Published: 2022-08-24

A rare case report on rheumatoid arthritis with pleuro-parenchymal fibroelastosis

K. Siva Kumar, B. Lakshmi, R. Abarna Lakshmi, Ashish Jain


Pleuroparenchymal fibroelastosis (PPFE) is a recently characterised type of interstitial lung disease that begins in the upper lung zones and extends to the entire lung. To date, the aetiology is unknown. However, the cause of PPFE in the current case report is rheumatoid arthritis. The symptoms are dyspnoea and dry cough. Pneumothorax is a common complication that occurs at the time of presentation or subsequently in the progression of the disease. It usually occurs in > 40% of ex-smokers with restrictive patterns in the pulmonary function test. Interstitial fibrosis appears pathologically as a thick consolidation in some preserved alveolar septal outlines and a markedly abrupt contact with the normal residual lung. Undiagnosed PPFE case scan be misdiagnosed as sarcoidosis, atypical idiopathic pulmonary fibrosis, or other unclassifiable interstitial pneumonia. The current case report will address the rare outline of Rheumatoid arthritis with PPFE.


PPFE, Interstitial lung disease, Rheumatoid arthritis

Full Text:



Fischer A, Antoniou KM, Brown KK. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46:976-87.

Watanabe, Kentaro. “Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics.” Current respiratory medicine reviews vol. 2013;9(4):299-37.

Raghu G, Collard HR, Egan JJ. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788-824.

Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis. Description of a novel clinicopathologic Entity. Chest. 2004;126:2007-13.

ATS/ERS Committee on Idiopathic Interstitial Pneumonias. 2013.

An official American Thoracic Society/European Respiratory Society statement: update of the international multi-disciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733-48.

Martinez FJ, Pardo A. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.

Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:8.

Orlandi M, Landini N, Bruni C, Sambataro G, Nardi C, Bargagli E, et al. Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review. Rheumatology. 2020;59(12):3645-56.

Richeldi L, Collard HR, Jones MG. Idiopathic pulmonaryfibrosis. Lancet. 2017;389:19411952.

Flaherty KR, Mumford JA, Murray S. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168:543-8.

Sgalla G, Iovene B, Calvello M. Idiopathic pulmonary fibrosis: pathogenesis and management. Respir Res. 2018;19:32.