{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T16:20:59Z","timestamp":1787934059826,"version":"build-2784847793"},"reference-count":19,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2021,3,2]],"date-time":"2021-03-02T00:00:00Z","timestamp":1614643200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pediatr Dev Pathol"],"published-print":{"date-parts":[[2021,6]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction and Aim<\/jats:title>\n                    <jats:p>Multiorgan autoimmunity and interstitial lung disease (ILD) are reported in patients with STAT3 GOF syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We present lung histopathology findings in 3 such children, two of whom underwent wedge biopsies with adequate diagnostic material. Wedge biopsies showed interstitial cellular expansion with linear and nodular aggregates of CD8 positive T lymphocytes, plasma cells, and histiocytes; consistent with lymphocytic interstitial pneumonia pattern (LIP). CD4+ T cells and CD20+ B cells were present but infrequent in the interstitium. FOXP3 cells ranged from 0-5%. Focal interstitial and intraalveolar histiocytes were also seen. Neutrophils and eosinophils were rare\/absent. Non-occlusive peribronchial lymphoid aggregates showed equal T and B cells; likely reactive in nature. Pulmonary vessels appeared normal without vasculitis or hypertensive change. There was no interstitial or subepithelial fibrosis or organizing pneumonia. Interlobular septa and visceral pleura were unremarkable.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Children with multi-system autoimmune disorders with ILD should be investigated for STAT3 GOF syndrome. Lung wedge biopsies are more informative than transbronchial biopsies, if a tissue sampling is indicated. CD8 dominant T cell inflammation seems to be a key driver of ILD. Although interstitial fibrosis was not seen in our small sample, longer follow up is needed to understand the natural history.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/1093526620980615","type":"journal-article","created":{"date-parts":[[2021,3,2]],"date-time":"2021-03-02T16:16:57Z","timestamp":1614701817000},"page":"227-234","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":10,"title":["Pulmonary Histopathology Findings in Patients With STAT3 Gain of Function Syndrome"],"prefix":"10.1177","volume":"24","author":[{"given":"Nahir","family":"Cortes-Santiago","sequence":"first","affiliation":[{"name":"Department of Pathology, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Forbes","sequence":"additional","affiliation":[{"name":"Department of Allergy and Immunology, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiphanie P","family":"Vogel","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"Silva-Carmona","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Hicks","sequence":"additional","affiliation":[{"name":"Department of Allergy and Immunology, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R Paul","family":"Guillerman","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akaluck","family":"Thatayatikom","sequence":"additional","affiliation":[{"name":"Department of Radiology, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kalyani","family":"Patel","sequence":"additional","affiliation":[{"name":"Department of Pediatric Immunology, University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2021,3,2]]},"reference":[{"key":"bibr1-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1202117"},{"key":"bibr2-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-med-051113-024537"},{"key":"bibr3-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaip.2019.02.018"},{"key":"bibr4-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.5RI0516-237RR"},{"key":"bibr5-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-09-602763"},{"key":"bibr6-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3040"},{"key":"bibr7-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-04-570101"},{"key":"bibr8-1093526620980615","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2017.00049"},{"key":"bibr9-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2018.07.020"},{"key":"bibr10-1093526620980615","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201906-1204LE"},{"key":"bibr11-1093526620980615","unstructured":"Musa G., Bolkent ME, Vogel TP, Thatayatikom A. Variable phenotypes in three patients with two novel STAT3 gain of function mutations. 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