Bonifazi M, Sverzellati N, Negri E, Jacob J, Egashira R, Moser J, Piciucchi S, Mei F, De Lauretis A, Visca D, Goh N, Bonini M, Cirilli L, La Vecchia C, Chua F, Kouranos V, Margaritopoulos G, Kokosi M, Maher TM, Gasparini S, Gabrielli A, Wells AU, and Renzoni EA
Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a major cause of morbidity and mortality, mostly presenting as non-specific interstitial pneumonia. Little is known about the prevalence of pleuroparenchymal fibroelastosis (PPFE), a specific entity affecting the visceral pleura and subpleural parenchyma. We set out to estimate PPFE prevalence in two large cohorts of SSc patients and to assess its impact on survival and functional decline.A total of 359 SSc patients, derived from two referral centres in two different countries (UK and Italy), were included. The first available high-resolution computed tomography scan was independently evaluated by two radiologists blind to clinical information, to quantify ILD extent, freestanding bronchial abnormalities, and lobar percentage involvement of PPFE on a four-point categorical scale. Discordant scores were adjudicated by a third scorer. PPFE extent was further classified as limited (≤2/18) or extensive (>2/18). Results were evaluated against functional decline and mortality.The overall prevalence of PPFE in the combined SSc population was 18% (11% with extensive PPFE), with no substantial difference between the two cohorts. PPFE was significantly linked to free-standing bronchial abnormalities (61% versus 25% in PPFE versus no PPFE; p<0.0001) and to worse survival, independently of ILD severity or short-term lung function changes (HR 1.89, 95% CI 1.10-3.25; p=0.005).In the current study, we provide an exhaustive description of PPFE prevalence and clinical impact in the largest cohort of SSc subjects published so far. PPFE presence should be carefully considered, due to its significant prognostic implications., Competing Interests: Conflict of interest: M. Bonifazi reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: N. Sverzellati reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: E. Negri has nothing to disclose. Conflict of interest: J. Jacob reports personal fees from Roche and Boehringer Ingelheim, grants from GlaxoSmithKline, outside the submitted work. Conflict of interest: R. Egashira reports personal fees and non-financial support from Shionogi, Boehringer Ingelheim Japan, Bayer Yakuhin, Eisai, Daiichi Sankyo and AstraZeneca, non-financial support from Kyorin, outside the submitted work. Conflict of interest: J. Moser has nothing to disclose. Conflict of interest: S. Piciucchi has nothing to disclose. Conflict of interest: F. Mei has nothing to disclose. Conflict of interest: A. De Lauretis has nothing to disclose. Conflict of interest: D. Visca has nothing to disclose. Conflict of interest: N. Goh has nothing to disclose. Conflict of interest: M. Bonini has nothing to disclose. Conflict of interest: L. Cirilli has nothing to disclose. Conflict of interest: C. La Vecchia has nothing to disclose. Conflict of interest: F. Chua has no conflict of interest to declare Conflict of interest: V. Kouranos has nothing to disclose. Conflict of interest: G. Margaritopoulos has nothing to disclose. Conflict of interest: M. Kokosi has nothing to disclose. Conflict of interest: T.M. Maher has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB and has received consultancy or speakers fees from Apellis, Astra Zeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos, GlaxoSmithKline R&D, Indalo, Novartis, Pliant, ProMetic, Respivnat, Roche, Samumed and UCB. Conflict of interest: S. Gasparini has nothing to disclose. Conflict of interest: A. Gabrielli has nothing to disclose. Conflict of interest: A.U. Wells reports personal fees from Boehringer Ingelheim, Roche and Bayer, outside the submitted work. Conflict of interest: E.A. Renzoni reports personal fees from Boehringer Ingelheim and Roche, outside the submitted work., (Copyright ©ERS 2020.)