Case 1 Diagnosis |
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Diagnosis:
C3 mesangial proliferative glomerulonephritis
(From 2012, this GN cloud be classified as C3 GN)
Isolated mesangial C3 proliferative glomerulonephritis was described several decades ago. It is a nephropathy poorly understood, and it is defined by mesangial deposition of C3, usually diffuse, without immunoglobulins. Glomeruli appear with variable mesangial cellular proliferation. The clinical picture is characterized by episodes of gross hematuria, persistent or recurrent microhematuria and/or proteinuria, usually in not nephrotic range. Prognosis is considered to be benign, although there are not large studies with long-term follow-up. In some cases renal function declins and they may develop terminal renal failure.
C3 mesangial proliferative glomerulonephritis is an uncommon chronic glomerular disease; long-term prognosis and optimal therapeutic intervention for it are not yet defined. At the moment we do not know an adequate treatment. Therapy combining prednisolone with immunosuppression seemed to reduce proteinuria and improve glomerular function in some patients.
In cases with C3 and IgG mesangial deposition the diagnosis is probably mesangial IgG glomerulonephritis and the clinical presentation usually is nephrotic syndrome. (Fakhouri F, et al. Mesangial IgG glomerulonephritis: a distinct type of primary glomerulonephritis. J Am Soc Nephrol. 2002;13:379-87. [PubMed link] [Free full text]). On the other hand, codeposition of IgA and C3 in the mesangium is frequent in IgA nephropathy. (Muda AO, et al. Spatial arrangement of IgA and C3 as a prognostic indicator of IgA nephropathy. J Pathol. 1995;177:201-8. [PubMed link]).W
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References
- Yagi K, Yanagida H, Sugimoto K, Kuwajima H, Tabata N, Morita K, Okada M, Takemura T. Clinicopathologic features, outcome, and therapeutic interventions in four children with isolated C3 mesangial proliferative glomerulonephritis. Pediatr Nephrol. 2005;20:1273-8. [PubMed link]
- Calls Ginesta J, Almirall J, Torras A, Darnell A, Revert L. Long-term evolution of patients with isolated C3 mesangial glomerulonephritis. Clin Nephrol. 1995;43:221-5. [PubMed link]
- Jarmolinski T, Borucka B, Wozniak A. [Regression of glomerulosclerosis in a girl with C3 nephropathy]. Wiad Lek. 2005;58 Suppl 1:73-6. [PubMed link]
- Fakhouri F, Fakhouri F, Darre S, Droz D, Lemaire M, Nabarra B, Machet MC, Chauveau D, Lesavre P, Grunfeld JP, Noel LH, Knebelmann B. Mesangial IgG glomerulonephritis: a distinct type of primary glomerulonephritis. J Am Soc Nephrol. 2002;13:379-87. [PubMed link] [Free full text]
- Manno C, Proscia AR, Laraia E, Giangrande M, Di Carlo M, Salvatore C, Tasco A, Schena FP. Clinicopathological features in patients with isolated C3 mesangial proliferative glomerulonephritis. Nephrol Dial Transplant. 1990;5 Suppl 1:78-80. [PubMed link]