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Diagnosis: "Idiopathic" Membranoproliferative Glomerulonephritis
When membranoproliferative glomerulonephritis (MPGN) was first delineated as a discrete clinico-pathological entity more than a half-century ago, most cases were regarded as idiopathic (or primary) in nature. Advances in analysis of pathogenetic mechanisms and etiologies underlying the lesion of MPGN have radically altered the prevalence of the truly idiopathic form of MPGN. In addition, MPGN as a category among renal biopsies showing glomerulonephritis has diminished over time. Idiopathic MPGN is a diagnosis of exclusion, at least in many adults and a portion of children, and a systematic approach to evaluation will often uncover a secondary cause, such as an infection, autoimmune disease, monoclonal gammopathy, neoplasia, complement dysregulation or a chronic thrombotic microangiopathy. Idiopathic MPGN remains an 'endangered species' after its separation from these known causes (Fervenza FC, et al. Idiopathic membranoproliferative glomerulonephritis: does it exist? Nephrol Dial Transplant. 2012;27(12):4288-94. [PubMed link]).
Idiopathic MPGN is very uncommon in developed countries, but remains a relatively common, although diminishing, cause of nephrotic syndrome in developing countries, especially those with a high burden of endemic infectious diseases (Olowu WA, et al. Childhood nephrotic syndrome in tropical Africa: then and now. Paediatr Int Child Health. 2017;37(4):259-268. [PubMed link]; Kidney Int Suppl (2011) 2012; 2(2): 198–199. [PubMed link]).
See the chapter: Membranoproliferative Glomerulonephritis of our Tutorial.
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References
- Olowu WA, Ademola A, Ajite AB, Saad YM. Childhood nephrotic syndrome in tropical Africa: then and now. Paediatr Int Child Health. 2017;37(4):259-268. [PubMed link]
- Kawasaki Y, Kanno S, Ono A, Suzuki Y, Ohara S, Sato M, Suyama K, Hashimoto K, Hosoya M. Differences in clinical findings, pathology, and outcomes between C3 glomerulonephritis and membranoproliferative glomerulonephritis. Pediatr Nephrol. 2016;31(7):1091-9. [PubMed link]
- Cook HT, Pickering MC. Histopathology of MPGN and C3 glomerulopathies. Nat Rev Nephrol. 2015;11(1):14-22. [PubMed link]
- Pavinic J, Miglinas M. The incidence of possible causes of membranoproliferative glomerulonephritis: a single-center experience. Hippokratia. 2015;19(4):314-8. [PubMed link]
- Fervenza FC, Sethi S, Glassock RJ. Idiopathic membranoproliferative glomerulonephritis: does it exist? Nephrol Dial Transplant. 2012;27(12):4288-94. [PubMed link]