title: Tubulointerstitial nephritis in a patient with probable autoimmune lymphoproliferative syndrome.
abstract: 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.
authors.affiliation: Department of Pediatrics, Aarhus University Hospital, Skejby, Denmark. miagleru@rm.dk
authors.forename: Mia
authors.initials: M
authors.lastname: Glerup
authors.forename: Troels
authors.initials: T
authors.lastname: Herlin
authors.forename: Søren
authors.initials: S
authors.lastname: Rittig
authors.forename: Kirsten
authors.initials: K
authors.lastname: Grønbæk
authors.forename: Marianne
authors.initials: M
authors.lastname: Hokland
authors.forename: Henrik
authors.initials: H
authors.lastname: Hasle
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D056735:Autoimmune Lymphoproliferative Syndrome / Q000150:complications* / Q000473:pathology; D002675:Child, Preschool; D005260:Female; D006801:Humans; D009395:Nephritis, Interstitial / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D011241:Prednisone