ISSN: 1305-385X
Hakkında: Özel sayılar şeklinde yayınlanır.
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Immunoglobulin A Nephropathy
Dr. Sedat ÜSTÜNDAĞa
aNefroloji BD, Trakya Üniversitesi Tıp Fakültesi, EDİRNE
Primary immunoglobulin A nephropathy (IgAN) is a mesangial proliferative glomerulonephritis (GN) characterized by diffuse mesangial deposition of IgA. IgAN was first recognized when immunofluorescence techniques were introduced for the study of renal biopsy. IgAN is the most common form of GN throughout the world. Although primary IgAN was considered a benign condition for many years, its now clear 25-50 percent of patients progress eventually to end-stage renal disease (ESRD). IgAN occurs at any age, most commonly with clinical onset in the second and third decades of life. The cause of primary IgAN, and the mechanism underlying glomerular IgA deposition in IgAN has not been fully defined. The strongest evidence from human studies of a role genetic factors in the development and progression of IgAN is provided by descriptive reports familial aggregation of gAN. A linked locus at 6q22-23 (IGAN1) has been demonstrated in a number of families. The deposited IgA in human IgAN is almost exclusively the IgA1 subclass. Recent studies had demostrated that glycosylation patern of serum and mesangial IgA1 was abnormal. Deglycosylated IgA1 had a higher affinity to other proteins and mesengial cells. It is an important cause of ESRD, and therefore treatment strategies to reduce the risk of IgAN progressing to ESRD would have substantial health benefit. Treatment options of IgAN patients currently lack a disease-spesific approach. There remains no consensus on the use of immunosuppressive agents for treatment of progressive IgAN. Tight blood pressure control will remain one of the cornerstones in the treatment of IgAN.Keywords: Immunoglobulin A nephropathy, berger’s disease, glomerulonephritis, end-stage renal diseaseTurkiye Klinikleri J Int Med Sci 2006, 2(21):37-48
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