[Renal involvement in autoimmune enteropathies].
The authors report on a infant who presented with an auto-immune enteropathy characterized by the association of a protracted diarrhea, a neonatal insulin-dependent diabetes, and a dermatitis and who developed a nephrotic syndrome at 4 months of age. A renal biopsy showed a membranous glomerulonephritis (MGN) with IgG linear deposits along the tubular basement membranes (TMB). By indirect immunofluorescence anti-enterocyte antibodies together with anti-TMB antibodies and anti-renal brush border (BB) antibodies were found in the serum of the patient. The patient received various immunosuppressive drugs that failed to improve the disease. In the course of the disease the anti-TBM antibodies disappeared progressively but the BB antibodies persisted. A review of the literature indicates that renal involvement is not uncommon in auto-immune enteropathy and in 5 cases it has been reported as being characterized by a nephrotic syndrome related to the presence of a MGN. In 4 of these cases MGN was associated with the presence of anti-TBM antibodies and in the remaining one with anti-BB antibodies. This case report shows that in human pathology, auto-antibodies to BB proteins may, as well as in experimental models, be responsible for the development of a MGN. It suggests a close relationship (probably a common epitope) between the renal BB proteins and the proteins of the gut epithelium.
Habib
R
R
INSERM U192, Hôpital Necker-Enfants Malades, Paris.
Beziau
A
A
Goulet
O
O
Blanche
S
S
Niaudet
P
P
D001327:Autoimmune Diseases / Q000150:complications* / Q000151:congenital / Q000175:diagnosis; D001706:Biopsy; D003872:Dermatitis / Q000150:complications* / Q000151:congenital; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000151:congenital / Q000175:diagnosis; D005455:Fluorescent Antibody Technique; D015433:Glomerulonephritis, Membranous / Q000150:complications* / Q000151:congenital / Q000175:diagnosis; D006801:Humans; D007231:Infant, Newborn; D007410:Intestinal Diseases / Q000150:complications* / Q000151:congenital / Q000175:diagnosis; D008297:Male