IgM nephropathy: Clinical picture and long-term prognosis

Myllymäki, Juhani; Saha, Heikki; Mustonen, Jukka; Helin, Heikki; Pasternack, Amos

doi:10.1053/ajkd.2003.50042

Cited by 75 publications

(135 citation statements)

References 23 publications

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“…They also reported that, in 11 patients who underwent repeated biopsy because of deterioration in kidney function, exacerbation of proteinuria, and/or poor response to pharmacotherapy, 5 renal pathologies showed FSGS, 5 showed minimal histological lesions, and 1 showed lupus nephritis. Moreover, 3 of 5 patients with FSGS developed end-stage renal failure, but none of the 5 patients with minimal lesions or 1 patient with lupus nephritis showed progressive renal dysfunction, although MPGN lesion was not detected according to the published report [12]. Kishimoto and Arakawa [13] also reported that MesPGN with mesangial IgM deposits demonstrated higher serum IgM level, lower glomerular filtration rate and serum CH50 level, higher level of proteinuria, and higher incidence of adhesion and duplication of capillary loops than MesPGN without mesangial IgM deposits, suggesting that predominant IgM deposition should be referred to as IgM nephropathy with impaired renal function and poor prognosis.…”

Section: Discussionmentioning

confidence: 91%

A case of membranoproliferative glomerulonephritis developed over twenty years with three different findings of renal pathology

et al. 2012

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A 31-year-old woman with proteinuria, hypocomplementemia, rheumatoid factor, and high serum polyclonal IgM concentration was admitted to our hospital for renal biopsy. She had a past history of two renal biopsies. When she was 12 years old, she developed proteinuria, microscopic hematuria, and hypocomplementemia. She was diagnosed as having 'IgM nephropathy' based on minor glomerular abnormalities as determined by light microscopy and IgM and C3 deposition in the mesangial region by immunofluorescence microscopy at the first biopsy. Despite corticosteroid treatment, her proteinuria did not improve and she discontinued regular outpatient checkups. When she was 29 years old and pregnant, she developed preeclampsia and, after delivery, a second renal biopsy was implemented. She was diagnosed as having progressed 'IgM nephropathy' with endotheliosis induced by preeclampsia. She was treated with angiotensin II receptor blocker and her proteinuria diminished; however, 1 year after the delivery, she developed proteinuria again, along with microscopic hematuria and hypocomplementemia. A third renal biopsy was conducted at 31 years of age and she was diagnosed as having membranoproliferative glomerulonephritis (MPGN) type I on the basis of diffuse mesangial proliferation, endocapillary hypercellularity with double contour of the capillary wall, and lobular formation in glomeruli, as determined by light microscopy. Immunofluorescence staining demonstrated deposits of C3, C4, C1q, and IgM in the mesangial region and capillary wall. She underwent corticosteroid therapy followed by normalization of urinalysis and serum complement level. Although she had initially been diagnosed with 'IgM nephropathy', she was finally diagnosed with secondary MPGN and was successfully treated by corticosteroid therapy.

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Section: Discussionmentioning

confidence: 91%

A case of membranoproliferative glomerulonephritis developed over twenty years with three different findings of renal pathology

et al. 2012

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“…However it has been noted that males are more commonly affected than females when the presentation is Nephrotic syndrome (NS), whereas females usually present with hematuria or sub-nephrotic proteinuria. Hypertension is roughly noted in up to 30% of the patients (8,11,20,23,(32)(33) (table 2).…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Most of the studies have reported on prevalence of IgMN as frequency/ percentage of renal biopsies with diagnosis of IgMN reported from 2 % to 18.5 % in native biopsies (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13). Occasional cases of IgMN in transplant biopsies have also been reported (14).…”

Section: Epidemiologymentioning

confidence: 99%

“…Unfortunately only one third of the patients, mainly with MCD/unremarkable glomerular morphology respond to steroids. Calcineurin inhibitors and Rituximab have been tried in limited studies and have shown favorable short term response (8,11,(31)(32)(33)(34)(35). However therapeutic strategies targeting suppressor T-cells and cell therapies should be tried in these patients.…”

Section: Treatment Modalitiesmentioning

confidence: 99%

“…Hypertension and proteinuria are poor prognosticators whereas age has no role in prognosis. In the largest reported series of 110 patients with 15 years follow-up, Myllymäki et al state that clinically hypertension and steroid resistance are bad prognosticators (8). Steroid resistant cases were found to have FSGS on re-biopsy.…”

Section: Prognostic Indicatorsmentioning

confidence: 99%

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