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

  • 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 ÖzenEmail author
Original Article

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.

Keywords

Antineutrophil cytoplasmic antibody–associated vasculitides Five-factor score Renal involvement ANCA Histopathology 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© IPNA 2019

Authors and Affiliations

  • Gül Özçelik
    • 1
  • Hafize Emine Sönmez
    • 2
  • Sezgin Şahin
    • 3
  • Ayşim Özağarı
    • 4
  • Meral Torun Bayram
    • 5
  • Rümeysa Yasemin Çiçek
    • 6
  • Evrim Kargın Çakıcı
    • 7
  • Elif Çomak
    • 8
  • Kenan Barut
    • 3
  • Nihal Şahin
    • 9
  • Sevcan Bakkaloğlu
    • 10
  • İbrahim Gökçe
    • 11
  • Ali Düzova
    • 12
  • Yelda Bilginer
    • 2
  • Ceyhun Açarı
    • 13
  • Engin Melek
    • 14
  • Beltinge Demircioğlu Kılıç
    • 15
  • Semanur Özdel
    • 16
  • Amra Adroviç
    • 3
  • Özgür Kasapçopur
    • 3
  • Erbil Ünsal
    • 13
  • Harika Alpay
    • 11
  • Diclehan Orhan
    • 17
  • Rezan Topaloğlu
    • 12
  • Ruhan Düşünsel
    • 9
  • Seza Özen
    • 2
    • 18
    Email author
  1. 1.Department of Pediatric NephrologySBÜ. Sisli Hamidiye Etfal Training and Research HospitalIstanbulTurkey
  2. 2.Department of Pediatric RheumatologyHacettepe University Faculty of MedicineAnkaraTurkey
  3. 3.Department of Pediatric Rheumatology, Cerrahpasa Medical SchoolIstanbul UniversityIstanbulTurkey
  4. 4.Department of PathologySBÜ. Sisli Etfal Training and Research HospitalIstanbulTurkey
  5. 5.Department of Pediatric NephrologyDokuz Eylul University Faculty of MedicineİzmirTurkey
  6. 6.Department of Pediatric Nephrology, Cerrahpasa Medical SchoolIstanbul UniversityIstanbulTurkey
  7. 7.Department of Pediatric NephrologySBÜ. Dr. Sami Ulus Training and Research HospitalIstanbulTurkey
  8. 8.Department of Pediatric NephrologyAkdeniz University Faculty of MedicineAntalyaTurkey
  9. 9.Department of Pediatric Nephrology and RheumatologyErciyes University Faculty of MedicineKayseriTurkey
  10. 10.Department of Pediatric NephrologyGazi University Faculty of MedicineAnkaraTurkey
  11. 11.Department of Pediatric NephrologyMarmara University Faculty of MedicineİstanbulTurkey
  12. 12.Department of Pediatric NephrologyHacettepe University Faculty of MedicineAnkaraTurkey
  13. 13.Department of Pediatric RheumatologyDokuz Eylul University Faculty of MedicineIzmirTurkey
  14. 14.Department of Pediatric NephrologyÇukurova University Faculty of MedicineAdanaTurkey
  15. 15.Department of Pediatric NephrologyGaziantep University Faculty of MedicineGaziantepTurkey
  16. 16.Department of Pediatric RheumatologySBÜ. Dr. Sami Ulus Training and Research HospitalIstanbulTurkey
  17. 17.Department of Pediatrics, Division of RheumatologyHacettepe University Faculty of MedicineAnkaraTurkey
  18. 18.Department of Pediatric PathologyHacettepe University Faculty of MedicineAnkaraTurkey

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