Nephrotic syndrome in a patient with IgA deficiency-associated mesangioproliferative glomerulonephritis

A case of mesangioproliferative glomerulonephritis in a 55-year-old woman with selective IgA deficiency and serum antinuclear antibodies who presented with nephrotic syndrome is described. The patient did not have clinical or laboratory features of systemic lupus erythematosus (SLE) other than antin...

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Bibliographic Details
Published in:Pathology 2000-02, Vol.32 (1), p.56-58
Main Authors: John, Mina, Lam, Minh, Latham, Bruce, Saker, Barry, French, Martyn A.H.
Format: Article
Language:English
Subjects:
Antibodies, Antinuclear
Biological and medical sciences
Diagnosis, Differential
Female
Fluorescent Antibody Technique, Direct
Glomerular Mesangium - metabolism
Glomerular Mesangium - ultrastructure
Glomerulonephritis
Glomerulonephritis, Membranoproliferative - etiology
Glomerulonephritis, Membranoproliferative - pathology
Glomerulonephritis, Membranous - diagnosis
Humans
IgA deficiency
IgA Deficiency - complications
IgA Deficiency - pathology
Immunoglobulin M - metabolism
Lupus Nephritis - diagnosis
Medical sciences
mesangioproliferative glomerulonephritis
Middle Aged
Nephrology. Urinary tract diseases
Nephropathies. Renovascular diseases. Renal failure
nephrotic syndrome
Nephrotic Syndrome - etiology
Nephrotic Syndrome - pathology
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Summary:A case of mesangioproliferative glomerulonephritis in a 55-year-old woman with selective IgA deficiency and serum antinuclear antibodies who presented with nephrotic syndrome is described. The patient did not have clinical or laboratory features of systemic lupus erythematosus (SLE) other than antinuclear antibodies. Histology of the patient’s renal biopsy revealed a mesangioproliferative glomerulonephritis and direct immunofluorescence showed that paramesangial deposits contained predominant IgM with lesser IgG, C3 and C1q. These findings are identical to those previously described in a form of glomerulonephritis associated with IgA deficiency and would be atypical for lupus nephritis. Glomerulonephritis is not a well recognized complication of IgA deficiency, though it has been rarely reported in the literature. This case provides further evidence that IgA deficiency is associated with a unique immune complex-mediated glomerulopathy with characteristic immunopathological and ultrastructural features. It is the first reported case to present with nephrotic syndrome.
ISSN:0031-3025
1465-3931
DOI:10.1080/003130200104600