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Diagnosis: IgA Dominant Postinfectious Glomerulonephritis
Immunoglobulin A (IgA) dominant postinfectious glomerulonephritis (IgA PIGN) is an increasingly recognized morphologic variant of acute PIGN, particularly in the elderly. In contrast to classic acute PIGN, which has typical glomerular deposition of IgG and C3 or C3 only, IgA is the sole or dominant immunoglobulin in IgA PIGN. Because the majority of reported cases occur in association with staphylococcal infections, the alternative designation 'IgA-dominant acute poststaphylococcal glomerulonephritis' has been applied. Diabetes is a major risk factor, likely reflecting the high prevalence of staphylococcal infection in diabetics, particularly involving skin. Patients typically present with severe renal failure, proteinuria and hematuria. Prognosis is guarded with less than a fifth of patients fully recovering renal function. This variant of acute PIGN must be distinguished from IgA nephropathy. Features that favor IgA-dominant PIGN over IgA nephropathy include initial presentation in older age or in a diabetic patient, acute renal failure, intercurrent culture-documented staphylococcal infection, hypocomplementemia, diffuse glomerular endocapillary hypercellularity with prominent neutrophil infiltration on light microscopy, stronger immunofluorescence staining for C3 than IgA, with a granular pattern suggesting post-infectious GN, with "lumpy-bumpy" (or "garland" or "starry sky") appearance, and the presence of subepithelial humps. The pathogenetic mechanism of selective IgA deposition in patients with poststaphylococcal glomerulonephritis likely involves specific host responses to the inciting pathogen (Nasr SH, D'Agati VD. IgA-dominant postinfectious glomerulonephritis: a new twist on an old disease. Nephron Clin Pract. 2011;119(1):c18-25 [PubMed link]).
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References
- Huang Z, Chen B, Zhou Y, Liang Y, Qiu W, Lv Y, Ding X, Zhang J, Chen C. Clinicopathological and prognostic study of IgA-dominant postinfectious glomerulonephritis. BMC Nephrol. 2021 Jul 5;22(1):248. [PubMed link]
- Carbayo J, Rodriguez-Benitez P, Diaz-Crespo F, Muñoz de Morales A. IgA dominant postinfectious glomerulonephritis secondary to cutaneous infection by methicillin-resistant Staphylococcus aureus. Nefrologia (Engl Ed). 2019;39(4):446-448. [PubMed link]
- Mascarenhas R, Fogo AB, Steele RW, Baliga R. IgA-Dominant Postinfectious Glomerulonephritis. Clin Pediatr (Phila). 2016;55(9):873-6. [PubMed link]
- Rajakumar V, Mohamed SA, Kurien AA, Fernando ME. IgA dominant postinfectious glomerulonephritis: Report of two cases. Indian J Nephrol. 2014;24(3):181-4. [PubMed link]
- Nasr SH, D'Agati VD. IgA-dominant postinfectious glomerulonephritis: a new twist on an old disease. Nephron Clin Pract. 2011;119(1):c18-25 [PubMed link]