Severe cognitive dysfunction and shrinking lung syndrome in systemic lupus erythematous

• Published in: Autopsy & case reports Vol. 4; no. 4; pp. 63 - 69
• Main Authors: Barbosa, Breno José Alencar Pires, Cardozo, Francisco Akira Malta, Ferraz, João Francisco Figueiredo Marcondes, Rays, Jairo, Kanegae, Márcia Yoshie, Takayasu, Vilma
• Format: Journal Article
• Language: English
• Published: Brazil São Paulo, SP: Universidade de São Paulo, Hospital Universitário 01.10.2014
• Subjects: Artigo / Relato de Caso Clínico; Clinical Case Reports; Cognition Disorders; Lupus Erythematosus, Systemic; Lung Diseases
• ISSN: 2236-1960; 2236-1960
• DOI: 10.4322/acr.2014.041

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.