Crestani B, Schneider S, Adle-Biassette H, Debray M P, Bonay M, Aubier M
Service de Pneumologie A, APHP, Hôpital Bichat, Paris, France.
Rev Mal Respir. 2007 Apr;24(4 Pt 1):535-51. doi: 10.1016/s0761-8425(07)91575-2.
Sjogren's syndrome is a common auto-immune disease.
Clinically significant pulmonary involvement affects approximately 10% of patients and may be the first manifestation of the disease, putting the respiratory physician in a position to suspect and confirm the diagnosis. Besides interstitial lung disease and bronchial disorders, cough is a common symptom of the disease and particularly difficult to treat. Lung cysts and amyloid deposits, sometimes associated with lymphoma, have recently been described. The development of a primary pulmonary lymphoma, usually from MALT, is a major complication of the disease.
Characterisation of the pathophysiology of pulmonary involvement in Sjogren's syndrome and the institution of specific treatment merits the interest of the respiratory physician.
The respiratory physician should consider the diagnosis of Sjogren's syndrome in many different clinico-pathological situations.
干燥综合征是一种常见的自身免疫性疾病。
具有临床意义的肺部受累影响约10%的患者,且可能是该疾病的首发表现,使呼吸内科医生能够怀疑并确诊。除间质性肺疾病和支气管疾病外,咳嗽是该疾病的常见症状且尤其难以治疗。肺囊肿和淀粉样沉积物,有时与淋巴瘤相关,最近已有描述。原发性肺淋巴瘤的发生,通常源自黏膜相关淋巴组织(MALT),是该疾病的主要并发症。
对干燥综合征肺部受累的病理生理学特征进行描述以及开展特异性治疗值得呼吸内科医生关注。
呼吸内科医生应在多种不同的临床病理情况下考虑干燥综合征的诊断。