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Year : 2007  |  Volume : 17  |  Issue : 2  |  Page : 70-72

Spontaneous partial remission of idiopathic collapsing glomerulopathy

1 Department of Nephrology, Ram Manohar Lohia Hospital, Delhi, India
2 Division of Nephrology, University College of Medical Sciences and GTB Hospital, Delhi, India
3 Department of Pathology, All India Institute of Medical Sciences, Delhi, India

Correspondence Address:
O P Kalra
Division of Nephrology, University College of Medical Sciences, G. T. B. Hospital, Dilshad Garden, Delhi - 110 095
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-4065.37025

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Patients with collapsing glomerulopathy usually present with severe nephrotic syndrome and rapid progression to end-stage renal disease. Remissions are extremely rare. We report a case of idiopathic collapsing glomerulopathy who had spontaneous partial remission.

Keywords: Collapsing glomerulopathy, focal segmental glomerulosclerosis, nephrotic syndrome

How to cite this article:
Sunder S, Kalra O P, Waghmare V, Yadav A K, Dinda A K. Spontaneous partial remission of idiopathic collapsing glomerulopathy. Indian J Nephrol 2007;17:70-2

How to cite this URL:
Sunder S, Kalra O P, Waghmare V, Yadav A K, Dinda A K. Spontaneous partial remission of idiopathic collapsing glomerulopathy. Indian J Nephrol [serial online] 2007 [cited 2022 Dec 5];17:70-2. Available from:

  Introduction Top

Collapsing glomerulopathy (CG) is an aggressive form of glomerular disease characterized by histopathological features of glomerular collapse, visceral epithelial cell damage and tubulointerstitial changes. This pattern of renal injury has been reported in association with various conditions such as HIV-associated nephropathy, lymphoproliferative disorders, collagen vascular diseases, other immune deficiency syndromes and viral infections, such as parvovirus B 19, and in patients under immunosuppressive therapy, including the renal allograft recipients. [1] At present, this entity is being increasingly recognized in the absence of secondary disorders. It shares several clinical and histopathological features with focal segmental glomerulosclerosis (FSGS). Some investigators consider it as a variant of FSGS, while others consider it a different entity. [2]

Patients with CG present with severe nephrotic syndrome, marked proteinuria - generally more than 10 g/day - and despite treatment progress rapidly to chronic renal failure or die due to complications of the nephrotic syndrome, despite any form of treatment. However, partial or complete spontaneous remission has been reported. [3] Here we report a case of idiopathic CG who had spontaneous partial remission.

  Case Report Top

A 56-year-old male presented with a history of fever for two weeks, rashes over lower limbs for 10 days and swelling all over the body and decrease in urine output for 7 days. There was no history of diabetes mellitus, hypertension, drug abuse or alcoholism. Physical examination revealed a blood pressure of 134/86 mm Hg, anasarca and rash. His investigations showed hemoglobin of 10.5 g/dl, total leucocyte of 9500/mm 3 with normal differential count and an erythrocyte sedimentation rate of 14 mm in the first hour. Urinalysis revealed the following: protein 4+, red cells: 40 to 50/hpf, plenty of leucocytes and presence of granular casts and 24-h urinary protein excretion of 5 g. His renal function parameters were deranged with blood urea (180 mg/dl) and serum creatinine (2.4 mg/dl). Other biochemical investigations, including serum electrolytes, blood sugar, liver function tests and lipid profile, were normal. Antineutophil cytoplasmic antibody and antinuclear antibody tests were negative and serum complement and IgA levels were normal. The serology for HIV-1 and HIV-2 tested on two occasions was negative. Besides this, the hepatitis B surface antigen and serology for hepatitis C virus were also negative. Ultrasound examination revealed bilateral normal-sized kidneys with increased cortical echogenicity and poor corticomedullary differentiation.

Kidney biopsy showed 12 glomeruli, of which 2 had global sclerosis and 5 had collapse of glomerular capillaries with marked hyperplasia of visceral epithelial cells. Some capillaries showed fibrin deposition. There was patchy tubular atrophy, interstitial inflammation and fibrosis. Blood vessels were unremarkable [Figure - 1]. Immunofluorescence study showed predominantly mesangial deposits of IgA, IgM and C 3 . Staining for IgG was negative. The features of kidney biopsy were suggestive of CG. The patient was treated with angiotensin converting enzyme inhibitors and diuretics. Over the next 6 weeks, he showed marked clinical improvement and his serum creatinine decreased to 1.3 mg/dl and 24-h urinary protein excretion decreased from 5 g to 2 g. He has been on regular follow up during the last 30 months. At the time of last visit, his proteinuria was 1.2 g/day and blood urea and serum creatinine were 28 mg/dl and 1.2 mg/dl, respectively. Repeat renal biopsy performed after 6 months revealed 19 glomeruli, of which 5 had global sclerosis and 2 showed segmental sclerosis involving the perihilar region. Visceral epithelial cell hyperplasia was not present. Patchy tubular atrophy and interstitial fibrosis was present. Compared to the initial biopsy, there were no features of CG, and the interstitial fibrosis did not progress.

  Discussion Top

Idiopathic CG is considered by many investigators to be a variant of FSGS having rapid progression to renal death and relative steroid resistance. However, as distinct from idiopathic FSGS, CG has the following histopathologic features: (1) extensive collapse of the glomerular tufts with little expansion of mesangial matrix; (2) visceral epithelial cell hypertrophy, usually accompanied by cytoplasmic hyaline droplets; (3) no predilection for perihilar segment as in idiopathic FSGS; and (4) more extensive tubulointerstitial changes. Detwiler et al . [4] studied 16 patients of CG with no evidence of HIV infection and compared them with 25 patients of noncollapsing FSGS. Patients with CG had black predominance, higher serum creatinine, greater urinary protein excretion (13.2 7.7 g/day vs 4.6 4.5 g/day) and poor renal survival in comparison to those with noncollapsing FSGS. Valeri et al . conducted a study of 43 cases of CG over a period of 20 years and reported similar findings of predominance in black race, higher serum creatinine and greater proteinuria. When compared to 50 patients with idiopathic FSGS, patients of CG had a shorter median time of progression to end-stage renal disease (13.0 months vs 62.5 months, P < 0.05). However, identical to our case, three of their patients had partial or complete spontaneous remissions; however, none of the patients treated with steroids responded. High initial serum creatinine and failure to undergo remission predicted a rapid progression to end-stage renal disease. Similarly, in a study by Grcevska et al. , [5] in contrast to patients with idiopathic FSGS, only 40% of whom needed dialysis over a 5-year period, all CG patients reached ESRD in less than 5 years. All these studies show that CG has considerably worse renal prognosis and rapid progression to end-stage renal disease. Because these histopathologic features are also common for HIV-associated nephropathy, it is necessary to exclude the possibility of HIV infection. In our patient, there was no evidence of HIV infection and the preceding illness with fever and rash could be due to a viral illness that may have played a role in etiopathogenesis of CG.

Both recurrent and de novo CG have been described in renal allografts with features similar to CG in native kidneys. [6] Stokes et al . [7] reported seven cases of CG in renal allograft recipients presenting with the graft dysfunction. In six out of the seven cases, renal disease could be considered as delayed de novo CG. The mean time until the diagnosis of allograft CG was 74 months. None of these seven cases had nephrotic syndrome, which is the usual presentation of CG in native kidneys. The development of CG in renal allograft biopsy specimens was associated with a high rate of graft loss.

There is no consensus regarding the treatment of CG. Angiotensin-converting enzyme inhibitors, angiotensin II receptor blockers and lipid-lowering agents are used. Steroids and cyclosporine have been used without success. The role of mycophenolate mofetil is not clear. Further studies are required to define an optimal therapy.

  References Top

1.Laurinavicius A, Rennke HG. Collapsing glomerulopathy: A new pattern of renal injury. Semin Diagn Pathol 2002;19:106-15.  Back to cited text no. 1  [PUBMED]  
2.Schwimmer JA, Markowiyz GS, Valeri A, Appel GB. Collapsing glomerulopathy. Semin Nephrol 2003;23:209-18.  Back to cited text no. 2    
3.Valeri A, Barisoni L, Appel GB, Seigle R, D'Agati V. Idiopathic collapsing focal segmental glomerulosclerosis: A clincopathologic study. Kidney Int 1996;50:1734-46.  Back to cited text no. 3  [PUBMED]  
4.Detwiler RK, Falk RJ, Hogan SL, Jennete JC. Collapsing glomerulopathy: A clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney Int 1994;45:1416-24.  Back to cited text no. 4    
5.Grcevska L, Polenakovik M. Collapsing glomerulopathy: Clinical characteristics and follow up. Am J Kidney Dis 1999;33:652-7.  Back to cited text no. 5  [PUBMED]  
6.Toth CM, Pascual M, Williams WW Jr, Delmonico FL, Cosimi AB, Colvin RB, et al. Recurrent collapsing glomerulopathy. Transplantation 1998;65:1009-10.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Stokes MB, Davies CL, Alpers CE. Collapsing glomerulopathy in renal allografts: A morphological pattern with diverse clinicopathologic associations. Am J Kidney Dis 1999;33:658-66.  Back to cited text no. 7    


  [Figure - 1]


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