Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review

Objectives. Pleuroparenchymal fibroelastosis (PPFE) is characterized by predominantly upper lobepleural and subjacent parenchymal fibrosis; PPFE features were described in patients with rheumaticautoimmune diseases (RAID). A systematic literature review was performed to investigate the prevalence,prognosis and potential association of PPFE with previous immunosuppression in RAID.Methods. EMBASE, Web of Science and PubMed databases were questioned from inception to 1September 2019. Articles published in English and addressing PPFE in patients with RAID were selected.Results. Twenty out of 794 papers were selected with a total of 76 cases of RAID-PPFE patients (20SSc, 9 RA, 6 IIM6 primary SS, 5 overlap syndromes, 3 ANCA-associated vasculitides, 2 granulomatosiswith polyangiitis, 1 microscopic polyangiitis, 1 UCTD, 1 SLE, 1 GCA and 21 patients with non-specifiedRAID). Dyspnoea was the most frequently reported symptom (37/48 patients, 77%). Patients frequentlypresented with a restrictive pattern and decline in diffusing lung capacity for carbon monoxide. During thefollow-up, 7/12 patients had progression at imaging, 22/39 presented a generic clinical worsening, 19/38had a functional deterioration and 15/43 remained stable.Conclusion. The present systematic literature review confirms that PPFE features are present in RAID.Rheumatologists should be aware of this new radiological pattern that holds a bad prognosis.