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Isolated bladder vasculitis: a rare presentation of Wegener's granulomatosis.

Introduction

Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis mainly involving the lungs and kidney. (1,2) Urogenital involvement of WG is mostly prostate and kidneys; it rarely involves the urinary bladder. (2,3) Bladder involvement of WG can mimick bladder neoplasia and should be included in the differential diagnosis of hematuria. We present a case of WG initially presenting as isolated bladder vasculitis (IBV) which has never been reported to the best of our knowledge.

Case report

A seventy year old Caucasian female was admitted with hemoptysis, fatigue and worsening anemia. Her past medical history was significant for hypertension, hyperlipidemia, gout and anemia of chronic disease. Eight months prior she had been admitted with chest pain. Cardiac enzymes, electrocardiogram, and chest x-ray were normal. A normocytic, normochromic anemia of chronic disease was noted with a hemoglobin of 10 g/dL. Over the next two months, her hemoglobin declined to 8.3 g/dL. A urine analysis showed microhematuria. Due to persistent microhematuria, cystoscopy was performed and revealed very small papillary lesions in the bladder and biopsy showed focal vasculitis and perivasculitis of a small artery of the lamina with no evidence of malignancy (Figure 1). One month later she developed a cough with hemoptysis for two weeks. Hemoglobin was found to be 8.0 g/dL. Chest x-ray showed a faint area of consolidation at the right lung base. A CT scan showed the area of consolidation in the right lower lobe consistent with pneumonia and poorly defined areas of increased density in the right middle and left lower lobes.

[FIGURE 1 OMITTED]

Over the ensuing months her cough and hemoptysis never completely resolved. She felt increasingly fatigued and dyspneic. A chest CT scan was repeated during present hospitalization which showed progression of the densities in both lungs. She also underwent bronchoscopy, which showed a diffuse alveolar hemorrhage. Her erythrocyte sedimentation rate was 99. She was found to be positive for antineutrophil cytoplasmic antibodies (C-ANCA). Based on the above findings she was diagnosed with Wegener's granulomatosis. She was started on corticosteroids and cyclophosphamide. She had an excellent recovery of her symptoms. Follow up CT scan of chest showed resolution of the previously described opacity.

Discussion

Wegener's granulomatosis is a systemic disorder but it can present in a limited form. (1) Involvement of the bladder in the systemic vasculitis has been rarely reported with only two cases reported so far which were initially diagnosed as bladder hamartoma. (4) Both cases had multiple recurrences of 'tumor like growth' in the bladder with no systemic involvement without immunosuppressive therapy. On further review they were diagnosed with polyarteritis nodosa like vasculitis and small vessel vasculitis respectively with good therapeutic response on appropriate treatment. This underscores the importance of including bladder vasculitis in the differential diagnosis of neoplasia as it can present as hematuria, irritative voiding symptoms, urgency and incontinence along with constitutional symptoms such as fatigue, malaise, weight loss or intermittent sweats. (5) WG may initially present with granulomas of the affected tissues and subsequently vasculitis develops as a complication of the granulomatosis. Recognizing isolated bladder involvement in WG is very important because its early treatment may help in the preservation of bladder function and reduce the need for surgical interventions. (5,6)

Conclusion

WG initially presents with granulomas of the affected tissues, later complicated by systemic vasculitis. Early diagnosis and treatment is important because it involves less toxic regimens than systemic vasculitis which helps prevent many complications. Surgical treatment should reserved for severe impairment of bladder function. (5,6)

References

(1.) Davenport A, Downey SE, Goel S, Maciver AG. Wegener's granulomatosis involving the urogenital tract. Br J Urol. 1996 Sep; 78 (3): 354 -7.

(2.) Huong DL, Papo T, Piette JC, Wechsler B, Bletry O, Richard F, Valcke JC, Godeau P. Urogenital manifestations of Wegener granulomatosis. Medicine (Baltimore). 1995 May; 74(3):152-61.

(3.) Cameron JS. Renal vasculitis: microscopic polyarteritis and Wegener's granuloma. Contrib Nephrol. 1991;94:38-46.

(4.) Fischer AH, Wallace VL, Keane TE, Clarke HS. Two cases of vasculitis of the urinary bladder: diagnostic and pathogenetic considerations. Arch Pathol Lab Med. 1998 Oct; 122(10):903-6.

(5.) Katz DJ, Sengupta S, Snow RM. Isolated vasculitis of the bladder. Urology. 2005 Apr;65(4):797.

(6.) Fienberg R. A morphologic and immunohistologic study of the evolution of the necrotizing palisading granuloma of pathergic (Wegener's) granulomatosis. Sem Resp Med 1989;10:126-132.

Deepak Hooda, MD, MPH

Resident, Department of Medicine, WVU Hospitals, Morgantown

Jeremy Parsons, MD

Department of Pathology, WVU Hospitals, Morgantown

Shveta Hooda, MD

Department of Pathology, WVU Hospitals, Morgantown

Michelle Nuss, MD, FACP

Vice President, Medical Staff Affairs, Interim Associate Dean for Hospital Affairs, Associate Professor of Medicine & Psychiatry, Program Director, Internal Medicine, WVU Hospitals, Morgantown

Richard Layne, MD

Section Chief, Dept. of Medicine, WVU Hospitals, Morgantown
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Article Details
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Title Annotation:Scientific Article
Author:Hooda, Deepak; Parsons, Jeremy; Hooda, Shveta; Nuss, Michelle; Layne, Richard
Publication:West Virginia Medical Journal
Article Type:Case study
Date:Jan 1, 2011
Words:797
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