Ananyeva L P
V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
Ter Arkh. 2017;89(5):4-9. doi: 10.17116/terarkh20178954-9.
Damage to the respiratory system worsens prognosis and increases mortality in all systemic immunoinflammatory rheumatic diseases (SIRDs). Leading pulmonary manifestations in each disease are different, so the typical picture of each nosological entity is very peculiar. In all SIRDs, the disease starts with lung involvement in 10-20% of cases, preceding the expanded clinical presentation of a systemic disease. At a certain stage, lung damage may be the only manifestation of the disease or predominate in its clinical picture, determining the severity of a condition. Among the patients referred to consultation with a pulmonologist for interstitial lung disease, about 15% have manifestations of SIRDs that is confirmed by a special survey. The paper discusses the diagnostic features of SIRDs with lung involvement, as well as indications for consultation with a rheumatologist. The key clinical symptoms indicating a possible rheumatic disease in patients with lung involvement are considered. Evidence for the main circulating autoantibodies identifiable in high titers in patients with SIRDs in a serological test is given and its positivity is interpreted. The issues associated with the term 'undifferentiated connective tissue diseases-associated interstitial lung disease' are discussed; the expediency of using the term 'interstitial pneumonia with autoimmune signs (AIS)' is emphasized. The clinical characteristics of and classification criteria for AIS are presented. The prognosis and outcomes of interstitial pneumonias of varying etiologies, as well as the importance of the multidisciplinary approach to managing patients with SIRDs and lung involvement, which improves the quality of diagnosis, adequacy of treatment, and quality of life, are dealt with.
呼吸系统损伤会使所有系统性免疫炎症性风湿病(SIRDs)的预后恶化并增加死亡率。每种疾病的主要肺部表现各不相同,因此每个疾病实体的典型症状都非常独特。在所有SIRDs中,10%-20%的病例疾病始于肺部受累,早于系统性疾病的全面临床表现。在某个阶段,肺部损伤可能是疾病的唯一表现或在临床表现中占主导地位,决定病情的严重程度。在因间质性肺病转诊至肺科医生处咨询的患者中,约15%有SIRDs的表现,这通过特殊检查得以证实。本文讨论了伴有肺部受累的SIRDs的诊断特征以及转诊至风湿病科医生的指征。考虑了提示肺部受累患者可能患有风湿性疾病的关键临床症状。给出了在血清学检测中SIRDs患者可高滴度识别的主要循环自身抗体的证据并对其阳性结果进行了解释。讨论了与“未分化结缔组织病相关间质性肺病”这一术语相关的问题;强调了使用“自身免疫征象间质性肺炎(AIS)”这一术语的合理性。介绍了AIS的临床特征和分类标准。探讨了不同病因间质性肺炎的预后和结局,以及多学科方法对管理伴有肺部受累的SIRDs患者的重要性,这可提高诊断质量、治疗的充分性和生活质量。