title: Renal-limited vasculitis in children: a single-center retrospective long-term follow-up analysis.
abstract: Pauci-immune renal limited vasculitis (RLV) is a rare and aggressive autoimmune disease. We retrospectively analyzed the renal outcome of 6 children with biopsy proven RLV. Median age at diagnosis was 10.6 years (range 7.1 - 14.5) and the median follow-up was 4.4 years (range 2.3 - 6.6). At diagnosis, 5 patients were given induction therapy (methylprednisolone + cyclophosphamide pulses) followed by maintenance treatment (prednisolone + azathioprine) while 1 patient received maintenance treatment only. After induction, 4 patients either retained or recovered normal renal function, and 1 patient, in whom short-term plasma exchange was prescribed to try to rescue her renal function, became free from dialysis. Repeated biopsy showed no disease activity; however, renal scarring was evident in all renal specimens. At last follow-up, 2 patients had normal renal function, 3 patients had mild renal insufficiency, and 1 patient had advanced renal failure. In addition, 5 patients were treated for hypertension. Our case series suggests that an initial favorable response to immunosuppressive therapy might not necessarily prevent the occurrence of renal scarring and highlights the importance of regular follow-up.
authors.forename: Rafael T
authors.initials: RT
authors.lastname: Krmar
authors.forename: Mathias
authors.initials: M
authors.lastname: Kågebrand
authors.forename: Magnus E A
authors.initials: ME
authors.lastname: Hansson
authors.forename: Stella Edström
authors.initials: SE
authors.lastname: Halling
authors.forename: Kajsa
authors.initials: K
authors.lastname: Åsling-Monemi
authors.forename: Maria
authors.initials: M
authors.lastname: Herthelius
authors.forename: Ulla
authors.initials: U
authors.lastname: Holtbäck
authors.forename: Marta
authors.initials: M
authors.lastname: Christensson
authors.forename: Annika
authors.initials: A
authors.lastname: Wernerson
authors.forename: Annette
authors.initials: A
authors.lastname: Bruchfeld
mesh_terms: D000293:Adolescent; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents; D007668:Kidney / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D012189:Retrospective Studies
chemical_list: D007166:Immunosuppressive Agents