Emergent high fatality lung disease in systemic juvenile arthritis.
Vivian E SaperGuangbo ChenGail H DeutschR Paul GuillermanJohannes BirgmeierKarthik JagadeeshScott CannaGrant SchulertRobin DeterdingJianpeng XuAnn N LeungLayla BouzoubaaKhalid AbulabanKevin BaszisEdward M BehrensJames BirminghamAlicia CaseyMichal CidonRandy Q CronAliva DeFabrizio De BenedettiIan FergusonMartha P FishmanSteven I GoodmanT Brent GrahamAlexei A GromKathleen HainesMelissa HazenLauren A HendersonAssunta HoMaria IbarraChristi J InmanRita JerathKhulood KhawajaDaniel J KingsburyMarisa Klein-GitelmanKhanh LaiSivia LapidusClara LinJenny LinDeborah R LiptzinDiana MilojevicJoy MombourquetteKaren OnelSeza OzenMaria PerezKathryn PhillippiSampath PrahaladSuhas RadhakrishnaAdam ReinhardtMona RiskallaNatalie RosenwasserJohannes RothRayfel SchneiderDieneke Schonenberg-MeinemaSusan ShenoiJudith A SmithHafize Emine SönmezMatthew L StollChristopher ToweSara O VargasRichard K VeheLisa R YoungJacqueline YangTushar DesaiRaymond BaliseYing LuLu TianGill BejeranoMark M DavisPurvesh KhatriElizabeth D Mellinsnull nullPublished in: Annals of the rheumatic diseases (2019)
A rare, life-threatening lung disease in sJIA is defined by a constellation of unusual clinical characteristics. The pathology, a PAP/ELP variant, suggests macrophage dysfunction. Inhibitor exposure may promote LD, independent of sJIA severity, in a small subset of treated patients. Treatment/prevention strategies are needed.