An Unusual Cause of Renomegaly and Renal Insufficiency: A Case Report of Renal Involvement in Common Variable Immunodeficiency disease

Abstract

Common variable immunodeficiency disease (CVID) represents a heterogeneous group of primary hypogammaglobulinemias of unknown etiology, characterized by decreased serum immunoglobulin levels and recurrent bacterial infections and is often accompanied by autoimmune disease. Renal involvement is rare in CVID, despite widespread involvement of other organ systems. We describe a 21-year-old girl who presented with recurrent infections, hepatosplenomegaly, renomegaly, and renal insufficiency. Renal biopsy revealed a remarkable diffused interstitial infiltration and severe degenerative tubular lesions. Interstitial infiltration consisted mainly of CD8⁺ T cells and CD68⁺ macrophages with less CD4⁺ T and rare B cells. For the cases with recurrent infections, multiple organomegaly, and renal insufficiency, clinicians should consider to exclude CVID, so as to make the timely diagnosis and appropriate management.

• common variable immunodeficiency disease
• renomegaly
• renal insufficiency
• renal biopsy
• hepatosplenomegaly