wegener’s granulomatosis presenting as multiple kidney masses

2
plant associated thrombotic microangio- pathy: a case series. J Clin Apheresis. 1996; 11:176 –184. 4. Llamas P, Romero R, Cabrera R, et al. Man- agement of thrombotic microangiopathy following allogeneic transplantation: what is the role of plasma exchange. Bone Marrow Transplant. 1997;20:305–306. 5. Uchiba M, Okajima K, Murakami K, et al. Attenuation of endotoxin-induced pulmo- nary vascular injury by antithrombin III. Am J Physiol. 1996;270:L921–L930. 6. Harada N, Okajima K, Kushimoto S, et al. Antithrombin reduces ischemia/reperfu- sion injury of rat liver by increasing the he- patic level of prostacyclin. Blood. 1999;93: 157–164. 7. Uchiba M, Okajima K, Murakami K, et al. Effects of antithrombin III (ATIII) and TRP49-modified AT III on plasma level of 6-keto-PGF1 in rats. Thromb Res. 1995;80: 201–208. 8. Morris JD, Harris RE, Hashmi R, et al. An- tithrombin-III for the treatment of chemo- therapy-induced organ dysfunction follow- ing bone marrow transplantation. Bone Marrow Transplant. 1997;20:871–878. 9. Uchiba M, Okajima K, Murakami K. Effects of various doses of antithrombin III on en- dotoxin-induced endothelial cell injury and coagulation abnormalities in rats. Thromb Res. 1998;89:233–241. WEGENER’S GRANULOMATOSIS PRESENTING AS MULTIPLE KIDNEY MASSES To the Editor: Wegener’s granulomatosis is a ne- crotizing granulomatous vasculitis that typically involves the upper and lower respiratory tracts and the kid- neys. There are many reports of We- gener’s granulomatosis presenting as a single tumor-like lesion, but in only 2 cases was the lesion isolated to the kidneys (1). We report a case of We- gener’s granulomatosis that pre- sented as multiple kidney masses without extrarenal involvement. A 22-year-old man with a history of longstanding substance abuse was admitted 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 2.6 mg/dL, hematuria, and 13,671 mg of protein on 24-hour measurement. Complement levels were normal. Tests for antinuclear antibodies, the human immunodeficiency virus (HIV), rapid plasma reagin, and cryoglobulins yielded negative re- sults. Urinary cytology revealed no abnormalities. Serum immunoelec- trophoresis was consistent with a chronic inflammatory response. Re- sults of blood and urine cultures were negative. Transesophageal echocardi- ography showed no vegetation. Cyto- plasmic antineutrophil autoantibod- ies (C-ANCA, anti-PR3) were posi- tive at more than 11 enzyme-linked immunosorbent assay (ELISA) units. A renal ultrasonogram revealed large bilateral echogenic masses. Magnetic resonance imaging (MRI) with gado- linium showed many rounded, lobu- lar areas of increased signal intensity dispersed throughout the paren- chyma, with distortion of the renal calyces bilaterally (Figure). Biopsy specimens of the masses demon- strated a necrotizing vasculitis and crescentic glomerulonephritis. In view of the positive C-ANCA test and histopathologic findings, Wegener’s granulomatosis was diagnosed. The patient refused therapy, and hemodi- alysis was initiated for progressive azotemia. After 9 months of follow- up, the patient remained free of extra- renal involvement. Vasculitis may produce isolated mass lesions that mimic neoplasm. Such lesions have been described in patients with giant cell arteritis, poly- arteritis nodosa, granulomatous an- giitis, and hypersensitivity angiitis. However, this presentation has been described most frequently in Wegen- er’s granulomatosis. The breast and the kidney are the most common sites, but lesions have been described in the pancreas, eustachian tube, pleura, gingiva, heart, and orbit (1). Despite an extensive inflammatory response in our patient’s kidney, no extrarenal disease was present to fa- cilitate diagnosis. Awareness of the potential for Wegener’s granuloma- tosis to present as multiple as well as single mass lesions may be important in the diagnostic evaluation of such radiographic 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 demonstrates bilateral hyerintense renal masses. Letters to the Editor 82 January 2002 THE AMERICAN JOURNAL OF MEDICINE Volume 112

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Page 1: Wegener’s granulomatosis presenting as multiple kidney masses

plant associated thrombotic microangio-pathy: a case series. J Clin Apheresis. 1996;11:176 –184.

4. Llamas P, Romero R, Cabrera R, et al. Man-agement of thrombotic microangiopathyfollowing allogeneic transplantation: whatis the role of plasma exchange. Bone MarrowTransplant. 1997;20:305–306.

5. Uchiba M, Okajima K, Murakami K, et al.Attenuation of endotoxin-induced pulmo-nary vascular injury by antithrombin III.Am J Physiol. 1996;270:L921–L930.

6. Harada N, Okajima K, Kushimoto S, et al.Antithrombin reduces ischemia/reperfu-sion injury of rat liver by increasing the he-patic level of prostacyclin. Blood. 1999;93:157–164.

7. Uchiba M, Okajima K, Murakami K, et al.Effects of antithrombin III (ATIII) andTRP49-modified AT III on plasma level of6-keto-PGF1� in rats. Thromb Res. 1995;80:201–208.

8. Morris JD, Harris RE, Hashmi R, et al. An-tithrombin-III for the treatment of chemo-therapy-induced organ dysfunction follow-ing bone marrow transplantation. BoneMarrow Transplant. 1997;20:871–878.

9. Uchiba M, Okajima K, Murakami K. Effectsof various doses of antithrombin III on en-dotoxin-induced endothelial cell injury andcoagulation abnormalities in rats. ThrombRes. 1998;89:233–241.

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

Page 2: Wegener’s granulomatosis presenting as multiple kidney masses

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

January 2002 THE AMERICAN JOURNAL OF MEDICINE� Volume 112 83