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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
LETTER TO THE EDITOR  
Year : 2018  |  Volume : 29  |  Issue : 6  |  Page : 1515-1517
Ig M nephropathy – A clinicopathological study – Our experience


1 Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
2 Vydehi Institute of Medical Sciences, Bengaluru, Karnadaka, India

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Date of Submission03-Apr-2018
Date of Acceptance04-Apr-2018
Date of Web Publication27-Dec-2018
 

How to cite this article:
Devi BV, Lakshmi B S, Padmavathi Devi S V, Ram R, Kumar VS. Ig M nephropathy – A clinicopathological study – Our experience. Saudi J Kidney Dis Transpl 2018;29:1515-7

How to cite this URL:
Devi BV, Lakshmi B S, Padmavathi Devi S V, Ram R, Kumar VS. Ig M nephropathy – A clinicopathological study – Our experience. Saudi J Kidney Dis Transpl [serial online] 2018 [cited 2019 Jan 23];29:1515-7. Available from: http://www.sjkdt.org/text.asp?2018/29/6/1515/248282
To the Editor,

Most studies of IgM nephropathy have consisted of patients with nephrotic syndrome (NS). However, IgM nephropathy is also associated with hematuria and asymptomatic proteinuria. It appears that there are two subgroups of IgM nephropathy in presentation: a sub-group with predominance of men usually manifest NS associated with progressive disease and the other group mainly seen in women with microscopic hematuria with favorable prognosis. In the long-term follow-up approximately one-third of the NS patients found to develop renal failure of some degree, half the patients experienced hypertension and some developed focal segmental glomerular sclerosis (FSGS).[1],[2]

A retrospective descriptive study was done from the records of authors’ institution. The patients’ information obtained from the review records between 1994 and 2015. From a total number of 1730 renal biopsies performed during this period, we identified 38 patients of IgM nephropathy. Clinical history and findings of age, sex, hypertension, quantitative protei-nuria, and serum creatinine (SCr) were considered for evaluation. The diagnosis of IgM nephropathy was determined by the presence of typical light microscopy features and glome-rular IgM deposition on immunofluorescence (IF). Electron microscopy is not available at authors’ institution.

All patients managed as follows, the first episode was treated with prednisone of 0.5 to 2.0 mg/kg/day for six months. If necessary, the prednisone dose was reduced to 0.5 mg/kg/ day, but only after three months. The treatment of steroid-dependent IgM nephropathy was with cyclophosphamide, 2 mg/kg/d for 12 weeks. The treatment of steroid-resistant IgM nephropathy was with cyclosporine at 6 mg/kg/day for children or 5 mg/kg/day for adults for at least six months. All patients were treated with levamisole 2.5 mg/kg/alternate day for 18 months after remission.

From a total number of 1730 renal biopsies, performed during this period, we encountered 38 patients (2.1%) of IgM nephropathy with renal biopsy proof. The number of adults were 28 (73.6%), the number of males were 22 (52.6%). The mean age was 28 years and age range was one year to 64 years; male to female ratio was 1.2:1. The renal syndrome presentation at the first instance was NS in all 38. In addition to NS the other clinical manifestations included hypertension in six, hematuria in eight, and elevated serum creatinine in four patients.

The pattern of response to steroids in our patients included, steroid-sensitive NS (SSNS) in 28, steroid resistant NS in four, steroiddependent NS in six. Among 28 patients of SSNS, 16 were infrequent relapsers, six were frequent relapsers and another six never had a relapse.

The details of renal biopsy findings were tabulated [Table 1].
Table 1: Renal biopsy findings in 38 patients of IgM nephropathy.

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At the end of follow-up for a mean duration of 3.4 ± 0.6 years, 34 patients achieved remission and four patients had reached chronic kidney disease with serum creatinine 3.8, 4.0, 4.5 and 5.1 mg/dL.

There are only few studies have been reported on the population-based incidence and prevalence, mode of presentation, immunopatho-logic features, pattern of steroid response, and the long-term prognosis of IgM nephropathy in either children or adults. There is a wide variation in the prevalence of IgM nephro-pathy and its long-term prognosis. The reason for this is unclear. Two studies found that frequencies of 2% and 6.1%, respectively in their biopsy series.[3],[4]

In our study, the adults were more in number than children and a similar observation was reported in the earlier studies.[2] We did not find sex predilection. In earlier studies, predominance of either sex was reported.

In the earlier reports, steroid resistance was reported in 28%[5] and steroid dependence in about 33%.[1] While, in our study, among the NS patients, steroid-sensitive were 82%, steroid resistance in 9% and steroid dependence in 9%. In our study, 15% had raised SCr at presentation while it was reported as 23% and 27% in earlier studies.[1]

In renal histopathology, the majority had matrix expansion, interstitial inflammation with mono-nuclear infiltration with IgM deposits on IF. FSGS was found in only one patient. Similar observation was reported in earlier studies.[1]

The clinical course and prognosis of IgMN nephropathy were very variable. Inpart the variation in these studies was due to variable follow-up duration In the largest and longest follow-up study, renal failure was observed in 35% of cases at 15 years after initial biopsy, and 23% of patients went on to develop end-stage renal disease.[2]

In conclusion, IgM nephropathy is an important cause of NS in both children and adults. It shows a spectrum of morphologic changes ranging from minor changes to FSGS. The diagnosis depends on IF. Although a poor clinical response to steroid therapy distinguished this disease from minimal change disease, in our patients the steroid sensitivity was greater.

Conflict of interest: None declared.

 
   References Top

1.
Mokhtar GA. IgM nephropathy: Clinical picture and pathological findings in 36 patients. Saudi J Kidney Dis Transpl 2011;22: 969-75.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Myllymäki J, Saha H, Mustonen J, Helin H, Pasternack A. IgM nephropathy: Clinical picture and long-term prognosis. Am J Kidney Dis 2003;41:343-50.  Back to cited text no. 2
    
3.
Cohen AH, Border WA, Glassock RJ. Nehprotic syndrome with glomerular mesangial IgM deposits. Lab Invest 1978; 38:610-9.  Back to cited text no. 3
    
4.
Bhasin HK, Abuelo JG, Nayak R, Esparza AR. Mesangial proliferative glomerulonephritis. Lab Invest 1978;39:21-9.  Back to cited text no. 4
    
5.
Border WA. Distinguishing minimal-change disease from mesangial disorders. Kidney Int 1988;34:419-34.  Back to cited text no. 5
    

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Correspondence Address:
Dr. Rapur Ram
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh
India
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DOI: 10.4103/1319-2442.248282

PMID: 30588990

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