Clinical and histopathological prognostic factors affecting the renal outcomes in childhood ANCA-associated vasculitis.

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, an...

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Published in:Pediatric Nephrology Vol. 34; no. 5; pp. 847 - 855
Main Authors: Özçelik, Gül, Sönmez, Hafize Emine, Şahin, Sezgin, Özağarı, Ayşim, Bayram, Meral Torun, Çiçek, Rümeysa Yasemin, Çakıcı, Evrim Kargın, Çomak, Elif, Barut, Kenan, Şahin, Nihal, Bakkaloğlu, Sevcan, Gökçe, İbrahim, Düzova, Ali, Bilginer, Yelda, Açarı, Ceyhun, Melek, Engin, Kılıç, Beltinge Demircioğlu, Özdel, Semanur, Adroviç, Amra, Kasapçopur, Özgür
Format: research tables/charts Journal Article
Published: Springer Nature May2019
Online Access:View this record in EBSCOhost
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      dt: May2019
      vid: 34
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      pub: Springer Nature
      place: New York, New York
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        10.1007/s00467-018-4162-5
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        atl: Clinical and histopathological prognostic factors affecting the renal outcomes in childhood ANCA-associated vasculitis.
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        au:
          Özçelik, Gül
          Sönmez, Hafize Emine
          Şahin, Sezgin
          Özağarı, Ayşim
          Bayram, Meral Torun
          Çiçek, Rümeysa Yasemin
          Çakıcı, Evrim Kargın
          Çomak, Elif
          Barut, Kenan
          Şahin, Nihal
          Bakkaloğlu, Sevcan
          Gökçe, İbrahim
          Düzova, Ali
          Bilginer, Yelda
          Açarı, Ceyhun
          Melek, Engin
          Kılıç, Beltinge Demircioğlu
          Özdel, Semanur
          Adroviç, Amra
          Kasapçopur, Özgür
        affil: Department of Pediatric Nephrology, SBÜ. Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey
      sug:
        subj:
          Antibodies, Antineutrophil Cytoplasmic Adverse Effects
          Vasculitis Diagnosis
          Kidney Diseases Prognosis
          Human
          Child
          Turkiye
          Autoimmune Diseases
          Biopsy Methods
          Autoantibodies Blood
          Logistic Regression
          Creatinine
          Kidney Failure, Chronic Prognosis
          Vasculitis Mortality
          Socioeconomic Factors
          Outcome Assessment
          Pediatric Care
          Quality of Health Care
          Child: 6-12 years
      ab: Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS).Methods: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically.Results: Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ≥ 2, compared to patients with FFS < 2. Patients with FFS ≥ 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007).Conclusions: Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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