Session Type: Poster Session A
Session Time: 1:00PM-3:00PM
Background/Purpose: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) that can be idiopathic or associated with a variety of different conditions, including connective tissue diseases (CTD) [Ann Am Thorac Soc. 2019 Nov;16(11):1351-1359, Eur Respir J. 2020 Jul 9;56(1):1902135]. In this regard, the presence of PPFE has been reported as an independent predictor of worse prognosis in CTD-ILD patients [Eur Respir J. 2020 Jul 9;56(1):1902135, Rheumatology (Oxford). 2020 Dec 1;59(12):3645-3656]. Consequently, the aim of this study was to determine the prevalence of PPFE in a cohort of Spanish patients with CTD-ILD as well as to compare the characteristics between CTD-ILD patients with and without PPFE.
Methods: A total of 99 patients with CTD-ILD (31 rheumatoid arthritis (RA), 31 systemic sclerosis (SSc), 21 idiopathic inflammatory myositis (IIM), 6 primary Sjögren’s syndrome (SS), 4 systemic lupus erythematosus (SLE) and 6 with other CTDs) from the Hospital Universitario Marqués de Valdecilla (Santander, Spain) were included in this study. The presence of PPFE was confirmed by experienced radiologists evaluating chest high-resolution computed tomography images from all patients. In addition, demographic, clinical and radiological characteristics were collected.
Results: The presence of PPFE was found in 9 (9.1%) of the 99 CTD-ILD patients, whereas the remaining 90.9% had no signs of PPFE. In particular, it was confirmed in 4 patients with RA (12.9%), 2 with SSc (6.5%), 1 with IIM (4.8%), 1 with primary SS (16.7%) and 1 with SLE (25.0%) (Figure). The prevalence of bronchial dilation was statistically higher in CTD-ILD patients with PPFE compared to those without PPFE (44.4% versus 6.74%, respectively, p=0.006, Table). There were no significant differences in age at CTD or ILD diagnosis, sex, smoking history, body mass index and pulmonary function tests at ILD diagnosis between both groups (Table).
Conclusion: We provide the prevalence and clinical data of PPFE in patients with CTD-ILD from a national referral centre. Approximately 1 out of 10 of our patients presented associated PPFE, closely correlated to bronchial dilation. The identification of this condition in CTD-ILD patients should be considered routinely since it may worsen their prognosis.
Acknowledgements: Personal funds, SR-M: RD16/0012/0009 (ISCIII-ERDF).
To cite this abstract in AMA style:Atienza-Mateo B, Remuzgo-Martinez S, Ferrer D, Blanco Rodríguez G, Izquierdo Cuervo S, Mora-Cuesta V, Iturbe-Fernández D, Blanco R, Cifrián J, González-Gay M. Pleuroparenchymal Fibroelastosis: A Special Clinical Situation in Patients with Interstitial Lung Disease Associated with Connective Tissue Diseases. Descriptive Study from a Referral Centre [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/pleuroparenchymal-fibroelastosis-a-special-clinical-situation-in-patients-with-interstitial-lung-disease-associated-with-connective-tissue-diseases-descriptive-study-from-a-referral-centre/. Accessed .
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pleuroparenchymal-fibroelastosis-a-special-clinical-situation-in-patients-with-interstitial-lung-disease-associated-with-connective-tissue-diseases-descriptive-study-from-a-referral-centre/