Barbosa Breno José Alencar Pires, Cardozo Francisco Akira Malta, Ferraz João Francisco Figueiredo Marcondes, Rays Jairo, Kanegae Márcia Yoshie, Takayasu Vilma
Department of Neurology - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.
Department of Internal Medicine - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.
Autops Case Rep. 2014 Mar 30;4(4):63-69. doi: 10.4322/acr.2014.041. eCollection 2014 Oct-Dec.
Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any organ or system. Neuropsychiatric and pulmonary involvement can occur in 40 and 50% of patients respectively, and may occur in several different clinical forms. While the main neuropsychiatric manifestations are represented by cognitive impairment, organic cerebral syndromes, delirium, psychosis, seizures, and peripheral neuropathies, the main forms of pulmonary involvement are pleurisy with or without pleural effusion, pneumonitis, interstitial disease, pulmonary hypertension, and alveolar hemorrhage. The authors report the case of a 49-year-old woman whose first manifestation of SLE was represented by two rare manifestations: rapidly progressive cognitive impairment, which was associated with respiratory failure caused by the shrinking lung syndrome. The authors call attention to the under-diagnosis of lupus pulmonary complications and its association with severe cognitive impairment that often necessitates aggressive treatment.
系统性红斑狼疮(SLE)是一种可累及任何器官或系统的自身免疫性疾病。神经精神和肺部受累分别可发生于40%和50%的患者中,且可能以几种不同的临床形式出现。虽然主要的神经精神表现为认知障碍、器质性脑综合征、谵妄、精神病、癫痫发作和周围神经病变,但肺部受累的主要形式为伴有或不伴有胸腔积液的胸膜炎、肺炎、间质性疾病、肺动脉高压和肺泡出血。作者报告了一例49岁女性患者,其SLE的首发表现为两种罕见表现:快速进展的认知障碍,这与肺萎陷综合征导致的呼吸衰竭相关。作者提醒注意狼疮肺部并发症的诊断不足及其与严重认知障碍的关联,后者往往需要积极治疗。