Radiological pleuroparenchymal fibroelastosis associated to limited cutaneous systemic sclerosis: a case report

BMC Pulmonary Medicine
D HassounC Agard

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease (ILD) characterized by progressive fibrotic lesions of the visceral pleura and the sub-pleural parenchyma, affecting predominantly the upper lobes. PPFE may occur in different contextes like bone marrow or lung transplantations, but also in the context of telomeropathy with mutations of telomerase reverse transcriptase (TERT), telomerase RNA component (TERC) or regulator of telomere elongation helicase 1 (RTEL1) genes. PPFE-like lesions have recently been described in patients with connective tissue disease (CTD)-related ILD. We report here the first detailed case of PPFE associated to systemic sclerosis (SSc) in a woman free of telomeropathy mutations. A caucasian 46 year old woman was followed for SSc in a limited form with anti-centromere Ab since 1998, and seen in 2008 for a routine visit. Her SSc was stable, and she had no respiratory signs. Pulmonary function tests showed an isolated decreased cTLCO at 55.9% (of predicted value). Cardiac ultrasonography was normal. Thoracic CT-scan showed upper lobes predominant mild and focal pleural and subpleural thickenings, suggestive of PPFE, with a slight worsening at 8 years of follow-up. She remained...Continue Reading

References

Mar 24, 2012·The European Respiratory Journal·Taryn L ReddyAndrew G Nicholson
Sep 12, 2012·Nature Reviews. Genetics·Mary Armanios, Elizabeth H Blackburn
Sep 17, 2013·American Journal of Respiratory and Critical Care Medicine·William D TravisUNKNOWN ATS/ERS Committee on Idiopathic Interstitial Pneumonias
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Sep 2, 2015·European Respiratory Review : an Official Journal of the European Respiratory Society·Susanna CappelliMarco Matucci-Cerinic
Aug 20, 2016·The European Respiratory Journal·Chad A NewtonChristine Kim Garcia

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Citations

Aug 17, 2018·Nature Reviews. Rheumatology·Christopher P DentonJohn G Coghlan

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