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Diagnosis: IgA nephropathy

Two aspects are important in this case: 1) deaf-mutism history in the patient and 2) a test positive for ANA: 1:80, homogeneous pattern.

1) The deaf-mutism in the patient made some doctors think it was Alport's disease; however, deafness in Alport's disease is an acquired disorder that usually occurs in adulthood. Congenital deaf-mitism is NOT a sign suggesting Alport.

2) ANA is not an specific test for lupus. Positivity is a relatively common event in the general population.

Then, the interesting aspects about this case are not the morphological or immunopathological findings, but clinical features.

IgA nephropathy is the second more frequent primary glomerular disease (biopsy-proven) in adults in our center: 15.8%. The more frequent is focal and segmental glomerulosclerosis (37.7%). Other frequent primary glomerulopatrhies in adults in our series are: membranous GN (14.0%), crescentic GN (6.7%), minimal change disease (5.9%) (Arias LF et al. Glomerular diseases in a Hispanic population: review of a regional renal biopsy database. Sao Paulo Med J. 2009;127(3):140-4. [PubMed link] [Free full text])

See the chapter IgA Nephropathy of our Tutorial.

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Bibliography

• Mubarak M. IgA nephropathy: an update on pathogenesis and classification. J Coll Physicians Surg Pak. 2011;21(4):230-3. [PubMed link]
• Glassock RJ. The pathogenesis of IgA nephropathy. Curr Opin Nephrol Hypertens. 2011;20(2):153-60. [PubMed link]
• Shen PC, He LQ, Tang Y, Wang Q, Wang W, Li J. Clinicopathological characteristics and prognostic factors of asymptomatic IgA nephropathy. J Investig Med. 2010;58(3):560-5. [PubMed link]

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