All of the following are features of scleroderma except -
Ans. is `d' i.e., Calcification in long bones Clinical manifestations of scleroderma (systemic sclerosis) Skin o The distinctive feature of scleroderma are striking cutaneous changes, notably skin thickning. o A majority of the patients have diffuse sclerotic atrophy of the skin which usually begins in the fingers and distal regions of upper extremities and extends proximally to involve upper arm, shoulders, neck and face. o In advanced stage, the fingers take on a tappered, clawlike appearance with limitation of motion in the joints and sometimes the tips of the fingers undergo autoamputation. o Face becomes a drawn mask. o Nail fold capillaries are distoed early in the disease which shows abnormalities in microvasculature. Alimentry tract o Affected in 90% of the patients. o Most severe involvement is in esophagus. o Lower third esophageal involvement develops a rubberhose inflexibility that causes gastroesophageal reflex. o Esophagial fibrosis causes dysphagea. o Abdominal pain, intestinal obstruction, malabosorption (due to loss of villi) with weight loss, and anemia reflects intestinal involvement. Raynaud's phenomenon o It is episodic digital ischemia due to vasoconstriction of aeries and aerioles of the extremities, precipitated by cold. o It is usually the first manifestation of disease. Musculoskeletal symptoms o Inflammation of synovium (synovitis) with hyperophy and hyperplasia of synol tissues. o Joint destruction is not common. o Inflammatory myositis may develop in 10% of patients. Kidney o The most prominant changes are in vessel walls and cause appearance similar to those of malignant hypeension, i.e. onion skin concentric proliferation of smooth muscles with thickening of intima, and fibrinoid necrosis. o Hypeension may occur due to kidney involvement. o Most common cause of death is development of malignant hypeension with subsequent renal failure. Lungs o Pulmonary hypeension. o Interstitial fibrosis. Hea o Pericardial effusion / Pericarditis o Myocardial fibrosis CREST syndrome (with limited systemic sclerosis).
Dysphagia
Raynaud's phenomenon
Skin contracture
Calcification in long bones