Systemic sclerosis, Scleroderma

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Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma. It is characterized by deposition of collagen in the skin and, less commonly, in the kidneys, heart, lungs & stomach. Female to male ratio is 4:1. The peak age of onset is between 30-50 years. Initial symptoms are nonspecific and include fatigue, vague musculoskeletal complaints, diffuse swelling of hands, and Raynaud phenomenon. Etiology and pathogenesis are unknown. Disease course is variable, but the condition rarely subsides spontaneously. There are two main subtypes of systemic sclerosis (SSc): limited cutaneous SSc and diffuse cutaneous SSc. The limited cutaneous form tends to have less severe internal organ involvement and a better prognosis, but these subjects still need to be followed closely for possible complications. Clinical course is determined by extent of vascular and fibrosing complications. Vascular involvement includes Raynaud phenomenon, ischemic digital ulcers, hypertensive crisis, and pulmonary arterial hypertension. Fibrosis can involve lungs, heart, and GI tract. Treatment is targeted on disease processes that are potentially reversible (e.g., active inflammation or vasoconstriction) and aims to minimize functional impairment of the patient. Scleroderma renal crisis is characterized by the onset of acute renal failure; abrupt onset of moderate-marked hypertension; a urinary sediment that is frequently normal, or reveals only mild proteinuria with few cells or casts; and a microangiopathic hemolytic anemia.


Laboratory Test Procedures:

cold fingers and toes
sequence of color changes in skin in response to cold or stress
decreased body hair
dry skin
areas of skin may get darker or lighter in color
blackening of the skin
skin thickening, stiffness, and tightness of fingers, hands, and forearm
small white lumps beneath the skin, sometimes oozing a white substance that looks like toothpaste
sores (ulcers) on the fingertips or toes
tight and mask-like skin on the face
joint aches
joint swelling
joint stiffness
pain, numbness, weakness or coldness in legs or arms
numbness or tingling in the feet
wrist pain
shortness of breath
bloating of the abdomen with fluid
difficulty swallowing
problems controlling stools

Rheumatoid Factor, titers
Extractable Nuclear Antigen Antibodies
Basophil %
Basophil Absolute
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