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Tubulointerstitial Nephritis in a Patient With Probable Autoimmune Lymphoproliferative Syndrome

Glerup, Mia MD*; Herlin, Troels DMSc*; Rittig, Søren DMSc*; Grønbæk, Kirsten DMSc; Hokland, Marianne DMSc; Hasle, Henrik DMSc*

Journal of Pediatric Hematology/Oncology: July 2013 - Volume 35 - Issue 5 - p e187–e189
doi: 10.1097/MPH.0b013e31828ac9fe
Online Articles: Original Articles

Autoimmune lymphoproliferative syndrome (ALPS) is caused by a nonmalignant defective Fas-mediated apoptosis. The main clinical manifestations are chronic lymphadenopathy, splenomegaly, and autoimmune cytopenia. Most patients with ALPS have a FAS germline mutation. ALPS has occasionally been associated with glomerulonephritis and we present the first report of tubulointerstitial nephritis associated with probable ALPS. A 5-year-old girl presented with fever, vomiting, hypertension, and azotemia. No autoantibodies, viral, or streptococcal antibodies were detected. A renal biopsy showed small-vessel vasculitis with normal glomeruli and inflammation in the interstitium. The patient responded to prednisolone treatment and obtained a full renal recovery. Symptoms of connective tissue disorder supervened and after the development of more pronounced splenomegaly, a diagnosis of ALPS was confirmed.

*Department of Pediatrics, Aarhus University Hospital, Skejby

Department of Biomedicine, Aarhus University, Aarhus

Department of Hematology, Rigshospitalet, Copenhagen, Denmark

The authors declare no conflict of interest.

Reprints: Mia Glerup, MD, Department of Pediatrics, Aarhus University Hospital, Brendstrupgaardsvej 100, 8210 Århus N Denmark (e-mail:

Received March 21, 2012

Accepted January 30, 2013

© 2013 by Lippincott Williams & Wilkins.