ESPN 51th Annual Meeting

ESPN 2018


 
Clinical and histopathological prognostic factors affecting the renal outcomes in childhood ANCA-associated vasculitis
Gül Özçelik 1 Hafize Emine Sönmez 2 Sezgin Şahin 3 Ayşe 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 Adrovic 3 Özgür Kasapçopur 3 Erbil Ünsal 13 Harika Alpay 11 Diclehan Orhan 17 Rezan Topaloğlu 12 Ruhan Düşünsel 18 Seza Özen 2

1- Department of Pediatric Nephology, SBÜ. Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey
2- Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey
3- Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
4- Department of Pathology, SBÜ. Sisli Etfal Training and Research Hospital, Istanbul, Turkey
5- Department of Pediatric Nephrology, Dokuz Eylul University Faculty of Medicine, İzmir, Turkey.
6- Department of Pediatric Nephrology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
7- Department of Pediatric Nephrology, SBÜ. Dr. Sami Ulus Training and Research Hospital, Istanbul, Turkey
8- Department of Pediatric Nephrology, Akdeniz University Faculty of Medicine, Antalya, Turkey.
9- Department of Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey.
10- Department of Pediatric Nephrology, Gazi University Faculty of Medicine, Ankara, Turkey.
11- Department of Pediatric Nephrology, Marmara University Faculty of Medicine, İstanbul, Turkey.
12- Department of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey
13- Department of Pediatric Rheumatology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey.
14- Department of Pediatric Neprology, Çukurova University Faculty of Medicine, Adana, Turkey.
15- Department of Pediatric Nephrology, Gaziantep University Faculty of Medicine, Gaziantep, Turkey.
16- Department of Pediatric Rheumatology, SBÜ. Dr. Sami Ulus Training and Research Hospital, Istanbul, Turkey
17- Department of Pediatric Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey
18- Department of Pediatric Nephrology, Erciyes University Faculty of Medicine, Kayseri, Turkey.
 
Introduction:

Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) are very rare in childhood vasculitides with an increased risk of morbidity and mortality. Five factor score (FFS) is a tool designed to assess prognosis at diagnosis of the patient with systemic necrotizing vasculitides and is found to be a good predictor for survival rate in adult studies. This study was aimed to evaluate renal prognostic factors in childhood AAV with the perspective of ANCA serotype, histopathological classification and FFS. 

Material and methods:

 

Pediatric AAV patients from 11 referral centers in Turkey, who were followed up between January 2006 and March 2018, were included to study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically according to the Berden classification.

Results:

 

Totally 39 patients, including 68.4% GPA, 21.1% MPA and 10.5% EGPA were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-nose- throat (ENT) involvement, 51.3% had musculoskeletal involvement.  PR3 ANCA was positive in 48.7% and MPO ANCA was positive in 30.8%. During the follow-up, impaired renal function and end-stage-renal-disease (ESRD) were assessed in 69.2% and 28.2%, respectively. At the diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows; crescentic type in 40.7%, sclerotic type in 25.9%. ETN and renal involvement statistical significance among the AAV subgroups (p <0.001, p = 0.001, respectively). Presence of PR3-ANCA antibody was higher in the GPA group. 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:

 

We have presented the association between poor histopathologic findings and MPO-ANCA positivity, impaired renal function and FFS ≥ 2 at the diagnosis. The most important predictor factor for renal prognosis was initial serum creatinine level.  In conclusion, evaluation of the FFS, ANCA serology and the creatinine levels may help to predict renal prognosis.