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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 4  |  Page : 599-602
Crescentic Transformation in Primary Membranous Glomerulopathy: Association with Anti-GBM Antibody

Department of Nephrology, St. John’s Medical College Hospital, Bangalore, India

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Crescentic transformation of primary membranous nephropathy (MN) associated with the development of anti-GBM antibody is a rare cause of acute renal failure in patients with MN. We report a 54-year-old lady, diagnosed to have MN four years earlier, who presented with acute deterioration of renal functions. Renal biopsy revealed circumferential crescents in all glomeruli. Serum anti-GBM antibodies were positive. Despite therapy with plasmapheresis, steroids and oral cyclophosphamide, she did not improve and continues to be dialysis dependent.

Keywords: Membranous nephropathy, crescentic glomerulonephritis, anti-GBM antibody, plasmapheresis

How to cite this article:
Nayak SG, Satish R. Crescentic Transformation in Primary Membranous Glomerulopathy: Association with Anti-GBM Antibody. Saudi J Kidney Dis Transpl 2007;18:599-602

How to cite this URL:
Nayak SG, Satish R. Crescentic Transformation in Primary Membranous Glomerulopathy: Association with Anti-GBM Antibody. Saudi J Kidney Dis Transpl [serial online] 2007 [cited 2022 Nov 27];18:599-602. Available from: https://www.sjkdt.org/text.asp?2007/18/4/599/36519

   Introduction Top

Membranous nephropathy (MN) is charac­terized by sub-epithelial deposits of comple­ment and immunoglobulin. Crescentic trans­formation of MN is a rare entity and one of the factors responsible for rapid deterioration in renal function. This transformation has been associated with de novo development of either anti-glomerular basement membrane (GBM) antibody [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14] or anti-neutrophil cytoplasmic antibody (ANCA). [15] In a subset of patients, neither anti-GBM antibodies nor ANCA have been detected and these patients constitute the idiopathic cases of crescentic change. [16],[17],[18] We report a patient with the relatively rarely seen anti-GBM associated crescentic transforma­tion of underlying MN and also review the literature on this subject.

   Case Report Top

A 50-year-old lady was diagnosed to have MN in 2002 when she had presented with features of the nephrotic syndrome.

She had normal renal functions (serum crea­tinine 1.0 mg/dl) at last follow-up four months prior to admission and had been on angioten­sin converting enzyme inhibitors and statins. She presented in January 2006 with a history of worsening of edema, oliguria and vomiting of one-week's duration. She had a history of fever with yellowish productive sputum three days prior to the onset of the above symptoms for which a course of amoxycillin had been prescribed elsewhere. She did not have hemoptysis, hematuria or skin rashes. At ad­mission, she had bilateral pitting pedal edema (2+), pulse rate of 84 per minute, blood pre­ssure of 140/96 mm Hg with normal cardio­vascular and respiratory evaluation. The he­moglobin was 9.5 gm/dl, white blood cell (WBC) count was 8200/cumm with 74% neutrophils, 23% lymphocytes and 2% eosi­nophils, platelet count was 75,000/cu mm, blood urea was 134 mg/dl, serum creatinine 11.6 mg/dl, total protein 6.1 gm/dl and serum albumin 3.0 gm/dl. Urine analysis showed 2+ protein with numerous red blood cells and WBC of 8-10/HPF. Viral screening for human immunodeficiency virus, Hepatitis B and Hepatitis C was negative. Serum anti-nuclear antibody (ANA), ANCA, serum comple­ments C3 and C4 were normal. Circulating anti-GBM antibodies were positive by ELISA. Chest radiograph was normal and renal ultrasound showed normal sized kidneys. Renal doppler did not reveal any evidence of arterial or venous thrombosis.

She was hemodialyzed and a percutaneous renal biopsy was performed on the third hos­pital day. The renal biopsy [Figure - 1] revea­led ten glomeruli with 100% circumferential crescents. Immunoflourescence demonstrated granular deposits of IgG co-existent with linear deposition of IgG along the capillary wall. A diagnosis of anti-GBM glomerulo­nephritis super-imposed on pre-existing MN was made.

The patient was treated with plasmapheresis, 2-liter exchanges for a total of four sessions, pulse intravenous methylprednisolone of 1 gm daily for three days followed by oral predni­solone 1 mg/kg and oral cyclophosphamide (1 mg/kg). However, she did not show any response to therapy; hence steroids and cyclo­phosphamide were discontinued after three months. She continues to be on regular main­tenance hemodialysis at the time of making this report.

   Discussion Top

A rapid decline in renal function may occur in patients with MN in the setting of super­imposed factors such as severe hypertension, renal vein thrombosis, acute interstitial nep­hritis and crescentic transformation. Since the time Klassen et al[1] reported in 1974 a patient with MN with rapidly progressive glomerulo­nephritis associated with anti-GBM antibodies, 14 such cases have been reported [Table - 1]. Our patient had biopsy proven MN four years earlier with normal renal function before pre­senting to us with acute deterioration.

Cresentic transformation has been associa­ted with de novo formation of anti-GBM anti­bodies. Association with ANCA is rare and there is only one reported patient with Wege­ner's granulomatosis, [15] who had cresentic glomerulonephritis and MN.

Human anti-GBM disease is an autoimmune disorder characterized by autoantibodies di­rected against the non-collagenous NC 1 do­main of a 3 chain of Type-4 collagen. It is likely that the intra-membranous and epi­membranous immune complexes found in MN alter the GBM and cause release of normal or altered GBM material with the sub­sequent development of crescentic glomeru­lonephritis.

The clinical course and optimal treatment of patients with this unusual combination is not well defined. In the majority of reported cases, the prognosis has been poor despite immuno­suppressive therapy, with progression to end­ stage kidney disease in eight patients, [2],[3],[4],[8],[9],[10],[13],[14] and death occurring in four others.[1],[5],[6],[11]

Patients who develop crescentic transforma­tion with detectable anti-GBM antibodies or ANCA (idiopathic variety) may have a slight­ly better prognosis and recovery of renal function has been reported. [17] Our patient, des­pite adequate therapy for anti-GBM disease, failed to show any response and progressed to end-stage renal disease. Olieviera[19] suggests that MN may be caused by circulating rather than in situ antigen-antibody complexes involving immunoglobulin subclass IgG4. [19] It is at present unclear whether crescentic trans­formation of underlying MN implies an in­crease in the severity of the disease or deve­lopment of a second process.Optimal treat­ment regimens need to be clarified and further research in this area is necessary.

   References Top

1.Klassen J, Elwood C, Grossberg AL, et al. Evolution of membranous nephropathy into antiglomerular basement membrane glome­rulonephritis. N Engl J Med 1974;290: 1340-4.  Back to cited text no. 1  [PUBMED]  
2.Moorthy AV, Zimmerman SW, Burkholder PM, Harrington AR. Association of crescentic glomerulonephritis with membranous glome­rulopathy: A report of three cases.Clin Nephrol 1976;6:319-25.  Back to cited text no. 2  [PUBMED]  
3.Agodoa LC, Striker GE, George CR, Glassock R, Quadracci LJ. The appearance of nonlinear deposits of immunoglobulins in Goodpasture's syndrome. Am J Med 1976;61:407-13.  Back to cited text no. 3  [PUBMED]  
4.Pasternack A, Tornroth T, Linder E. Evidence of both anti-GBM and immuno­complex mediated pathogenesis in the initial phase of Goodpasture's syndrome. Clin Nephrol 1978;9:77-85.  Back to cited text no. 4    
5.Sharon Z, Rohde RD, Lewis EJ. Report of a case of Goodpasture's syndrome with unusual immunohistology and antibody reactivity. Clin Immunol Immunopathol 1981;18:402-14.  Back to cited text no. 5  [PUBMED]  
6.Richman AV, Rifkin SI, McAllister CJ. Rapidly progressive glomerulonephritis combined antiglomerular basement membrane antibody and immunecomplex pathogenesis. Hum Pathol 1981;12:597-604.  Back to cited text no. 6  [PUBMED]  
7.Patterson E, Tornnoth T, Miettinen A. Simultaneous antiglomerular basement membrane and membranous glomerulo­nephritis: Case report and literature review. Clin Immunol Immunopathol 1984;31:171-80.  Back to cited text no. 7    
8.Kurke P, Helve T, vonBonsdorff M, , et al. Transformation of membranous glome­rulonephritis into crescentic glomerulo­nephritis with glomerular basement membrane antibodies. Nephron 1984;38:134-7.  Back to cited text no. 8    
9.Hayano K, Miura H, Fukui H, Otsuka Y, Hattori S. A case of anti-GBM nephritis (cresentic glomerulonephritis) associated with membranous nephropathy. Jpn J Nephrol 1992:7:821-6.  Back to cited text no. 9    
10.Thiatiarchakul S, Lal SM, Luger A, Ross G. Goodpsture's syndrome superimposed on membranous nephropathy. A case report. Int J Artif Organs 1995;18:763-5.  Back to cited text no. 10    
11.Takeuchi K, Takeda T, Sakai I, Taneichi K, Shibaki H, An autopsy case of Goopasture syndrome preceded with membranous glomerulonephritis. Ryumachi 1997;37:781-7.  Back to cited text no. 11    
12.Meisels IS, Stillman IE, Kuhlik AB. Antiglomerular basement membrane disease and dual positivity for antineutrophil cytoplasmic antibody in a patient with membranous nephropathy. Am J Kidney Dis 1998;32:646-8.  Back to cited text no. 12  [PUBMED]  
13.Sano T, Kamata K, Shigematsu H, Kabayashi Y. A case of antiglomerular basement membrane glomerulonephritis superimposed on membranous nephropathy. Nephrol Dial Transplant 2000;15:1238-41.  Back to cited text no. 13    
14.Troxella ML, Saxena AB, Kambham N. Concurrent antiglomerular basement mem0­brane disease and membranous glomerulo­nephritis: A case report and literature review. Clin Nephrol 2006;66:120-7.  Back to cited text no. 14    
15.Gaber LW, Wall BM, Cooke CR. Co­existence of antineutrophil cytoplsmic antibody associated glomerulonephritis and membranous nephropathy. Am J Clin Pathol 1993;99:211-5.  Back to cited text no. 15  [PUBMED]  
16.James S, Lien YH, Ruffenach SJ, Wilcox GE. Acute renal failure in membranous glomerulopathy: A result of superimposed cresentic glomerulonephritis. J Am Soc Nephrol 1995;6:1541-6.  Back to cited text no. 16    
17.Hall MA, Symington EM, Sampson SA, Kwan TC. Cresentic transformation of membranous glomerulopathy: A reversible condition. Nephrol Dial Transplant 2006; 21:1136-7.  Back to cited text no. 17    
18.Kwan JT, Moore RH, Dodd SM, Cunningham J. Crescentic transformation in primary membranous glomerulonephritis. Postgrad Med J 1991;67:574-6.  Back to cited text no. 18  [PUBMED]  
19.Olieveira DB. Membranous nephropathy: an IgG4 -mediated disease. Lancet 1998;351: 670-1.  Back to cited text no. 19    

Correspondence Address:
Shobhana G Nayak
Department of Nephrology, St. John’s Medical College Hospital, Sarjapur Road, Bangalore-560034
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Source of Support: None, Conflict of Interest: None

PMID: 17951950

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  [Table - 1]

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