title: 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.
authors.affiliation: Department of Nephrology, Division of Nephrology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
authors.forename: Sha
authors.initials: S
authors.lastname: Liu
authors.forename: Qi-Dong
authors.initials: QD
authors.lastname: Zhang
authors.forename: Dong-Liang
authors.initials: DL
authors.lastname: Zhang
authors.forename: Wen-Hu
authors.initials: WH
authors.lastname: Liu
mesh_terms: D017074:Common Variable Immunodeficiency / Q000150:complications*; D005260:Female; D006801:Humans; D006984:Hypertrophy / Q000209:etiology; D007668:Kidney / Q000473:pathology*; D051437:Renal Insufficiency / Q000209:etiology*; D055815:Young Adult