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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 6  |  Page : 1118-1121
Focal segmental glomerulosclerosis as the sole renal lesion in Wegener's granulomatosis


Department of Internal Medicine, Cairo University, Egypt

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Date of Web Publication4-Nov-2010
 

   Abstract 

Wegener's granulomatosis usually presents with focal necrotizing glomerulonephritis with crescents. We present here a 45-year old man who was treated for tuberculosis and later pre­sented with bilateral ankle swelling. His serum creatinine was 2.4 mg/dL and urine analysis revealed hematuria and proteinuria. His 24-hour urine protein excretion was 1.9 g. Anti neutrophil cytoplasmic antibodies (ANCA) test was positive with cytoplasmic florescence pattern. Renal biopsy revealed focal segmental sclerosis with no active vasculitis and lung biopsy revealed extensive breakdown with cavitations and scattered granulomas.

How to cite this article:
Ibrahim S. Focal segmental glomerulosclerosis as the sole renal lesion in Wegener's granulomatosis. Saudi J Kidney Dis Transpl 2010;21:1118-21

How to cite this URL:
Ibrahim S. Focal segmental glomerulosclerosis as the sole renal lesion in Wegener's granulomatosis. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2019 Jul 19];21:1118-21. Available from: http://www.sjkdt.org/text.asp?2010/21/6/1118/72302

   Introduction Top


Wegener's granulomatosis (WG) is a syste­mic inflammatory disease characterized by the presence of granulomas, necrosis, and/or vas­culitis. [1] A focal, segmental, necrotizing, cres­centic glomerulonephritis (GN) is the classic renal lesion in WG. [1] It is typically associated with scant or no immunocomplex deposits (pauci-immune). The most common initial fea­tures are ear, nose and throat symptoms. Nasal mucosa inflammation leads to obstruction, crus­ting, pain, epistaxsis, and nasal discharge. [2] Nasal chondritis may result in collapse of nasal bridge (saddle nose). [3] Lung involvement may include focal or diffuse infiltrates, and nodules that may cavitate. [4]

The diagnosis of WG is based on a combina­tion of clinical, laboratory, and pathologic features. If a typical clinical picture is associa­ted with a positive anti neutrophil cytoplasmic antibodies (ANCA) with specificity for pro­teinase 3 (PR3), the diagnosis of WG can be presumed. [4] The American College of Rheuma­tology (ACR) proposed specific criteria for the classification of WG. [5] The four criteria are:

  1. an abnormal urinary sediment (red cell casts > 5 red blood cells per high power field (RBCs/HPF),
  2. abnormal findings on chest radiograph (nodules, cavities, or infiltrates),
  3. oral ulcers or nasal discharge, and
  4. granulomatous inflammation on biopsy.


The presence of two or more of these criteria is associated with a sensitivity of 88.2% and a specificity of 92% in distinguishing WG from other forms of vasculitis. The presence of red blood cell casts in the urine sediment can be used as a reliable surrogate marker for the diag­nosis of glomerulonephritis and may eliminate the need for renal biopsy in some patients. [6] We describe here a case of WG with history of per­sistent pulmonary infiltrates. Renal biopsy re­vealed focal segmental glomerulosclerosis with no necrotizing or vasculitic lesions.


   Case Report Top


A 45-year-old man presented to the outpa­tient clinic with a 2-month history of bilateral ankle swelling. He was treated for pulmonary tuberculosis with a history of prolonged fever, cough expectoration and chest X-ray cavitary lung lesions. He also complained of anorexia, loss of 10 kg of weight over the last year and recurrent attacks of epistaxsis. He had visited many ENT clinics and was prescribed some nasal drops. He noticed change in the appea­rance of his nose with loss of smell.

On physical examination, he looked cachec­tic, pale and unwell. His body mass index (BMI) was 17 kg/m 2 , BP was 150/98 mmHg, pulse rate was 102/minute and he was afebrile. He had saddle nose, digital clubbing and no lym­phadenopathy. Chest examination revealed sca­ttered crepitations and bronchial breathing over mid lung zones and bases posteriorly. Cardiac examination was unremarkable. He had hepato­megaly, no ascites and bilateral ankle edema. There was no joint affection and neurological examination was normal except for loss of sense of smell and bilateral conductive deaf­ness.

Initial laboratory workup revealed moderate normochromic, normocytic anemia, elevated erythrocyte sedimentation rate, serum create­nine level of 2.4 mg/dL and blood urea nitro­gen (BUN) level of 50 mg/dL. Liver function tests were normal except for low serum albu­min (2.2 mg/dL). Urine analysis revealed he­maturia (30-40 HPF), pyuria (13-28 HPF) and albuminuria (+3). His 24-hour urine protein excretion was 1.9 g. Sputum examination re­vealed Pseudomonas aeruginosa. Computed tomography (CT) chest examinations revealed cavitary lesion [Figure 1] and sputum analysis for acid-fast bacilli (AFB) was repeatedly nega­tive. U/S abdominal examination revealed nor­mal sized with grade II parenchymal changes in the kidneys. ANCA test was positive with cytoplasmic florescence pattern. Renal and lung biopsies were scheduled soon after admission.
Figure 1 :CT examination of the chest showed cavitary lesion.

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WG was the presumptive diagnosis and was confirmed by ANCA results.

Renal and lung biopsy findings

Lung biopsy showed extensive breaking down with cavitations admixed with fibrinoid mate­rial, excess neutrophils and few esinophils in­volving lung parenchyma and bronchi with scattered granulomas.

Renal biopsy examination revealed the fol­lowing. Three glomeruli were seen, none was obsolescent. The tufts showed focal segmental glomerulosclerosis and one of them showed periglomerular fibrosis. The tubules showed cloudy swelling and some contained hyaline casts. The interstitium showed fibrosis and lym­phocytic infiltration with focal perivascular infiltrate and no active vasculitis [Figure 2].
Figure 2 :(A) Focal segmental glomerulosclerosis with capsular adhesion; (B) interstitial fibrosis and granulomas; (C) periglomerular fibrosis.

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   Discussion Top


The renal biopsy in our case lacked the pre­sence of necrotizing lesions commonly repor­ted in active WG. Crescentic GN was found in nearly 88% of 96 patients of WG [Figure 3]. [7] Segmental and global glomerulosclerosis were the sole lesions in some patients with WG in a clinicopathological analysis conducted by Hauer and colleagues in 2002. [7] Dana and colleagues more recently reported a case of WG with focal segmental sclerosis in the absence of nec­rotizing lesions. [8] Healing process of active glomerular lesions may result in glomerular scarring and interstitial fibrosis as seen in the present case. In fact, autopsy study of 31 pa­tients with ANCA positive vasculitis found ad­vanced sclerosing glomerular lesions in 69% of autopsies, co-existing with necrotizing glo­merular lesions. [9] Sclerotic lesions were more often associated with myelo-peroxidase-ANCA, while necrotizing lesions predominated in pa­tients with ANCA of proteinase 3 specificity. [9]
Figure 3 :Clinicopathological analysis of renal lesions in WG conducted by Hauer and colleagues in 2002.

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Our patient was prescribed prednisolone 50 mg/day and cyclophosphamide 100 mg daily orally for one month plus antibiotics according to the results of sputum culture and sensitivity test. After 3 weeks, he had no hemoptysis or arthralgias. He felt better and gained 2 kg as his appetite improved. Hemoglobin level in­creased to 10.2 g/dL, and renal function tests improved with a decrease in serum creatinine from 2.4 to 1.6 mg/dL. Urine analysis showed + albumin with 3-5 RBCs/HPF and there was some resolution in the cavitary lesions in the lungs. He was discharged on oral dose of 50 mg prednisolone every other day and 100 mg/ day cyclophosphamide and was given an out­patient appointment in one month for follow­up.

In conclusion, we have reported here a case of WG with focal segmental glomeruloscle­rosis as the sole lesion in renal biopsy in the absence of necrotizing lesions. The patient was successfully treated with cyclophosphamide and prednisolone with improvement of his chest and renal symptoms.

 
   References Top

1.Bacon P. The spectrum of Wegener's granulo­matosis and disease relapse. N Engl J Med 2005;352(4):330-332.  Back to cited text no. 1
    
2.Wodecki M, Brzosko I, Przepiera-Bedzak H, et al. Ear involvement in Wegener's Granulo­matosis. Waid Lek 2007;60(11-12):574-7.  Back to cited text no. 2
    
3.Pagnoux C. Wegener's granulomatosis and mic­roscopic polyangiitis. Rev Prat 2008;58(5): 522-32.  Back to cited text no. 3
    
4.Cordier JF. Pulmonary manifestations of the vasculitides. Rev Prat 2008;58(5):492-8.  Back to cited text no. 4
    
5.Leavitt R, Fauci A, Bloch D, et al. The Ame­rican College of Rheumatology 1990 criteria for the classification of Wegener's Granulo matosis. Arthritis Rheum 1990;33:1101-7.  Back to cited text no. 5
    
6.Isaac J, Clayton F, Rohr R, Herrera GA. ANCA-associated necrotizing/granulomatous glomerulonephritis; importance of renal biopsy. Rev Invest Clin 2001;53(1):88-90  Back to cited text no. 6
    
7.Hauer HA, Bajema IM, Van Houwelingen HC, et al. Determinants of outcome in ANCA­associated glomerulonephritis: A prospective clinico-histopathological analysis of 96 patients. Kidney Int 2002;62(5):1732-42.  Back to cited text no. 7
    
8.Danda D, Mathew AJ, Mathew J. Wegener's granulomatosis: A rare presentation. Clin Rheumatol 2008;27(2):273-5.  Back to cited text no. 8
    
9.Rott T, Vizjak A, Koselj M. ANCA-associated vasculitis-an autopsy study. Wien Klin Wochenschr 2000;112(15-16):671-5.  Back to cited text no. 9
    

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Correspondence Address:
Salwa Ibrahim
Department of Internal Medicine, Cairo University
Egypt
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PMID: 21060184

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    Abstract
    Introduction
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