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February 22, 2026Pediatric Nephrology0 citationsOpen Access

A nephritic puzzle: C3-dominant glomerulonephritis as a sentinel of hidden autoinflammatory disease

RWRyan C. WardUniversity of Iowa PFPolly J. FergusonUniversity of Iowa PRPrerna RastogiUniversity of Iowa

Key Points

To investigate a case of C3-dominant membranoproliferative glomerulonephritis (MPGN) associated with hyper-IgD syndrome.
Analyzed a pediatric case with biopsy-confirmed C3-dominant MPGN.
Performed genetic testing for hyper-IgD syndrome.
Monitored disease response to IL-1β blockade therapy.
Patient's MPGN was refractory to conventional immunosuppressive therapy.
IL-1β blockade resulted in remission of the MPGN.
The case emphasized the need to assess for autoinflammatory conditions in MPGN cases without complement dysregulation.

Abstract

Abstract Membranoproliferative glomerulonephritis (MPGN) is among the most challenging glomerulonephritides to diagnose and manage, given its clinico-immunopathologic heterogeneity. Although recent advances have introduced new therapies for complement-mediated and immune-complex MPGN, a substantial subset of cases remains poorly understood and lacks effective treatment. We report a pediatric case of biopsy-confirmed C3-dominant MPGN without evidence of complement dysregulation, with disease flares triggered by febrile episodes. Genetic testing identified underlying hyper-IgD syndrome (HIDS), a rare autoinflammatory disorder characterized by recurrent fevers and immune dysregulation. The patient’s MPGN was refractory to conventional immunosuppressive therapy but achieved remission with IL-1β blockade that targets HIDS. This case highlights the importance of considering alternative etiologies—including autoinflammatory diseases—in patients with MPGN when complement dysregulation is not evident.

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