Division of Pulmonary and Critical Care Medicine, Clinical Immunology, and Allergy, David Geffen School of Medicine at UCLA, Los Angeles, California.
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
Semin Respir Crit Care Med. 2019 Apr;40(2):235-254. doi: 10.1055/s-0039-1688448. Epub 2019 May 28.
Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by mononuclear cells (principally lymphocytes) infiltrating exocrine glands (e.g., salivary and lacrimal glands), leading to destruction of exocrine epithelial cells and dryness of mucosal surfaces. Cardinal symptoms are dry eyes (xerophthalmia) and dry mouth (xerostomia). Extraglandular sites are affected in 30 to 40% of cases of SS (particularly neurological, kidneys, skin, and lungs). B cell hyperactivity, autoantibody production, and hypergammaglobulinemia are cardinal features of SS. Primary SS is not associated with other autoimmune diseases. However, SS can complicate diverse autoimmune disorders (particularly systemic lupus erythematosus, rheumatoid arthritis, and scleroderma); this form is termed "secondary SS." Pulmonary involvement is usually not a dominant feature of SS, but may be severe in some cases. In this review, we discuss specific tracheal, bronchiolar, and pulmonary complications of SS including xerotrachea, bronchiolitis, bronchiectasis, interstitial lung disease, nonspecific interstitial pneumonia, usual interstitial pneumonia, lymphoid interstitial pneumonia, organizing pneumonia, acute fibrinous and organizing pneumonia, pulmonary cysts, pleural effusions, pulmonary amyloidosis, and bronchus- or lung-associated lymphomas.
干燥综合征（SS）是一种慢性自身免疫性疾病，其特征为单个核细胞（主要为淋巴细胞）浸润外分泌腺（如唾液腺和泪腺），导致外分泌上皮细胞破坏和黏膜表面干燥。主要症状为眼干（干眼症）和口干（口干燥症）。30%至 40%的 SS 病例会出现腺外部位受累（特别是神经系统、肾脏、皮肤和肺部）。B 细胞过度活跃、自身抗体产生和高丙种球蛋白血症是 SS 的主要特征。原发性 SS 不与其他自身免疫性疾病相关。然而，SS 可使多种自身免疫性疾病复杂化（特别是系统性红斑狼疮、类风湿关节炎和硬皮病）；这种形式被称为“继发性 SS”。肺部受累通常不是 SS 的主要特征，但在某些情况下可能很严重。在本次综述中，我们讨论了 SS 特定的气管、细支气管和肺部并发症，包括干燥性气管、细支气管炎、支气管扩张、间质性肺病、非特异性间质性肺炎、寻常型间质性肺炎、淋巴样间质性肺炎、机化性肺炎、急性纤维性和机化性肺炎、肺囊肿、胸腔积液、肺淀粉样变性和支气管或肺相关淋巴瘤。
