Tubulointerstitial Nephritis in Common Variable Immunodeficiency
Yassaman Raissian, Samih H Nasr, Paul J Kurtin, Sanjeev Sethi, Thomas C Smyrk, Lynn D Cornell. Mayo Clinic, Rochester, MN
Background: Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by decreased serum immunoglobulin levels and recurrent infections, and is often accompanied by autoimmune disease. Renal involvement in CVID has rarely been described and only in case reports. We describe a series of CVID patients (pts) with a characteristic tubulointerstitial nephritis (TIN).
Design: Renal biopsies from pts with CVID were identified from a database. Light microscopy, immunofluorescent (IF), immunohistochemical (IHC), and ultrastructural (EM) features were reviewed.
Results: Five cases of TIN in pts with CVID were identified. The mean age at biopsy was 33 yrs (range 14-58); F:M ratio was 2:3. All pts had an established diagnosis of CVID. Four pts underwent renal biopsy for acute or acute on chronic renal failure. The mean serum creatinine (SCr) was 4.1 mg/dl (range 0.8-8.0). Biopsies revealed a diffuse interstitial inflammatory cell infiltrate composed of many macrophages and lymphocytes with occasional eosinophils, plasma cells and neutrophils; and mononuclear cell tubulitis, along with mild to severe cortical interstitial fibrosis and tubular atrophy (10-75%). Two biopsies also showed focal non-necrotizing granulomatous inflammation. IHC revealed numerous CD68+ macrophages with a moderate number of CD3+ T cells and fewer scattered CD20+ B cells. Glomeruli and vessels showed no specific changes. IF was negative in the tubulointerstitial compartment and was negative or showed trace segmental granular capillary loop staining in glomeruli in 4 cases with IF studies performed; EM showed no specific features. Two pts had similar histologic findings in the lung, one of whom also had follicular and paracortical hyperplasia on a lymph node biopsy. Three pts had liver disease, one with panacinar hepatitis on biopsy, one with presumed cryptogenic cirrhosis, and one with cirrhosis presumed due to hepatitis C.
Follow-up data were available on 4 pts, at a mean of 44 months (range, 2-129) after the biopsy. Two pts were treated with corticosteroids and another with cidofovir for an unconfirmed viral infection. These 3 pts showed improved SCr but continued CRF (mean SCr 2.0 mg/dl, range 1.4-2.3). The fourth pt had end-stage renal disease at one month after presentation and required maintenance hemodialysis.
Conclusions: CVID should be added to the expanding list of systemic diseases associated with TIN. CVID-associated TIN is characterized histologically by increased interstitial macrophages and T cells, tubular atrophy, interstitial fibrosis, and sometimes non-necrotizing granulomas.
Category: Kidney (does not include tumors)
Wednesday, March 21, 2012 1:00 PM
Poster Session VI # 294, Wednesday Afternoon