Wegener’s Granulomatosis Complicated by Pericardial Tamponade and Renal Failure

Prof. Saime Paydaş, Çukurova University, Faculty of Medicine, Dep. of Internal Medicine, TR-013 30 Adana (Turkey) Dear Sir, A 50-year-old woman was admitted to hospital on March 30, 1994, because of coughing and uremia. She had a history of coughing, white mucoid sputum of small volume, weakness, anorexia and pleuritic chest pain. There was nothing in her past and family history. Her physical examination revealed a temperature of 36 °C, pulse 80/min and regular, blood pressure 100/60 mm Hg, respiration 22/min. She was pale. Cardiac examination was normal. The breath sounds were heard less on the basal segments of the right lung. We could not find any organomegaly and LAP. Laboratory findings were as follows: hemoglobin 8.1 g/dl, WBC 9,600/mm3, platelets 147,000/mm3, ESR 120mm/h, BUN 145 mg/dl, creatinine 13.6mg/dl, Na 142 mEq/dl, K 6.1 mEq/dl, Cl 102mEq/dl, Ca 5.6 mg/dl, P 6.1 mg/dl, serum protein 6.6 g/ dl, albumin 2.7 g/dl; blood smear and bone marrow were normal, microscopic analysis of urine revealed 15-20 RBC, fine granular casts and 10-15 leukocytes/high-power field; ANF, RF and anti-DNA were negative, ANCA was not tested. C3 and C4 were normal; daily proteinuria 1 g. HBsAg and antiHCV were negative; computed tomography of the lung revealed patchy infiltrates and bilateral hilar lymphadenopathy, X-ray examination of paranasal sinuses revealed left maxillar sinusitis. In abdominal ultrasonog-raphy, the sizes of the left and right kidneys were 98 × 37 and 97 × 34 mm, respectively. Abdominal computed tomography revealed no further pathology. In renal biopsy, in the glomerular compartment, there were segmental changes, excessive polymorpho-nuclear leukocyte (PMNL) infiltration, nuclear debris, plasma cells and attachments on Bowman’s capsules; in the interstitial compartment, granulomas which revealed necrosis in the inner zones, some fibrosis, atrophy of some tubules, leukocyte and hyaline casts in tubule lumens were seen. In the vessel walls, thickening, necrosis that consisted of PMNL, plasma cells and lymphocytes and in some areas occlusion were seen. Immunohistochemically, IgM was localized in the glomerular tufts and along the capillaries, IgG and IgA were found positive in the glomerulus and vessel walls, locally. Amyloid was negative. Pathologic examination of pericardial biopsy was necrotizing vasculitis that suggested Wegener’s granulomatosis (WG). Epidermal hyperkeratosis and atrophy, collagenous degeneration in band form and mononuclear inflammatory cell infiltration around vessels in the upper dermis were observed in a dermal