{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T05:44:50Z","timestamp":1781070290634,"version":"3.54.1"},"reference-count":32,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T00:00:00Z","timestamp":1672531200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T00:00:00Z","timestamp":1672531200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dermatology"],"published-print":{"date-parts":[[2023]]},"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Background:&lt;\/i&gt;&lt;\/b&gt; Vascular anomalies (VAs) are increasingly being treated with PI3K\/AKT\/mTOR pathway inhibitors. These drugs have immunosuppressive properties and thus theoretically overexpose patients to opportunistic infections, especially &lt;i&gt;Pneumocystis jirovecii&lt;\/i&gt; pneumonia (PJP). PJP prophylaxis use lacks consensus. We aimed to investigate the prevalence of PJP in patients receiving mTOR\/PI3K\/AKT inhibitors for VAs and determine any indication for pneumocystis prophylaxis in this population. &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; The study was conducted in 2 parts: (1) we sent a survey to a panel of international experts of VAs asking about their use of pneumocystis prophylaxis drugs and (2) we performed a systematic review of the literature of all published cases of patients receiving these drugs for VA to estimate the prevalence of PJP in this population. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; Answers from 68 experts were analyzed: 21 (30.9%) answered they always add PJP prophylaxis when prescribing mTOR inhibitors, 20 (29.4%) case-by-case, and 27 (39.7%) never. For the systematic review, among 3,053 reports screened, 217 were included involving 1,189 patients (1,143 received sirolimus, 38 everolimus, 4 alpelisib, 4 miransertib). Among the 1,189 cases, 2 (0.2%) PJP were reported: one under sirolimus and one under everolimus. Thus, the prevalence of PJP was estimated at 0.88 cases\/1,000 patients under sirolimus (95% CI: \u22120.84 to 2.59) and 26.31 cases\/1,000 under everolimus (95% CI: \u221224.58 to 77.18). Patients with PJP never received prophylaxis drugs. We found no PJP cases under alpelisib and miransertib. PJP prophylaxis was given in 218 (18.3%) cases, more frequently for children (91.3 vs. 77.2% in the non-prophylaxis group, &lt;i&gt;p&lt;\/i&gt; = 0.012), mostly trimethoprim-sulfamethoxazole (186 patients, 85.3%). &lt;b&gt;&lt;i&gt;Conclusion:&lt;\/i&gt;&lt;\/b&gt; Our study shows that even if PJP is a rare event, it may occur in patients with VAs treated with an mTOR inhibitor. Although our results cannot allow for revising guidelines, prophylaxis with TMP-SMX might be appropriate for a subgroup of patients with risk factors for PJP. <\/jats:p>","DOI":"10.1159\/000533675","type":"journal-article","created":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T21:03:05Z","timestamp":1697058185000},"page":"942-951","source":"Crossref","is-referenced-by-count":10,"title":["Indication for a Pneumocystis Prophylaxis Therapy in Patients with Vascular Anomalies Treated with PIK3\/AKT\/mTOR Pathway Inhibitors: Experts\u2019 Opinion and Systematic Review from the Literature"],"prefix":"10.1159","volume":"239","author":[{"given":"Maxime","family":"Navarro","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aude","family":"Allemang-Trivalle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Leducq","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annie-Pierre","family":"Jonville-Bera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana\u00efs","family":"Maurier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tarik","family":"Zejli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Afi-Emili\u00e8ne","family":"Ed\u00e9e","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilie","family":"Harchaoui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Giraudeau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annabel","family":"Maruani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2023,10,4]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-3673"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36836"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2016.220"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.28036"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.23124"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-3257"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2021.3459"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18077"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-021-01290-y"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-018-0217-9"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ki.5002112"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1080\/08923973.2021.1966033"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2011.01575.x"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.28936"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.5863\/1551-6776-27.5.447"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1086\/503908"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1051\/parasite\/2011182121"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1097\/QCO.0b013e32834cac17"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1159\/000487713"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1055\/s-0031-1295725"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1080\/14656566.2021.1915989"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1007\/s00228-019-02730-0"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0b013e3181aad256"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00210714"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2017-1044"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(93)91884-o"},{"key":"ref27","doi-asserted-by":"publisher","DOI":"10.1016\/j.phrs.2018.06.010"},{"key":"ref28","doi-asserted-by":"publisher","DOI":"10.1177\/2049936121989548"},{"key":"ref29","doi-asserted-by":"publisher","DOI":"10.1093\/cid\/ciaa1611"},{"key":"ref30","doi-asserted-by":"publisher","DOI":"10.1177\/1060028016650107"},{"key":"ref31","doi-asserted-by":"publisher","DOI":"10.4065\/82.9.1052"},{"key":"ref32","doi-asserted-by":"publisher","DOI":"10.1016\/j.burns.2021.08.009"}],"container-title":["Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/drm\/article-pdf\/239\/6\/942\/4054243\/000533675.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/drm\/article-pdf\/239\/6\/942\/4054243\/000533675.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:39:01Z","timestamp":1745422741000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000533675"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023]]},"references-count":32,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2023,12,5]]},"published-print":{"date-parts":[[2023,12,5]]}},"URL":"https:\/\/doi.org\/10.1159\/000533675","archive":["Portico"],"relation":{},"ISSN":["1018-8665","1421-9832"],"issn-type":[{"value":"1018-8665",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