, elbows, knees, ankles, and small joints of the feet. Flexion contractures caused by changes in the skin or joints are common. Muscle involvement is usually mild but may be clinically indistinguishable from that of polymyositis, with muscle weakness, tenderness, and pain of proximal muscles of the upper and lower extremities.
Lungs. The lungs are frequently involved in progressive systemic sclerosis, either clinically or at autopsy. Interstitial fibrosis is the major pulmonary manifestation and may occur early in patients with truncal involvement. Pulmonary hypertension, which can best be detected by echocardiography, is more likely to be seen in limited scleroderma. Patients with diffuse pulmonary involvement have intimal proliferation of small and medium-size pulmonary arteries and arterioles and may have an intense bronchiolar epithelial proliferation.
Heart. Because of the frequency of pulmonary fibrosis, cor pulmonale is the most common cardiac finding. Myocardial fibrosis, leading to resistant left-sided heart failure, carries a poor prognosis. Cardiac arrhythmias and conduction disturbances are common manifestations of myocardial fibrosis. Pericarditis is usually asymptomatic and is found incidentally at autopsy. Although 40% of patients have pericardial effusion, tamponade is extremely rare.
Kidneys. Renal involvement is an uncommon but life-threatening development in patients with diffuse disease. Although renal insufficiency may follow an indolent course, it frequently presents as rapidly progressive oliguric renal failure with or without malignant hypertension. Marked changes are seen on renal arteriography in patients with scleroderma kidney. Irregular arterial narrowing, tortuousity of the interlobular arterioles, persistence of the arterial phase, and absence of a nephrogram phase are typical findings.
Gastrointestinal tract. The gastrointestinal tract is commonly affected. The esophagus is the most frequent site of involvement, with dysphagia or symptoms of reflux esophagitis occurring in 80% of patients. Gastric and small-bowel involvement presents with cramping, bloating, and diarrhea alternating with constipation. Hypomotility of the gastrointestinal tract with bacterial overgrowth may result in malabsorption. Colonic scleroderma is associated with chronic constipation. The barium enema may reveal large, wide-mouthed diverticula along the antimesenteric border of the colon.
