Which of the following conditions is associated with Coomb’s positive hemolytic anaemia –
Correct Answer: Progressive systemic sclerosis
Description: The most impoant clinical complication of scleroderma involving the kidney is scleroderma renal crisis. Symptoms of scleroderma renal crisis are malignant hypeension (high blood pressure with evidence of acute organ damage), hyperreninemia (high renin levels), azotemia (kidney failure with accumulation of waste products in the blood) and microangiopathic hemolytic anemia (destruction of red blood cells showing Coombs positive ) Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small aeries. There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. The limited form affects areas below, but not above, the elbows and knees with or without involvement of the face. The diffuse form affects also the skin above the elbows and knees and can spread also to the torso. Visceral organs, including the kidneys, hea, lungs, and gastrointestinal tract can also be affected by the fibrotic process. Prognosis is determined by the form of the disease and the extent of visceral involvement. Patients with limited systemic sclerosis have a 10-year survival rate of 75%; less than 10% develop pulmonary aerial hypeension after 10 to 20 years. Patients with diffuse cutaneous systemic sclerosis have a 10-year survival rate of 55%. Death is most often caused by lung, hea, and kidney involvement. There is also a slight increase in the risk of cancer Ref Davidson 23rd edition pg 911
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