Henoch-Schönlein purpura and IgA nephropathy in father and son.
Considerable controversy exists as to whether Henoch-Schönlein purpura and Iga nephropathy are different clinical manifestations of the same disease or if, on the contrary, they are separate entities. We report on the development of Henoch-Schönlein purpura and IgA nephropathy in 2 members of the same family. Patient 1, a 63-year-old man, presented with purpura in the legs, abdominal pain, hematuria, renal failure and proteinuria. A biopsy of a purpuric skin lesion showed small-vessel vasculitis, and a renal biopsy showed diffuse proliferative glomerulonephritis with prominent IgA deposits, thus making the diagnosis of Henoch-Schönlein purpura. Serum IgA was increased. Patient 2, the 30-year-old son of patient 1, underwent renal biopsy for the investigation of microscopic hematuria and proteinuria. There was no history of skin rash, and serum creatinine was normal. A renal biopsy showed expansion of the mesangial matrix and marked IgA deposition. HLA typing confirmed that they shared a haplotype. HLA B35 or DR4 were absent. These results demonstrate that Henoch-Schönlein purpura and IgA nephropathy can possibly be genetically related and therefore support the notion that these two diseases probably share a common pathogenesis.
Nephrology Services, Hospital Clínic, Barcelona, Spain.
J
J
Montoliu
X M
XM
Lens
A
A
Torras
L
L
Revert
D000328:Adult; D005455:Fluorescent Antibody Technique; D005922:Glomerulonephritis, IGA / Q000150:complications / Q000235:genetics*; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000235:genetics*; D007070:Immunoglobulin A / Q000032:analysis; D008297:Male; D008875:Middle Aged
D006680:HLA Antigens; D007070:Immunoglobulin A