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04.01.2019 | Original Article

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

verfasst von: Gül Özçelik, Hafize Emine Sönmez, Sezgin Şahin, Ayşim Özağarı, Meral Torun Bayram, Rümeysa Yasemin Çiçek, Evrim Kargın Çakıcı, Elif Çomak, Kenan Barut, Nihal Şahin, Sevcan Bakkaloğlu, İbrahim Gökçe, Ali Düzova, Yelda Bilginer, Ceyhun Açarı, Engin Melek, Beltinge Demircioğlu Kılıç, Semanur Özdel, Amra Adroviç, Özgür Kasapçopur, Erbil Ünsal, Harika Alpay, Diclehan Orhan, Rezan Topaloğlu, Ruhan Düşünsel, Seza Özen

Erschienen in: Pediatric Nephrology | Ausgabe 5/2019

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Abstract

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.

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Titel: Clinical and histopathological prognostic factors affecting the renal outcomes in childhood ANCA-associated vasculitis
verfasst von: Gül Özçelik
Hafize Emine Sönmez
Sezgin Şahin
Ayşim Özağarı
Meral Torun Bayram
Rümeysa Yasemin Çiçek
Evrim Kargın Çakıcı
Elif Çomak
Kenan Barut
Nihal Şahin
Sevcan Bakkaloğlu
İbrahim Gökçe
Ali Düzova
Yelda Bilginer
Ceyhun Açarı
Engin Melek
Beltinge Demircioğlu Kılıç
Semanur Özdel
Amra Adroviç
Özgür Kasapçopur
Erbil Ünsal
Harika Alpay
Diclehan Orhan
Rezan Topaloğlu
Ruhan Düşünsel
Seza Özen
Publikationsdatum: 04.01.2019
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 5/2019
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-018-4162-5

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