IgM nephropathy revisited

Abstract

IgM nephropathy (IgMN) is an idiopathic immune complex-mediated glomerulopathy that was first described as a distinct disease in a nephropathology literature in 1978. Here, a historical review and the current status of IgMN in the light of world literature and the current experience will be presented. The Pubmed (www.pubmed.gov) search was made for articles on IgMN as the sole subject of the study or where it constituted a significant number of cases in a biopsy series in the world literature written in English. A total of 41 articles were found. A critical review of the literature was made. Soon after 1978, a series of reports were published mostly from the western world, but the interest in the entity did not withstand the test of time. No substantial basic medical research was carried out and the disease was largely ignored by the western researchers. More recently, a flurry of articles have appeared in the literature on the topic, mostly from tropical countries, and have renewed the interest in the entity. However, most of the current literature on IgMN is based on clinical observations, and experimental models and mechanistic studies of IgMN are lacking. There is an urgent need to develop consensus based criteria for the diagnosis of the condition, as well as, to focus the research on mechanistic studies to understand the pathogenesis of the disease better.

Acute Cerebellar Thrombosis in an Adult Patient with IgM Nephropathy

“…Focal segmental glomerular sclerosis is not uncommon. 7 Electron microscopy may show dense deposits in the mesangium and effacement of foot processes as seen in our patient.…”

“…8 Hypertension and focal segmental glomerulosclerosis are factors associated with progression to ESRD. [7][8][9] Women with hematuria have a favorable prognosis, and age appears to have no role as a prognostic indicator in this disease. 3 Our patient presented primarily with nonspecific complaints of epigastric discomfort and generalized malaise.…”

“…Although some studies have shown increased serum IgM or immune complex deposits, there has been no association with a structural abnormality of the IgM molecule. 7 It appears that immune complex deposits such as C1q and C4d in the glomerular mesangium play a key role in the pathogenesis of the disease. 7 It has been postulated that IgM-immune complex deposits in the mesangium result in a T-cell mediated inflammatory response leading to mesangial proliferation and glomeruli injury.…”

“…7 It appears that immune complex deposits such as C1q and C4d in the glomerular mesangium play a key role in the pathogenesis of the disease. 7 It has been postulated that IgM-immune complex deposits in the mesangium result in a T-cell mediated inflammatory response leading to mesangial proliferation and glomeruli injury. 8 IgM deposits may be seen in systemic diseases such as systemic lupus erythematosus, Alport syndrome, and rheumatoid arthritis.…”

“…Currently, there is no consensus on the diagnosis of IgM nephropathy. 1 It is defined by its immunohistologic features, 7 and an underlying characteristic of this disease entity is diffuse IgM deposits on immunofluorescence. Complement deposits of C3 and C1q are often found along with the IgM deposits.…”