PDF(Pubmed)
Abstract：
Usual Interstitial Pneumonia (UIP) is characterized by progression of lung parenchyma that may be observed in various autoimmune rheumatic diseases (ARDs), including rheumatoid arthritis and connective tissue diseases. From a diagnostic point of view, a UIP pattern related to ARDs may display imaging and pathological features able to distinguish it from that related to IPF, such as the \"straight-edge\" sign at HRCT and lymphoplasmacytic infiltrates at histologic specimens. Multidisciplinary approach (MDD), involving at least pulmonologist, rheumatologist and radiologist, is fundamental in the differential diagnosis process, but MDD is also required in the evaluation of severity, progression and response to treatment, that is based on the combination of changes in symptoms, pulmonary function trends, and, in selected patients, serial CT evaluation. Differently from IPF, in patients with ARDs both functional evaluation and patient-reported outcomes may be affected by systemic involvement and comorbidities, including musculoskeletal manifestations of disease. Finally, in regards to pharmacological treatment, immunosuppressants have been considered the cornerstone of therapy, despite the lack of solid evidence in most cases; recently, antifibrotic drugs were also proposed for the treatment of progressive fibrosing ILDs other than IPF. In ARD-ILD, the therapeutic choice should balance the need for the control of systemic and lung involvements with the risk of adverse events from multi-morbidities and -therapies. Purpose of this review is to summarize the definition, the radiological and morphological features of the UIP pattern in ARDs, together with risk factors, diagnostic criteria, prognostic evaluation, monitoring and management approaches of the UIP-ARDs.
摘要：
通常的间质性肺炎（UIP）的特征是肺实质的进展，可以在各种自身免疫性风湿性疾病（ARD）中观察到，包括类风湿性关节炎和结缔组织疾病。从诊断的角度来看，与ARD相关的UIP模式可以显示能够区分其与IPF相关的成像和病理特征，如HRCT的“直缘”标志和组织学标本的淋巴浆细胞浸润。多学科方法(MDD)至少包括肺科医生，风湿病学家和放射科医生,是鉴别诊断过程的基础，但在严重程度评估中也需要MDD，进展和对治疗的反应，这是基于症状变化的组合，肺功能趋势，and,在选定的患者中，串行CT评估。与IPF不同，在ARDs患者中，功能评估和患者报告的结局都可能受到全身受累和合并症的影响，包括肌肉骨骼疾病的表现。最后,关于药物治疗，免疫抑制剂被认为是治疗的基石，尽管在大多数情况下缺乏确凿的证据；最近，抗纤维化药物也被提议用于治疗除IPF以外的进行性纤维化ILD.在ARD-ILD中,治疗选择应平衡控制全身和肺部受累的需要与多种疾病和治疗引起的不良事件的风险.这篇综述的目的是总结定义，ARD中UIP模式的放射学和形态学特征，连同风险因素，诊断标准,预后评估，UIP-ARD的监测和管理方法。
