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Saudi Journal of Kidney Diseases and Transplantation
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LETTER TO THE EDITOR  
Year : 2016  |  Volume : 27  |  Issue : 4  |  Page : 825-827
An unusual case of idiopathic crescentic membranoproliferative glomerulonephritis with posterior reversible encephalopathy syndrome


1 Department of Nephrology, Gandhi Medical College, Hyderabad, Telangana, India
2 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

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Date of Web Publication5-Jul-2016
 

How to cite this article:
Yadla M, Sailaja S, Bottu M, Uppin M. An unusual case of idiopathic crescentic membranoproliferative glomerulonephritis with posterior reversible encephalopathy syndrome. Saudi J Kidney Dis Transpl 2016;27:825-7

How to cite this URL:
Yadla M, Sailaja S, Bottu M, Uppin M. An unusual case of idiopathic crescentic membranoproliferative glomerulonephritis with posterior reversible encephalopathy syndrome. Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2021 Oct 19];27:825-7. Available from: https://www.sjkdt.org/text.asp?2016/27/4/825/185283
To the Editor,

Crescents occur in <10% of cases with membranoproliferative glomerulonephritis (MPGN). Crescentic MPGN with >50% crescents is extremely uncommon. Posterior reversible encephalopathy syndrome (PRES) in MPGN is rare. Herein, we report a case of idiopathic crescentic MPGN with PRES.

A 30-year-old woman presented with pedal edema of insidious onset of one-month duration. She was evaluated elsewhere and was found to have blood pressure (BP) of 170/110 mm Hg. Complete urine examination showed 3+ albumin and active sediment. She developed sudden onset of visual impairment and generalized seizures. Computerized tomography (CT) scan of the brain showed features suggestive of PRES [Figure 1]. Her serum creatinine was 2.8 mg/dL. She was managed with antiepileptic and antihypertensive drugs. Subsequently, over the next three days, she developed anuria. She was referred to us for the further management. At the time of admission, she had tachypnea, tachycardia, and BP was 220/110 mm Hg. There was no peripheral bruit or renal bruit. Fundus examination showed features suggestive of hypertensive neuroretinopathy. Her hemoglobin was 8.2 g/dL, total leukocyte count was 8200 cells/mm 3 , and platelets were 230,000/μL. Her serum creatinine was 8.2 mg/dL, complete urine analysis showed 3+ albumin with active urine sediment. She was initiated on hemodialysis. Urine spot protein was 2. Ultrasound abdomen was within normal limits. Her C3 was low and C4 was normal. The antinuclear antibody, anti-double stranded antibody, anti-smith, and antiphospholipid antibody were negative. Serology for hepatitis B and hepatitis C was negative. Renal biopsy showed nine glomeruli, of which five had cellular crescents [Figure 2]. There was increase in cellularity along with neutrophilic infiltration. The tubules and interstitium were normal. Vessels showed hypertensive changes but there was no evidence of fibrinoid necrosis. Immunofluorescence showed IgG 3+, coarse granular pattern, and C3 1+ suggestive of MPGN.
Figure 1: Plain computerized tomography scan of the brain showing hypodensities in bilateral occipital regions suggestive of posterior reversible encephalopathy syndrome.

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Figure 2: (a) Light microscopy of renal biopsy showing glomeruli with crescents, mild tubular atrophy, and interstitial inflammation on H and E. (b and c) Cellular crescents seen along with glomerular sclerosis in few glomeruli. (d) Thickened basement membrane on high power microscope.

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Of all the six variants of MPGN reported based on histopathology, crescentic MPGN is rare. Although small focal crescents can occur in <10% of MPGN cases, crescentic MPGN with >50% crescents is uncommon. The clinical features, management, and prognosis of crescentic MPGN are not known due to paucity of literature. In a study of 46 cases of crescentic glomerulonephritis, only one case was reported to be due to MPGN. [1] The occurrence of crescentic MPGN due to secondary causes like hepatitis B, hepatitis C, and urticarial vasculitis has been reported. In our patient, the evidence of secondary causes of MPGN was negative. There was no evidence of systemic lupus. Hence, the diagnosis of idiopathic crescentic MPGN was considered.

Although crescentic transformation of diagnosed cases of MPGN was reported during tapering or withdrawal of immunosuppression, crescentic MPGN with anuria as the initial presentation was not reported. Anuria in our case may be due to cellular crescents. Renal recovery in cases of crescentic transformation is known to be poor.

Our patient had crescentic MPGN at initial presentation. Although she was treated with immunosuppression soon after the diagnosis, there was no improvement. Extent of disease activity at the outset and lack of improvement with therapy probably suggests poor renal prognosis.

In addition, she had yet another rare feature; PRES. Only one case of PRES was reported in a patient with hepatitis B induced type 1 MPGN. [2] In our case, PRES was probably due to malignant hypertension. She had seizures, visual loss, and high BP with hypertensive neuroretinopathy. CT scan of the brain showed hypodensities in bilateral occipital and parietal areas. With the control of BP, her vision improved.

At the end of three months, she remained anuric and was dialysis dependent. Her serum creatinine was stable at 5.2 mg/dL.

The points of interest in this case are twofold:

  1. Idiopathic crescentic MPGN with anuria as the initial presentation
  2. The occurrence of PRES in a case of idiopathic MPGN.


Conflict of interest: None declared.

 
   References Top

1.
Gupta R, Singh L, Sharma A, Bagga A, Agarwal SK, Dinda AK. Crescentic glomerulonephritis: A clinical and histomorphological analysis of 46 cases. Indian J Pathol Microbiol 2011;54:497-500.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.
Sathyanarayanan V, Razak A, Narayan G, et al. Posterior reversible encephalopathy syndrome in a patient with hepatitis B induced type 1 membranoproliferative glomerulonephritis. Clin Exp Nephrol 2010;14:614-8.  Back to cited text no. 2
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Correspondence Address:
Dr. Manjusha Yadla
Department of Nephrology, Gandhi Medical College, Hyderabad, Telangana
India
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DOI: 10.4103/1319-2442.185283

PMID: 27424710

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