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Case Reports
. 2018 Dec 21:26:126-130.
doi: 10.1016/j.rmcr.2018.12.014. eCollection 2019.

Interstitial Lung Disease in patients with Polymyalgia Rheumatica: A case series

Gianluca Sambataro  1   2 , Domenico Sambataro  2 , Francesca Pignataro  3 , Sebastiano Emanuele Torrisi  1 , Ada Vancheri  1 , Mauro Pavone  1 , Stefano Palmucci  4 , Nicoletta Del Papa  3 , Carlo Vancheri  1
Affiliations
Case Reports

Interstitial Lung Disease in patients with Polymyalgia Rheumatica: A case series

Gianluca Sambataro et al. Respir Med Case Rep. .

Abstract

Introduction: Severe morning stiffness with painful involvement of the girdles are often referred by patients with Interstitial Lung Disease (ILD), but the association between ILD and Polymyalgia Rheumatica (PMR) is rarely reported. The purpose of the work is to describe a series of patients classified as having PMR with ILD.

Material and methods: We retrospectively enrolled patients with a diagnosis of PMR referred to our center during the previous year for respiratory symptoms. Data concerning clinical and serological manifestations suggesting Connective Tissue Disease (CTD), High-Resolution Chest Tomography (HRCT), and Pulmonary Function Tests (PFTs) were systematically collected in order to verify the diagnosis.

Results: Fifteen out of seventeen PMR patients had ILD. Ten patients had a confirmed diagnosis of PMR, while in five patients a CTD was discovered. Seven patients showed a severe restrictive pattern at PFTs requiring oxygen supplementation (five with PMR and two with CTD). In thirteen patients pulmonary symptoms started before or together with muscular symptoms. Regarding HRCT patterns, patients showed a Nonspecific Interstitial Pneumonia in nine cases, Usual Interstitial Pneumonia (UIP) and possible UIP in two and three cases, and a single case of Organizing Pneumonia and Combined Pulmonary Fibrosis and Emphysema Syndrome.

Conclusions: Lung involvement should be evaluated in PMR patients, especially if asthenia is poorly responsive to low doses of steroids. In these cases, the diagnosis should be re-evaluated in depth, looking for a seronegative Rheumatoid Arthritis, a clinically amyopathic myositis or Interstitial Pneumonia with Autoimmune features.

Keywords: Antisynthetase syndrome; Connective tissue disease; Interstitial lung disease; Interstitial pneumonia with autoimmune features; Myositis; Polymyalgia rheumatica.

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Figures

Fig. 1
Fig. 1
Interstitial Lung Disease with a Nonspecific Interstitial Pneumonia pattern in patients initially diagnosed as Polymyalgia Rheumatica. The diagnosis was corrected in patient A in Antisynthetase Syndrome, while in patient B was confirmed.
See this image and copyright information in PMC

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