IGA NEPHROPATHY

Immunoglobulin A (IgA) nephropathy was first described by Berger and Hinglais in 1968; it is considered the most common glomerular disease in the world. IgA nephropathy is viewed as a chronic disease; clinical remission may be found in approximately one-third of patients several years after renal biopsy, however, disappearance of the IgA has been documented by renal biopsy in only a few patients.

After its 1968 description, IgA nephropathy was assumed to follow an uneventful course and was equated with benign recurrent hematuria. Over the years, loss of renal function was observed in about 40 percent of patients, and approximately half of these patients reached end-stage renal failure after 20 years of known disease. Loss of renal function varies, some patients reach renal failure within months after diagnosis, others demonstrate an indolent course lasting over 20 years. Around 10 percent of patients develop the nephrotic syndrome.

The duration of the disease is difficult to define, since the onset is likely to be unknown; urinary testing may be performed at random intervals, and the disease may be undetected. Duration of the disease can only be measured from the first documented hematuria or proteinuria.

Macroscopic hematuria is found to be more common among pediatric patients and its frequency decreases with age. IgA nephropathy is common in young adults. Safety of pregnancy in women with the disease is a concern; reports descri

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countries. Prevalences in Japan approach 50 percent; in Europe, prevalences of 10 to 30 percent are reported. The United States report rates as low as 2 percent in some areas, and American Indians in the Southwest demonstrate a prevalence of over 35 percent. Health screening practices influence these statistics and prevalence of other diseases characterized by IgA mesangial deposition, such as liver diseases, need to be excluded when a group shows a high prevalence of apparent IgA nephropathy. Gender and race are said to be important factors regarding epidemiology. Studies show a male predominance of at least 2:1. High frequencies of other glomerular diseases are found in blacks, but IgA nephropathy is uncommon; this is postulated to be related to a structural property of IgA2. Familial and regional clustering of this disease have been found. IgA nephropathy has been found in identical twins, and confirmed by kidney biopsy. Since 1978, scatter reports of single families with this disease, encompassing seven generations, demonstrate a genetic predisposition for at least some patients. Additional studies (48 families) found urinary abnormalities in 23 percent of relatives, with a variety of abnormalities of IgA immunobiol

Category: Medical - I