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Open Access Case report

Tubulointerstitial nephritis complicating IVIG therapy for X-linked agammaglobulinemia

Keisuke Sugimoto, Hitomi Nishi, Tomoki Miyazawa, Norihisa Wada, Akane Izu, Takuji Enya, Mitsuru Okada and Tsukasa Takemura*

Author Affiliations

Department of Pediatrics, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, 589-8511 Osaka-Sayama, Japan

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BMC Nephrology 2014, 15:109  doi:10.1186/1471-2369-15-109

Published: 8 July 2014



Patients with X-linked agammaglobulinemia (XLA) develop immune-complex induced diseases such as nephropathy only rarely, presumably because their immunoglobulin (Ig) G concentration is low. We encountered a patient with XLA who developed tubulointerstitial nephritis during treatment with intravenous immunoglobulin (IVIG).

Case presentation

A 20-year-old man was diagnosed with XLA 3 months after birth and subsequently received periodic γ-globulin replacement therapy. Renal dysfunction developed at 19 years of age in association with high urinary β2-microglobulin (MG) concentrations. A renal biopsy specimen showed dense CD3-positive lymphocytic infiltration in the tubulointerstitium and tubular atrophy, while no IgG4-bearing cell infiltration was found. Fibrosclerosis and crescent formation were evident in some glomeruli. Fluorescent antibody staining demonstrated deposition of IgG and complement component C3 in tubular basement membranes. After pulse steroid therapy was initiated, urinary β2-MG and serum creatinine concentrations improved.


Neither drug reactions nor collagen disease were likely causes of tubular interstitial disorder in this patient. Although BK virus was ruled out, IgG in the γ-globulin preparation might have reacted with a pathogen present in the patient to form low-molecular-weight immune complexes that were deposited in the tubular basement membrane.

Bruton agammaglobulin tyrosine kinase (BKT) gene; Immune complexes; Steroid therapy; Tubular deposition; Tubular atrophy