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- Title
Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review.
- Authors
Orlandi, Martina; Landini, Nicholas; Bruni, Cosimo; Sambataro, Gianluca; Nardi, Cosimo; Bargagli, Elena; Tomassetti, Sara; Occhipinti, Mariaelena; Randone, Silvia Bellando; Guiducci, Serena; Vancheri, Carlo; Colagrande, Stefano; Matucci-Cerinic, Marco
- Abstract
Objectives Pleuroparenchymal fibroelastosis (PPFE) is characterized by predominantly upper lobe pleural and subjacent parenchymal fibrosis; PPFE features were described in patients with rheumatic autoimmune 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 1 September 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 (20 SSc, 9 RA, 6 IIM6 primary SS, 5 overlap syndromes, 3 ANCA-associated vasculitides, 2 granulomatosis with polyangiitis, 1 microscopic polyangiitis, 1 UCTD, 1 SLE, 1 GCA and 21 patients with non-specified RAID). Dyspnoea was the most frequently reported symptom (37/48 patients, 77%). Patients frequently presented with a restrictive pattern and decline in diffusing lung capacity for carbon monoxide. During the follow-up, 7/12 patients had progression at imaging, 22/39 presented a generic clinical worsening, 19/38 had 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.
- Subjects
AUTOIMMUNE diseases; DYSPNEA; IMMUNOSUPPRESSION; MEDICAL information storage &; retrieval systems; INTERSTITIAL lung diseases; MEDLINE; META-analysis; ONLINE information services; PLEURA; RHEUMATISM; RISK assessment; SYSTEMATIC reviews; IDIOPATHIC pulmonary fibrosis; DESCRIPTIVE statistics
- Publication
Rheumatology, 2020, Vol 59, Issue 12, p3645
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/keaa451