Hes T, Wong M, Tikly M, Govind N
Department of Medicine, Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa.
Department of Medicine, School of Clinical Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
Afr J Thorac Crit Care Med. 2024 Oct 14;30(3):e1663. doi: 10.7196/AJTCCM.2024.v30i3.1663. eCollection 2024.
Pulmonary complications cause significant morbidity and mortality in patients with idiopathic inflammatory myopathies (IIMs).
To describe the frequency and spectrum of pulmonary complications in patients with IIMs in South Africa (SA).
A retrospective records review of adult patients with IIMs or clinically amyopathic dermatomyositis (CADM) presenting with respiratory complaints at a tertiary care facility in SA was performed. Clinical features, results of laboratory and pulmonary function tests (PFTs), radiological findings and treatment were recorded.
Pulmonary complications were documented in 66 patients. Most patients (n=41; 62.1%) had dermatomyositis, 14 (21.2%) had polymyositis, and 3 (4.5%) had CADM. There were 8 patients with overlap syndromes. Dyspnoea and a dry cough were the most common presenting symptoms, in 52 (78.8%) and 36 (54.5%) patients, respectively. Bibasal crackles were noted in 38 patients (57.6%). Interstitial lung disease (ILD), followed by infection and pulmonary hypertension (PH), were documented in 46 (69.7%), 16 (24.2%) and 9 (13.6%) patients, respectively. Nine patients had microbiologically confirmed pulmonary tuberculosis. Patients who were anti-Jo1 antibody positive (n=16) had higher levels of acute inflammatory markers and muscle enzymes compared with the rest of the patients (p<0.0001). Dyspnoea and bibasal crackles were associated with significantly lower baseline and 12-month lung function parameters. Nonspecific interstitial pneumonia was the most common radiological pattern of ILD, present in 25 (62.5%) of the patients with ILD.
ILD was the most prevalent complication in this study of SA patients with IIMs. Pulmonary infections and PH were also significant contributors to morbidity. The presence of dyspnoea and crackles was predictive of lower baseline PFTs in this population.
Pulmonary complications, including interstitial lung disease (ILD) and infections, are significant contributors to morbidity and mortality in patients with idiopathic inflammatory myopathies (IIMs). There is very little research currently available to describe the spectrum of pulmonary manifestations in these patients in an African setting, a lack that this study aimed to address. ILD was the most common pulmonary complication in patients with IIMs in this cohort. Signs and symptoms of ILD may be present before symptoms of myositis, and dyspnoea and a dry cough were shown to be predictive of reduced lung volumes. Patients with IIMs on immunosuppressive therapy in our setting are at high risk of infection, particularly tuberculosis.
肺部并发症在特发性炎性肌病（IIM）患者中可导致显著的发病率和死亡率。
描述南非（SA）IIM患者肺部并发症的发生率及范围。
对在SA一家三级医疗机构就诊、有呼吸相关主诉的成年IIM患者或临床无肌病性皮肌炎（CADM）患者进行回顾性病历审查。记录临床特征、实验室及肺功能测试（PFT）结果、影像学表现及治疗情况。
66例患者记录有肺部并发症。多数患者（n = 41；62.1%）为皮肌炎，14例（21.2%）为多发性肌炎，3例（4.5%）为CADM。有8例患者为重叠综合征。呼吸困难和干咳是最常见的首发症状，分别见于52例（78.8%）和36例（54.5%）患者。38例患者（57.6%）可闻及双肺底湿啰音。分别有46例（69.7%）、16例（24.2%）和9例（13.6%）患者记录有间质性肺疾病（ILD）、感染和肺动脉高压（PH）。9例患者经微生物学证实患有肺结核。抗Jo1抗体阳性患者（n = 16）与其余患者相比，急性炎症标志物和肌酶水平更高（p < 0.0001）。呼吸困难和双肺底湿啰音与基线及12个月时显著更低的肺功能参数相关。非特异性间质性肺炎是ILD最常见的影像学表现，见于25例（62.5%）ILD患者。
在这项针对SA IIM患者的研究中，ILD是最常见的并发症。肺部感染和PH也是发病的重要因素。呼吸困难和湿啰音的存在可预测该人群较低的基线PFT值。
包括间质性肺疾病（ILD）和感染在内的肺部并发症是特发性炎性肌病（IIM）患者发病和死亡的重要因素。目前在非洲背景下，关于这些患者肺部表现范围的研究非常少，本研究旨在填补这一空白。在该队列中，ILD是IIM患者最常见的肺部并发症。ILD的体征和症状可能在肌炎症状出现之前就已存在，且呼吸困难和干咳被证明可预测肺容积减少。在我们的环境中，接受免疫抑制治疗的IIM患者感染风险高，尤其是肺结核。
