wegener’s granulomatosis presenting as multiple kidney masses
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WEGENER’SGRANULOMATOSISPRESENTING AS MULTIPLEKIDNEY MASSES
To the Editor:Wegener’s granulomatosis is a ne-
crotizing granulomatous vasculitisthat typically involves the upper andlower respiratory tracts and the kid-neys. There are many reports of We-gener’s granulomatosis presenting asa single tumor-like lesion, but in only2 cases was the lesion isolated to thekidneys (1). We report a case of We-gener’s granulomatosis that pre-sented as multiple kidney masseswithout extrarenal involvement.
A 22-year-old man with a historyof longstanding substance abuse wasadmitted for voluntary detoxifica-tion. He complained of recurrent fe-vers but denied any other symptoms.Initial laboratory investigation re-
vealed a serum creatinine level of 2.6mg/dL, hematuria, and 13,671 mg ofprotein on 24-hour measurement.Complement levels were normal.Tests for antinuclear antibodies, thehuman immunodeficiency virus(HIV), rapid plasma reagin, andcryoglobulins yielded negative re-sults. Urinary cytology revealed noabnormalities. Serum immunoelec-trophoresis was consistent with achronic inflammatory response. Re-sults of blood and urine cultures werenegative. Transesophageal echocardi-ography showed no vegetation. Cyto-plasmic antineutrophil autoantibod-ies (C-ANCA, anti-PR3) were posi-tive at more than 11 enzyme-linkedimmunosorbent assay (ELISA) units.A renal ultrasonogram revealed largebilateral echogenic masses. Magneticresonance imaging (MRI) with gado-linium showed many rounded, lobu-lar areas of increased signal intensitydispersed throughout the paren-chyma, with distortion of the renalcalyces bilaterally (Figure). Biopsyspecimens of the masses demon-strated a necrotizing vasculitis andcrescentic glomerulonephritis. Inview of the positive C-ANCA test andhistopathologic findings, Wegener’s
granulomatosis was diagnosed. Thepatient refused therapy, and hemodi-alysis was initiated for progressiveazotemia. After 9 months of follow-up, the patient remained free of extra-renal involvement.
Vasculitis may produce isolatedmass lesions that mimic neoplasm.Such lesions have been described inpatients with giant cell arteritis, poly-arteritis nodosa, granulomatous an-giitis, and hypersensitivity angiitis.However, this presentation has beendescribed most frequently in Wegen-er’s granulomatosis. The breast andthe kidney are the most commonsites, but lesions have been describedin the pancreas, eustachian tube,pleura, gingiva, heart, and orbit (1).
Despite an extensive inflammatoryresponse in our patient’s kidney, noextrarenal disease was present to fa-cilitate diagnosis. Awareness of thepotential for Wegener’s granuloma-tosis to present as multiple as well assingle mass lesions may be importantin the diagnostic evaluation of suchradiographic findings. At this time,noninvasive measures cannot distin-guish the tumor-like masses of vascu-litis from true neoplasm. A case-con-trol study has supported a greater
Figure. Coronal T2-weighted fast spin echo magnetic resonance image demonstratesbilateral hyerintense renal masses.
Letters to the Editor
82 January 2002 THE AMERICAN JOURNAL OF MEDICINE� Volume 112
than expected co-occurrence of We-gener’s granulomatosis and renal cellcarcinoma (2). Therefore, until dis-criminating noninvasive techniquesare developed, it will be necessary toobtain biopsies of lesions to confirmthe nature of the process.
Ajoy Kapoor, MD
Ruthia A. Balfour-Dorsey, MD
David L. George, MD
Department of Medicine
Reading Hospital and Medical Center
West Reading, Pennsylvania
1. Revital K, Yechezkel S, Hanan G. Systemicvasculitis presenting as a tumor-like lesion:four case reports and an analysis of 79 re-ported cases. Medicine. 2000;79:349 –359.
2. Tatsis E, Reinhold-Keller E, Steindorf K, etal. Wegener’s granulomatosis associatedwith renal cell carcinoma. Arthritis Rheum.1999;42:751–756.
Letters to the Editor
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