BERGERS DISEASE - HENOCH SCHONLEIN NEPHRITIS WITHOUT THE RASH

Abstract

There are similarities in the clinical and renal manifestations of Bergers disease (mesangial IgA disease) and Henoch-Schonlein nephritis. The diagnosis of Henoch-Schonlein syndrome is made only when the purpuric rash is seen. It can be suggested that some cases of Bergers disease are Henoch-Schonlein syndrome without the rash. The following family illness suggests that this is so.

Identical 7 yr old twin boys each had a proven adenovirus infection at the same time. A few days later one developed florid Henoch-Schonlein purpura, severe alimentary symptoms and transient joint symptoms. He had an acute nephritic syndrome which progressed to nephrotic syndrome and renal insufficiency. Biopsy showed severe proliferative glomerulonephritis with crescents and marked deposition of IgA, IgG, C₃ and fibrin. The twin presented with haematuria and abdominal pain but no rash. The bouts of haematuria recurred but gradually subsided to merely microscopic haematuria. His biopsy showed mesangial proliferative glomerulonephritis with mesangial deposits of IgA and to a less extent IgG and C₃. The appearance was characteristic of Bergers disease.

Immunological studies have not revealed why these two identical twins responded to the same provocation in different ways. Their illness suggests that Bergers disease may be considered a variety of Henoch-Schonlein nephritis (without the rash).

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