Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype?

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by the fibrotic thickening of subpleural and parenchymal areas of the upper lobes. It may be both idiopathic or secondary to infections, interstitial lung diseases and/or drug exposure. Often PPFE patients report recurrent lower respiratory tract infections, suggesting that repeated inflammatory alterations induced by pulmonary infections may contribute to the development/progression of PPFE. Here, we report for the first time the case of a patient affected by Giant cell Arteritis with histologically proven PPFE. The lung involvement in GCA is rare and interstitial lung diseases are usually reported as an uncommon clinical manifestation of GCA. Our patient is probably the first case presenting PPFE associated with GCA and we wonder if this is a real associative disease or a coincidence perhaps, secondary to drug effects.

Interstitial Lung Disease in Giant Cell Arteritis

“…Our results show that various ILD patterns may occur. Airway abnormalities, such as bronchiectasis and bronchial wall thickening, noted previously in other case reports [21][22][23] occurred in 43% of our patients. The analysis showed other findings also noted in the literature, [6][7][8][9][10][11][12][13] such as pleural effusions and dry cough improving with corticosteroids.…”

“…Compared with other pulmonary findings in GCA, ILD is rare in the literature and has been reported only in individual case reports or as an interesting finding in larger studies or case series. 5,22,37,38,42,43 The first case of ILD was reported by Karam and Fulmer 5 in 1982. They described a 58-year-old woman who presented with cough, sore throat, and fevers.…”

“…Transbronchial biopsy showed interstitial pneumonia with granulomas. In 2019, Perruzza et al 22 described a 63-year-old man who, 2 months after GCA diagnosis, had cough and recurrent fevers. High-resolution CT showed numerous nodules in a peribronchovascular distribution, traction bronchiectasis, and pleural and subpleural fibrosis.…”

Pleuroparenchymal fibroelastosis in interstitial lung disease with antineutrophil cytoplasmic antibody-associated vasculitis

“…PPFE is a rare form of progressive upper-lobepredominant ILD characterised by collagenous fibrotic thickening of sub pleural and parenchymal areas and sub pleural elastosis. This new form of ILD, with no clear relationship to smoking, can be idiopathic or secondary to organ transplants, drugs, lung infections or rheumatic disorders (4,5). A single case report by Japanese colleagues (6) described PPFE histological features in a patient positive for myeloperoxidase (MPO), without any definite parenchymal involvement, while Kinoshita et al described radiological PPFE retrospectively in a single MPO-positive ILD-AAV patient (7).…”

Pleuroparenchymal Fibroelastosis-like Lesions in Clinical Practice: A Rare Entity? Review of a Radiological Database

“…Recent studies have shown the strong association between PPFE and particular clinical states, such us organ transplantation, chemotherapy, infections, dust exposure, connective tissue diseases and vasculitis, suggesting that PPFE may not only be a specific disease, but that it is likely to represent a form of chronic lung injury [8][9][10][11][12].…”