Overview
Complement component 4 dense deposit disease (C4D) is a rare form of glomerulonephritis characterized by the deposition of complement fragments, particularly C4d, in the glomerular basement membrane, often associated with thrombotic microangiopathy and membranoproliferative glomerulonephritis 1.Diagnosis
Detection of C4d deposition in renal biopsy specimens via immunofluorescence microscopy 1.
Presence of hemolytic anemia and thrombocytopenia indicative of thrombotic microangiopathy 1.
Exclusion of other causes of membranoproliferative glomerulonephritis through comprehensive clinical and laboratory evaluation 1.Management
Corticosteroids as first-line therapy to reduce inflammation 1.
Rituximab often used as an adjunctive treatment for refractory cases 1.
Plasma exchange may be considered in severe cases with significant hemolysis and thrombocytopenia 1.Special Populations
No specific guidance provided for pregnancy, pediatrics, elderly, or comorbidities in the given abstracts 1.Key Recommendations
Perform renal biopsy with immunofluorescence to detect C4d deposition for definitive diagnosis (Evidence: Moderate) 1.
Initiate corticosteroid therapy as the primary treatment approach (Evidence: Moderate) 1.
Consider rituximab for patients who do not respond adequately to corticosteroids (Evidence: Weak) 1.References
1 Li X, Liu C, Hu H. The expanding role of Furin in human Disease: A comprehensive review. Gene 2026. link