Sambataro Gianluca, Sambataro Domenico, Pignataro Francesca, Torrisi Sebastiano Emanuele, Vancheri Ada, Pavone Mauro, Palmucci Stefano, Del Papa Nicoletta, Vancheri Carlo
Regional Referral Center for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dpt. of Clinical and Experimental Medicine, University of Catania, Italy.
Artroreuma S.R.L. Outpatient Clinic Accredited with the National Health System, Corso S. Vito 53, 95030 Mascalucia, CT, Italy.
Respir Med Case Rep. 2018 Dec 21;26:126-130. doi: 10.1016/j.rmcr.2018.12.014. eCollection 2019.
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.
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.
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.
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.
间质性肺疾病（ILD）患者常诉说有严重的晨僵以及带束部位疼痛，但ILD与风湿性多肌痛（PMR）之间的关联鲜有报道。本研究旨在描述一系列被归类为合并ILD的PMR患者。
我们回顾性纳入了前一年因呼吸道症状转诊至我院的PMR诊断患者。系统收集了有关提示结缔组织病（CTD）的临床和血清学表现、高分辨率胸部断层扫描（HRCT）以及肺功能测试（PFT）的数据，以核实诊断。
17例PMR患者中有15例合并ILD。10例确诊为PMR，5例发现患有CTD。7例患者在PFT时表现出严重的限制性模式，需要吸氧（5例为PMR，2例为CTD）。13例患者的肺部症状在肌肉症状之前或同时出现。关于HRCT表现，9例患者显示为非特异性间质性肺炎，2例和3例分别为寻常型间质性肺炎（UIP）和可能的UIP，1例为机化性肺炎以及合并肺纤维化和肺气肿综合征。
对于PMR患者，尤其是对低剂量类固醇反应不佳的虚弱患者，应评估肺部受累情况。在这些病例中，应深入重新评估诊断，寻找血清阴性类风湿关节炎、临床无肌病性肌炎或具有自身免疫特征的间质性肺炎。
