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Ann Saudi MedAnn Saudi MedAnnals of Saudi Medicine0256-49470975-4466King Faisal Specialist Hospital and Research Centre 10.5144/0256-4947.2007.215asm-3-215LettersRare manifestations of Wegener’s granulomatosis Rajai Morteza MDBaqiyatallah Medical University, Department of Internal Medicine, Tehran, Iran, m.rajai@gmail.comMay-Jun 2007 27 3 215 215 Copyright © 2007, Annals of Saudi Medicine2007This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
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To the Editor: Wegener’s granulomatosis (WG) is a necrotizing granulomatous small-vessel vasculitis.1 The frequency of cardiac involvement in WG varies from 6 to 12 percent.2 However, the occurrence of ascites and hemorrhagic effusion in WG were not reported in a MEDLINE search.

A 19-year-old man was referred to Baqyatollah hospital with a history of fever and polyarthritis for two weeks. The patient presented with weight loss, productive cough, and hemoptysis. His temperature was 39°C, heart rate 95/min and the respiratory rate was 28/min. A diffuse, coarse crackle was audible over both lungs. Severe joint inflammation was noted. His chest X-ray showed multiple pulmonary infiltration (Figure 1). He was treated for pulmonary abscess with clindamycin and ceftriaxone. The fever subsided gradually, but the polyarthritis, tachycardia, and tachypnea continued. Ultrasound study of the abdomen (Figure 2), showed mild splenomegaly and ascites. Moderate pericardial effusion was noticed by echocardiography. In the chest CT-scan, multiple isodense masses were found. Because of his severe clinical condition a high-dose steroid was given. Subsequently, marked hematuria, granular casturia and proteinuria developed.

C-ANCA (cytoplasmic antineutrophil cytoplasmic antibody) was positive. Pericardial effusion presented with a bloody appearance and the WBC count was 15000/mm3. Culture and PCR examinations were negative. No organism grew in ascites fluid culture. The WBC count in ascitic fluid was 2500/mm3, with a PMN value of 75%. A CT-guided lung biopsy was performed. Multiple foci of necrosis and infiltration of macrophages and neutrophils were seen. The diagnosis of WG was made, and combination therapy of high-dose steroid with cyclophosphamide was prescribed. In a few days, his general condition began to show signs of improvement. All clinical, laboratory, and imaging abnormalities also improved. He was discharged after 30 days.

WG is a disease manifested by necrotizing granuloma of the upper respiratory tract, lung, and renal involvement3 with a 5-year survival rate approaching 95%.2 Hemopericardium and ascites are a rare clinical picture not previously reported in WG.

Figure 1 Chest X-ray showing multiple pulmonary infiltrations.

Figure 2 Chest CT scans showing multiple isodense masses.
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REFERENCES
1 Walton EW   Giant cell granuloma of the respiratory tract (WG) Br Med J 1958 2 265 270 13560836 
2 Hoffman GS  Kerr GS  Leavitt RY   Wegener granulomatosis: an analysis of 158 patients Ann Intern Med 1992 116 488 498 1739240 
3 Stone JH   Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial Arthritis Rheum 2003 48 2299 12905485