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Demographic and laboratory data were retrospectively analyzed from patient charts. Disease activity was assessed using the pediatric vasculitis activity score (PVAS). Choroidal images were obtained with spectral domain-OCT to measure choroidal thickness (ChT) at 5 points (750 and 1500\u2009\u03bcm from the foveal center in the temporal and nasal quadrants and beneath the fovea), and to calculate the total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), and the choroidal vascularity index (CVI).<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>The median (min-max) age was 8 (4\u201316) years in PAN patients, 6 (5\u201316) years in DADA-2 patients and 8 (8\u201310) years in control group at the OCT visit (<jats:italic>p<\/jats:italic>\u2009=\u20090.214). The ChT at 3 points and the TCA, LA, and SA were higher in children with both PAN and DADA-2 patients compared to those of the control group (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001, <jats:italic>p<\/jats:italic>\u2009=\u20090.049, <jats:italic>p<\/jats:italic>\u2009=\u20090.007, p\u2009=\u20090.007, <jats:italic>p<\/jats:italic>\u2009=\u20090.006, <jats:italic>p<\/jats:italic>\u2009=\u20090.033, respectively). The CVI was similar in both groups. No association was observed between the OCT findings, PVAS, and the erythrocyte sedimentation rate, and serum leukocyte and C-reactive protein levels.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>Similar CVI scores were obtained from PAN and DADA2 patients under treatment and from healthy controls. Increased subfoveal ChT without any other signs of ocular involvement may suggest choroidal thickening as a sign of mild subclinical inflammation.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12969-020-0417-3","type":"journal-article","created":{"date-parts":[[2020,4,3]],"date-time":"2020-04-03T11:18:31Z","timestamp":1585912711000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Choroidal vascularity index as a biomarker of systemic inflammation in childhood Polyarteritis Nodosa and adenosine deaminase-2 deficiency"],"prefix":"10.1186","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1993-0403","authenticated-orcid":false,"given":"Ata","family":"BAYTARO\u011eLU","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5238-9535","authenticated-orcid":false,"given":"Sibel","family":"KADAYIF\u00c7ILAR","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7173-8617","authenticated-orcid":false,"given":"Abdullah","family":"A\u011eIN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0201-5332","authenticated-orcid":false,"given":"\u00d6zge","family":"DEL\u0130KTA\u015e","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4320-8632","authenticated-orcid":false,"given":"Selcan","family":"DEM\u0130R","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6232-0072","authenticated-orcid":false,"given":"Yelda","family":"B\u0130LG\u0130NER","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7222-7875","authenticated-orcid":false,"given":"Jale","family":"KARAKAYA","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2883-7868","authenticated-orcid":false,"given":"Seza","family":"\u00d6ZEN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6813-0862","authenticated-orcid":false,"given":"Bora","family":"ELDEM","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,4,3]]},"reference":[{"issue":"1","key":"417_CR1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/art.37715","volume":"65","author":"JC Jennette","year":"2013","unstructured":"Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised international Chapel Hill consensus conference nomenclature of Vasculitides. 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