Henoch-Schönlein purpura with immunoglobulin A nephropathy and abnormalities of immunoglobulin A in a Wiskott-Aldrich syndrome carrier.
Abnormalities of immunoglobulin A1 (IgA1) glycosylation have been described in patients with IgA nephropathy (IgAN), whether primitive or secondary to Henoch-Schönlein purpura. The Wiskott-Aldrich syndrome, an X-linked recessive disorder, is associated with abnormalities of IgA. Renal involvement with mesangial IgA deposition identical to that found in IgAN has been reported during this affection. We report the case of a female carrier of the Wiskott-Aldrich syndrome presenting with Henoch-Schönlein purpura and abnormalities of IgA glycosylation, as previously reported in patients with IgAN. The galactosylation abnormalities of IgA could be linked to the patient's status as carrier of the Wiskott-Aldrich syndrome and could contribute to the pathogenesis of IgAN.
Lasseur
C
C
Department of Nephrology, Hôpital Saint-André, Bordeaux, France.
Allen
A C
AC
Deminière
C
C
Aparicio
M
M
Feehally
J
J
Combe
C
C
D000328:Adult; D005260:Female; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000276:immunology; D006579:Heterozygote; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications*; D007070:Immunoglobulin A / Q000378:metabolism*; D037102:Lectins / Q000378:metabolism; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000235:genetics / Q000276:immunology
D007070:Immunoglobulin A; D037102:Lectins