News Release:
Halpin Foundation provides grant to assess the Causes of Membranous Nephropathy in a Patient with Hyper-IgG4 Syndrome

Bergamo, Italy (November '10) — Membranous nephropathy is a chronic kidney disease that, in most cases, undergoes spontaneous remission or evolves without complications. However, about 10-20% of patients experience a loss of large amounts of protein in the urine (nephrotic syndrome, often accompanied by swelling, hypertension, and high cholesterol levels) that can cause a progressive deterioration of renal function eventually leading to the need of dialysis or transplantation in a few years.

The cause of the disease is unknown. In 70% of patients there are antibodies against a protein of the kidney, but it is not yet clear how these antibodies can also damage the kidney. These antibodies (or immunoglobulins) are of a peculiar type, called immunoglobulin 4 (IgG4). There is a rare disease, hyper-IgG4 syndrome, in which the levels of these immunoglobulins are particularly high and this injuries various organs, including the kidney. Membranous nephropathy and one of the possible manifestations of the disease in the kidney. Understanding how in patients with this syndrome elevated levels of IgG4 may cause the disease represents a unique opportunity to assess the mechanisms - and develop treatments targeted - in most common cases of membranous nephropathy not related to high levels of IgG4.

On this basis, the investigators at Mario Negri Institute designed a study to analyze the characteristics of immunoglobulins - especially IgG4 - in one of the very rare patients with membranous nephropathy associated with hyper-IgG4 syndrome (so far, less than ten patients have been reported worldwide).

The Halpin Foundation supports this research because the study of a very special case may provide useful information on the causes of membranous nephropathy even in the more frequent cases of patients with normal levels of IgG4.

Mario Negri Institute investigators:
Paolo Cravedi
Ariela Benigni
Giuseppe Remuzzi