{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:14:09Z","timestamp":1753892049268,"version":"3.41.2"},"reference-count":40,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,1,4]],"date-time":"2023-01-04T00:00:00Z","timestamp":1672790400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:p>Multisystem inflammatory syndrome in children (MIS-C) is a newly identified clinical entity still not very well known in terms of epidemiology, pathogenesis, and long-term outcome. Pulmonary involvement with acute respiratory failure is an unusual life-threatening complication of MIS-C, often a reason for admission to the pediatric intensive care unit (PICU) and the use of mechanical ventilation. We present a case of a 7-year-old male patient, previously healthy, hospitalized for MIS-C, treated with intravenous immunoglobulins (IVIG), high dose methylprednisolone, and anakinra. After 2 days of the aforementioned therapy, the patient presented with hypoxia (SatO<jats:sub>2<\/jats:sub>: 85% in ambient air room) and breathing difficulties. A chest computed tomography (CT) scan showed the presence of multiple bilateral basal parenchymal thickening and small basal pleural effusion and an arterial blood gas analysis revealed severe hypoxia (PaO<jats:sub>2<\/jats:sub>\/FiO<jats:sub>2<\/jats:sub> ratio, 170\u2005mmHg). Because of a worsening of respiratory distress, the patient was transferred to the PICU, where invasive mechanical ventilation and a continuous infusion of anakinra (12\u2005mg\/kg\/day) were started. An echocardiogram was performed, which showed an increase in pulmonary pressure (40\u2005mmHg) with normal heart ejection fraction (55%), and the hypothesis of pulmonary vasculitis involving the pulmonary arterioles was made. Therefore, therapy with sildenafil (0.15\u2005mg\/kg\/day) was promptly set up, with an immediate improvement of the clinical picture of respiratory failure, reduction of pulmonary pressure (23\u2005mmHg), and subsequent extubation at 36\u2005h with a regular clinical course until discharge. As far as we know, our case represents the first report of pulmonary vasculitis in an MIS-C patient. The use of sildenafil and high-dose continuous anakinra may represent a rescue therapy in cases of MIS-C with pulmonary vasculitis or with difficulty in extubation, allowing a short-term hospitalization in intensive care and improving the long-term outcome in these patients.<\/jats:p>","DOI":"10.3389\/fped.2022.1015617","type":"journal-article","created":{"date-parts":[[2022,12,29]],"date-time":"2022-12-29T15:55:55Z","timestamp":1672329355000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Efficacy of sildenafil and high-dose anakinra in an MIS-C patient with pulmonary vasculitis: A case report"],"prefix":"10.3389","volume":"10","author":[{"given":"Francesco","family":"La Torre","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerolmina","family":"Calabrese","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katia","family":"Signorile","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Bizzoco","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carla","family":"Mastrorilli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonella","family":"Strippoli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Doriana","family":"Amato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Carella","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ugo","family":"Vairo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Giordano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leonardo","family":"Milella","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabio","family":"Cardinale","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,1,4]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1186\/s12969-021-00511-7","article-title":"Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey","volume":"19","author":"Cattalini","year":"2021","journal-title":"Pediatr Rheumatol Online J"},{"key":"B2","doi-asserted-by":"publisher","first-page":"fmab104","DOI":"10.1093\/tropej\/fmab104","article-title":"A comparative study of children with MIS-C between admitted to the pediatric intensive care unit and pediatric ward: a one-year retrospective study","volume":"67","author":"K\u0131ymet","year":"2021","journal-title":"J Trop Pediatr"},{"key":"B3","doi-asserted-by":"publisher","first-page":"1791","DOI":"10.1002\/art.41454","article-title":"American College of rheumatology clinical guidance for multisystem infammatory syndrome in children associated with SARS-CoV-2 and hyperinfammation in pediatric COVID-19: version 1","volume":"72","author":"Henderson","year":"2020","journal-title":"Arthritis Rheumatol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"e13","DOI":"10.1002\/art.41616","article-title":"American College of rheumatology clinical guidance for multisystem inflammatory syndrome in children associated with SARS-CoV-2 and hyperinflammation in pediatric COVID-19: version 2","volume":"73","author":"Henderson","year":"2021","journal-title":"Arthritis Rheumatol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"69","DOI":"10.3390\/children7070069","article-title":"Multi-system inflammatory syndrome in children (MIS-C) following SARS-CoV-2 infection: review of clinical presentation, Hypothetical Pathog Proposed Management","volume":"7","author":"Nakra","year":"2020","journal-title":"Children (Basel)"},{"key":"B6","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/j.prrv.2020.08.001","article-title":"Multi-system inflammatory syndrome in children & adolescents (MIS-C): a systematic review of clinical features and presentation","volume":"38","author":"Radia","year":"2020","journal-title":"PaediatrRespir Rev"},{"key":"B7","doi-asserted-by":"publisher","first-page":"667507","DOI":"10.3389\/fped.2021.667507","article-title":"Best practice recommendations for the diagnosis and management of children with pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS; multisystem inflammatory syndrome in children. 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