Lethrosne C, Pernot B, Diot P, Diot E
Inserm U1100/EA6305, service de pneumologie, hôpital Bretonneau, CHRU et université François-Rabelais de Tours, centre d'étude des pathologies respiratoires, 37044 Tours, France.
Rev Mal Respir. 2013 Apr;30(4):262-71. doi: 10.1016/j.rmr.2012.12.015. Epub 2013 Feb 8.
Cardiopulmonary involvement in adult-onset Still's disease is not as common as cutaneous and articular involvement. Pleuropericarditis is the most frequent thoracic manifestation. Although difficult, diagnosis of other thoracic manifestations, which may reveal the disease, is crucial, due to the high risk to life and the efficacy of new immunosuppressive agents. The pathophysiology involves essentially immunological factors, Still's disease being increasingly seen as an autoimmune inflammatory disease. Pro-inflammatory cytokines such as interleukine (IL) 1, 6 and 18 play a crucial role in macrophage activation, which is central in the pathophysiology of adult Still's disease. The classification of cardiopulmonary manifestations is based on anatomy. Cardiac lesions may involve all the tissues of the heart and the pulmonary arteries. Respiratory lesions may involve the pleura, the lung parenchyma (organizing pneumonitis, infiltrative lung disease, alveolar damage, amyloidosis), and the respiratory muscles, including the diaphragm. Finally, some manifestations may be provoked by the treatment itself. Steroids, the first-line treatment, are very effective in pleuropericarditis. Methotrexate used to be prescribed when steroids failed, but biotherapies such as IL1 and IL6 inhibitors have transformed the prognosis of forms resistant to these drugs.
成人斯蒂尔病的心肺受累不如皮肤和关节受累常见。胸膜炎性心包炎是最常见的胸部表现。尽管困难，但由于存在生命危险以及新型免疫抑制剂的疗效，诊断其他可能揭示该疾病的胸部表现至关重要。其病理生理学主要涉及免疫因素，斯蒂尔病越来越被视为一种自身免疫性炎症性疾病。促炎细胞因子如白细胞介素（IL）1、6和18在巨噬细胞激活中起关键作用，而巨噬细胞激活在成人斯蒂尔病的病理生理学中至关重要。心肺表现的分类基于解剖结构。心脏病变可能累及心脏的所有组织以及肺动脉。呼吸病变可能累及胸膜、肺实质（机化性肺炎、浸润性肺病、肺泡损伤、淀粉样变性）以及呼吸肌，包括膈肌。最后，一些表现可能由治疗本身引发。作为一线治疗的类固醇对胸膜炎性心包炎非常有效。过去在类固醇治疗失败时会使用甲氨蝶呤，但诸如IL1和IL6抑制剂等生物疗法已经改变了对这些药物耐药的类型的预后。
