Abstract
Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency syndrome with characteristic features of pulmonary infections, eczema, recurrent skin abscesses and elevated serum IgE. We present a case of an HIES patient referred for nephrology consultation with elevated serum creatinine and nephrotic-range proteinuria. The subsequent kidney biopsy revealed AA-type amyloidosis and a separate and distinct inactive immune complex-mediated glomerulopathy with frequent glomerular capillary wall and mesangial polyclonal deposits. Potential kidney pathology in the setting of HIES has not been well described previously, and this case provides insight into associated renal comorbidities faced by patients with this rare syndrome.