A case of primary immune deficiency presenting with nephrotic syndrome

NDT Plus. 2010 Oct;3(5):456-8. doi: 10.1093/ndtplus/sfq083. Epub 2010 May 11.

Abstract

Common variable immunodeficiency (CVID) is the most common form of severe antibody deficiency. The disorder is associated with a broad spectrum of clinical manifestations, including infections and chronic lung, gastrointestinal and autoimmune diseases. A 29-year-old female patient has had frequent sinopulmonary infections and gastroenteritis for the last 20 years and had been given broad-spectrum antibiotics for treatment. Immunoglobulin (Ig) levels were at undetectable levels. Renal biopsy was consistent with AA amyloidosis. She is now under follow-up with periodic intravenous Ig treatment without any infection during the last 10 months. CVID must be kept in mind in patients with recurrent sinopulmonary infections.

Keywords: amyloidosis; common variable immunodeficiency; nephrotic syndrome.