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   Abstract
  Introduction
  Case Report
  Discussion
   References
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  Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 24  |  Issue : 1  |  Page : 57-59
 

Membranous nephropathy superimposed on Churg-Strauss syndrome


1 Department of Nephrology, Nizam's Institute of Medical Sciences, Hyderabad, India
2 Department of Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, India

Date of Web Publication16-Jan-2014

Correspondence Address:
R Ram
Department of Nephrology, NIMS, Hyderabad
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-4065.125132

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  Abstract 

The association of membranous nephropathy with Churg-Strauss syndrome is not widely reported. We present a patient with myeloperioxidase-perinuclear antineutrophilic cytoplasmic antibody (MPO-pANCA)-positive necrotizing and crescentic glomerulonephritis who later developed membranous nephropathy.


Keywords: Churg-Strauss syndrome, membranous nephropathy, MPO, pANCA


How to cite this article:
Ram R, Rao C S, Swarnalatha G, Sireesha K, Madhuri H R, Rajasekhar L, Dakshinamurty K V. Membranous nephropathy superimposed on Churg-Strauss syndrome. Indian J Nephrol 2014;24:57-9

How to cite this URL:
Ram R, Rao C S, Swarnalatha G, Sireesha K, Madhuri H R, Rajasekhar L, Dakshinamurty K V. Membranous nephropathy superimposed on Churg-Strauss syndrome. Indian J Nephrol [serial online] 2014 [cited 2019 Nov 18];24:57-9. Available from: http://www.indianjnephrol.org/text.asp?2014/24/1/57/125132



  Introduction Top


The association of membranous nephropathy with Churg-Strauss syndrome is not widely reported. We report a patient who was first diagnosed as having Churg-Strauss syndrome and after 6 years, upon evaluation for a new onset nephrotic syndrome, the patient was found to have membranous nephropathy.


  Case Report Top


A 37-year-old man was treated for Churg-Strauss syndrome 6 years back. It was diagnosed when he had history of problems such as breathlessness, wheezing, sticky sputum, weight loss, and myalgias. He also had history of cough and expectoration. He had these symptoms for 10 years before that presentation. He had joint pains, particularly metacarpophalangeal, interphalangeal, and wrist joints. He complained of subcutaneous nodules over upper and lower limbs. He also had rhinorrhea and later he was found to have nasal polyp. He had no symptoms such as abdominal pain, diarrhea, gastrointestinal bleeding, weakness of limbs and symptoms related to stroke, ophthalmologic involvement, and cardiac failure. His previous investigations are reported in [Table 1].
Table 1: Investigations

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At present, he complained of facial puffiness which gradually progressed to entire body. There was no antecedent history of fever or skin disease. There was history of increased frothiness of urine. There was no history of oliguria and hematuria. He also complained of breathlessness, wheezing, cough, and expectoration, particularly in the morning. His blood pressure was 160/90 mm of Hg. The present investigations are reported in [Table 1].

On light microscopy, kidney biopsy revealed the following: 15 glomeruli, 3 sclerosed, and 5 showed focal necrosis and cellular crescents. Glomeruli showed diffuse thickening of glomerular basement membrane [Figure 1]. Spikes were present. Tubules, vessels, and interstitium were unremarkable.
Figure 1: Glomerulus showing thickening of glomerular basement membrane with a cellular crescent (PAS Periodic acid stain, × 100)

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Immunofluorescence assay revealed: IgG 3+, IgM 1+, κ 1+, and λ 1 + in glomeruli. Electron microscopy was not performed. HbsAg Hepatitis B surface antigen and anti-HCV anti- hepatitis C virus antibodies were negative. Human immunodeficiency virus was non-reactive. Cytoplasmic antineutrophilic cytoplasmic antibody cANCA, anti nuclear antibody ANA, and anti double stranded DNA antibody anti-dsDNA were negative. Complements were within normal limits.

At first presentation, he was treated with methylprednisolone 15 mg/kg/d intravenous for 3 days followed by oral prednisolone 0.5 mg/kg/d for 3 months and was tapered to 10 mg/day by 6 months. He was continued on that dose, till the present complaints.

At present, he received intravenous methylprednisolone 15 mg/kg/d intravenous for 3 days followed by intravenous cyclophosphamide 500 mg and oral prednisolone 0.5 mg/kg/d. He also received monthly doses of intravenous cyclophosphamide 500 mg for 6 months. At the end of 6 months, azathioprine (2 mg/kg/d) was started and angiotensin-converting inhibitors and angiotensin receptor blockers were continued. After 18 months of follow-up, the proteinuria was 0.2 g/day, RBCs in urine were absent, and serum creatinine was 1.2 mg/dl.


  Discussion Top


At first presentation, the patient had respiratory symptoms, joint pains, subcutaneous nodules, nasal polyp, raised absolute eosinophil count, and MPO-pANCA positive. All pointed to the diagnosis of Churg-Strauss syndrome. At second presentation, nephrotic syndrome and the subsequent kidney biopsy suggested the diagnosis of membranous nephropathy. The RBC casts in urine, renal impairment, raised absolute eosinophil count, cellular crescent on kidney biopsy, and PANCA-MPO positive suggested the diagnosis of Churg-Strauss syndrome. In English literature, not many reports of membranous nephropathy associated with Churg-Strauss syndrome were available, and there was none from India. In the present patient at second presentation, it was concurrent occurrence of necrotizing crescentic glomerulonephritis superimposed on membranous nephropathy. In the largest series [1] of 14 patients with both pANCA and cANCA associated necrotizing and crescentic glomerulonephritis and membranous nephropathy, MPO-pANCA positivity was present in four patients and pANCA was positive in three more patients. End stage renal disease (ESRD) was the outcome in three of the seven patients. Hanamura et al, [2] reported six patients of MPO positivity on ELISA (ANCA not reported) in patients of membranous nephropathy. Out of them, four patients deteriorated to ESRD. Yu et al, [3] reported both pANCA and cANCA positivity along with only MPO positivity in four patients of membranous nephropathy. Three of these patients reached ESRD. In addition, there were six reports [4],[5],[6],[7],[8],[9] of eight patients with MPO-pANCA positivity. Only one of these six patients reached ESRD. A recent report [10] presented the coexistence of MPO-pANCA-associated glomerulonephritis and anti-phospholipase A 2 receptor antibody positive membranous nephropathy.

It has been postulated [2] that in some patients of ANCA-associated glomerulonephritis, MPO may serve as an antigen and form immune complexes to develop membranous nephropathy-like lesions.

 
  References Top

1.Nasr SH, Said SM, Valeri AM, Stokes MB, Masani NN, D′Agati VD, et al. Membranous glomerulonephritis with ANCA-associated necrotizing and crescentic glomerulonephritis. Clin J Am Soc Nephrol 2009;4:299-308.  Back to cited text no. 1
[PUBMED]    
2.Hanamura K, Tojo A, Kinugasa S, Asaba K, Onozato ML, Uozaki H, et al. Detection of myeloperoxidase in membranous nephropathy-like deposits in patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis. Hum Pathol 2011;42:649-58.  Back to cited text no. 2
[PUBMED]    
3.Yu F, Chen M, Wang SX, Zou WZ, Zhao MH, Wang HY. Clinical and pathological characteristics and outcomes of Chinese patients with primary anti-neutrophil cytoplasmic antibodies-associated systemic vasculitis with immune complex deposition in kidney. Nephrology (Carlton) 2007;12:74-80.  Back to cited text no. 3
[PUBMED]    
4.Kanahara K, Yorioka N, Nakamura C, Kyuden Y, Ogata S, Taguchi T, et al. Myeloperoxidase-antineutrophil cytoplasmic antibody-associated glomerulonephritis with membranous nephropathy in remission. Intern Med 1997;36:841-6.  Back to cited text no. 4
[PUBMED]    
5.Dwyer KM, Agar JW, Hill PA, Murphy BF. Membranous nephropathy and anti-neutrophil cytoplasmic antibody-associated glomerulonephritis: A report of 2 cases. Clin Nephrol 2001;56:394-7.  Back to cited text no. 5
[PUBMED]    
6.Tse WY, Howie AJ, Adu D, Savage CO, Richards NT, Wheeler DC, et al. Association of vasculitic glomerulonephritis with membranous nephropathy: A report of 10 cases. Nephrol Dial Transplant 1997;12:1017-27.  Back to cited text no. 6
[PUBMED]    
7.Suwabe T, Ubara Y, Tagami T, Sawa N, Hoshino J, Katori H, et al. Membranous glomerulopathy induced by myeloperoxidase-anti-neutrophil cytoplasmic antibody-related crescentic glomerulonephritis. Intern Med 2005;44:853-8.  Back to cited text no. 7
[PUBMED]    
8.Taniguchi Y, Yorioka N, Kumagai J, Ito T, Yamakido M, Taguchi T. Myeloperoxidase antineutrophil cytoplasmic antibody-positive necrotizing crescentic glomerulonephritis and membranous glomerulonephropathy. Clin Nephrol 1999;52:253-5.  Back to cited text no. 8
[PUBMED]    
9.Granata A, Floccari F. Membranous glomerulonephritis with superimposed ANCA-associated vasculitis: Another case report. NDT Plus 2011;4:80-1.  Back to cited text no. 9
    
10.Surindran S, Ayalon R, Hasan N, Beck Jr LH, Salant DJ, Barisoni L, et al. Coexistence of ANCA-associated glomerulonephritis and anti-phospholipase A 2 receptor antibody-positive membranous nephropathy. Clin Kidney J 2012;5:162-5.  Back to cited text no. 10
    


    Figures

  [Figure 1]
 
 
    Tables

  [Table 1]

This article has been cited by
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[Pubmed] | [DOI]



 

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