J Korean Med Sci.  2012 Nov;27(11):1436-1438. 10.3346/jkms.2012.27.11.1436.

Membranous Nephropathy in a 13-Year-Old Boy with Common Variable Immunodeficiency

• Yim HE
• Yoo KH

• KMID: 2157960
• DOI: http://doi.org/10.3346/jkms.2012.27.11.1436

Abstract

Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.

Keyword

Autoimmunity; Common Variable Immunodeficiency; Immunoglobulins; Membranous Glomerulonephritis

MeSH Terms

Adolescent
Bronchiectasis/etiology
Common Variable Immunodeficiency/complications/*diagnosis/drug therapy
Cyclosporine/therapeutic use
Drug Therapy, Combination
Glomerulonephritis, Membranous/complications/*diagnosis/drug therapy
Humans
Immunoglobulins/therapeutic use
Immunosuppressive Agents/therapeutic use
Injections, Intravenous
Kidney/pathology
Male
Pneumonia/etiology
Proteinuria/etiology
Steroids/therapeutic use
Immunoglobulins
Immunosuppressive Agents
Steroids
Cyclosporine

Figure

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  Fig. 1 Renal biopsy findings. (A) Capillary walls are diffusely thickened in the absence of significant glomerular hypercellularity (periodic acid-Schiff, original magnification × 100). (B) Short spikes along the outer aspect of the glomerular basement membrane (arrow) (Jones' silver stain, original magnification × 400). (C) Electron micrograph showing multiple electron-dense deposits along the subepithelal side of the glomerular basement membrane (black arrows) and mesangial area (white arrows) (orginal magnification × 3,500).

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