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A 45-year-old man presents with a 3-day history of hemoptysis. He has had a cough and has also been complaining of sinus congestion and drainage. Furthermore, he has noted blood in the urine. He has no other medical problems and takes no medications. An arteriole biopsy indicates necrotizing inflammation. Urine sediment shows red cell casts and dysmorphic red cells. Wegener granulomatosis is suspected, and steroids are started. Presence of which of the following entities will confirm the diagnosis?
A. An elevated angiotensin converting enzyme (ACE)
B. Antiglomerular basement membrane antibodies (anti-GBM)
C. Antineutrophilic cytoplasmic antibodies (ANCA)
D. Eosinophilia
E. X-bodies
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C????. WHAT R X-BODIES ANY ONE PLZZZZ...........
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The correct answer is C. Wegener granulomatosis is identified by the classic clinical triad of upper and lower respiratory involvement, supported by a positive antineutrophilic cytoplasmic antibody (ANCA) test. The pathologic lesion is an angiitis of small vessels with characteristic tissue necrosis surrounded by mononuclear inflammatory cells, forming noncaseating granulomas. In the lung, this process results in excavation and destruction of the lung parenchyma. The renal lesion is a focal glomerulonephritis that can progress to renal failure.
Sarcoidosis is a multisystem disease that involves the lung in 90% of cases. Cough is nonproductive, and hemoptysis is rare. An elevated angiotensin converting enzyme (ACE) level (choice A) has been associated with this illness.
Goodpasture syndrome is a progressive autoimmune disease of the lungs and kidneys that produces intra-alveolar hemorrhage and glomerulonephritis. It is caused by an antiglomerular basement membrane (anti-GBM) antibody (choice B), usually IgG, that reacts with glomerular and alveolar basement membranes.
Systemic illnesses such as scleroderma are associated with eosinophilia (choice D). This may lead to lung involvement and ultimately cause pulmonary hypertension.
Histiocytosis X is a generic term for a group of systemic disorders characterized by various degrees of fibrosis with focal infiltrations of tissue by nonmalignant histiocytes and eosinophils. Eosinophilic granuloma of the lung is localized and may cause dyspnea and a cough. Pathology shows cytoplasmic inclusions, the so-called X bodies (choice E).
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actually guys the treatment of choice is cyclophosphamide and +or - steroids
