Pathology - Systemic Sclerosis
Although the etiology is uncertain, anomalies in immune system activation have been proposed. most frequently affects women in the 30 to 50 age range. General pathology: The skin, heart, lungs, joints, GI tract (particularly the esophagus), and kidneys are the organs most frequently affected by fibrosis and collagen deposition. Skin: Possible subcutaneous calcifications; eventual dermal fibrosis; perivascular lymphocytic infiltration with edema. GI tract: loss of villi and microvilli in the small bowel; fibrosis and atrophy of the muscles. Joints: Synovial tissue hypertrophy accompanied by synovium inflammation. Kidneys: Vascular fibrinoid necrosis; intimal thickening with collagenous deposition in vessel walls. Lungs: Vasospasm-induced pulmonary hypertension; interstitial fibrosis. Heart: myocardial fibrosis and pericarditis. gastroesophageal reflux disease, dysphagia, pulmonary fibrosis, pulmonary hypertension, Raynaud phenomenon, nephritic syndrome, arrhythmias, arthralgias with joint contractures, hypertension, and extensive skin thickening (scleroderma). Positive ANA and anti-Scl-70 antibodies were found in the lab. Systemic sclerosis has no FDA-approved treatments. Variable outcomes have been observed in small studies assessing the effects of D-penicillamine, rnycophenolate mofetil, cyclophosphamide, and bone marrow transplant. The term "CREST syndrome" describes a form of systemic sclerosis that mostly affects the face and hands. It is typically associated with telangiectasia, esophageal dysmotility (dysphagia), calcinosis, Raynaud phenomenon, and sclerodactyly (hands that resemble claws). An anticentromere antibody test result of positive is frequently linked to CREST syndrome.
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