What question did this study set out to answer?

To illustrate the complex presentation of adult-onset Still's disease (AOSD) with concurrent macrophage activation syndrome (MAS) and membranoproliferative glomerulonephritis (MPGN).

April 26, 2026Open Access

Stormy Course of Adult-Onset Still's Disease With Macrophage Activation Syndrome and Concurrent Membranoproliferative Glomerulonephritis: A Case Report

Key Points

To illustrate the complex presentation of adult-onset Still's disease (AOSD) with concurrent macrophage activation syndrome (MAS) and membranoproliferative glomerulonephritis (MPGN).
Case report of a 22-year-old male with AOSD fulfilling Yamaguchi criteria.
Assessment included renal biopsy demonstrating MPGN features.
Treatment involved pulsed corticosteroids, oral steroids, and tacrolimus.
Patient presented with MAS symptoms: cytopenias, hyperferritinemia (high ferritin levels), and acute kidney injury with proteinuria.
Successful management led to clinical and biochemical improvement post-treatment.
Renal biopsy confirmed MPGN, underscoring atypical renal involvement in AOSD.

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, characterized by fever, arthralgia, rash, and leukocytosis. Severe disease may be complicated by macrophage activation syndrome (MAS), a life-threatening hyperinflammatory state marked by cytopenias, hyperferritinemia, and multiorgan involvement. Renal manifestations in AOSD are uncommon and varied. We report the case of a 22-year-old Nigerian male who fulfilled the Yamaguchi criteria for AOSD and presented with features consistent with MAS, including cytopenias, hyperferritinemia, hypertriglyceridemia, and transaminitis. He also developed acute kidney injury with significant proteinuria. Renal biopsy demonstrated features consistent with membranoproliferative glomerulonephritis (MPGN). The patient was successfully treated with pulsed corticosteroids followed by oral steroids and tacrolimus, resulting in clinical and biochemical improvement. This case highlights the diagnostic complexity of AOSD complicated by MAS, particularly in the presence of atypical renal involvement. Early recognition and prompt initiation of immunosuppressive therapy are essential to improving outcomes.

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