Autopsy and Case Reports (Dec 2014)

Severe cognitive dysfunction and shrinking lung syndrome in systemic lupus erythematous

  • Breno José Alencar Pires Barbosa,
  • Francisco Akira Malta Cardozo,
  • João Francisco Figueiredo Marcondes Ferraz,
  • Jairo Rays,
  • Márcia Yoshie Kanegae,
  • Vilma Takayasu

DOI
https://doi.org/10.4322/acr.%y.98468
Journal volume & issue
Vol. 4, no. 4

Abstract

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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.

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