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Triboulet, Félicien; Guérin, Emmanuelle; Boussouar, Samia; Hékimian, Guillaume; Pha, Micheline; Rouvier, Philippe; Mathian, Alexis; Quentric, Paul; Moyon, Quentin; Hié, Miguel; Schmidt, Matthieu; Combes, Alain; Luyt, Charles-Edouard; Amoura, Zahir; Pineton de Chambrun, Marc

Systemic lupus erythematosus-related acute lung disease

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  • Media type: E-Article
  • Title: Systemic lupus erythematosus-related acute lung disease
  • Contributor: Triboulet, Félicien; Guérin, Emmanuelle; Boussouar, Samia; Hékimian, Guillaume; Pha, Micheline; Rouvier, Philippe; Mathian, Alexis; Quentric, Paul; Moyon, Quentin; Hié, Miguel; Schmidt, Matthieu; Combes, Alain; Luyt, Charles-Edouard; Amoura, Zahir; Pineton de Chambrun, Marc
  • imprint: SAGE Publications, 2023
  • Published in: Lupus
  • Language: English
  • DOI: 10.1177/09612033231188034
  • ISSN: 0961-2033; 1477-0962
  • Keywords: Rheumatology
  • Origination:
  • Footnote:
  • Description: <jats:sec><jats:title>Introduction</jats:title><jats:p> Systemic lupus erythematosus (SLE) is non-organ specific autoimmune disease with mainly skin, joint, and kidney involvement. SLE-related acute lung disease (ALD) is rare, poorly investigated and can lead to acute respiratory failure. We conducted a retrospective study aiming to describe clinical features, treatments and outcome of SLE-related APD. </jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p> We retrospectively included all patients with SLE and ALD admitted from November 1996 and September 2018 to La Pitié-Salpêtrière Hospital, after exclusion of viral or bacterial lung infection, cardiac failure or any other alternate diagnosis. </jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> During the time of the study, 14 patients with 16 episodes were admitted to our center: female 79%, mean age ± SD at admission 24 ± 11 years. ALD was inaugural of the SLE in 70% cases. SLE main organ involvement were: arthritis 93%, skin 79%, serositis 79%, hematological 79%, kidney 64%, neuropsychiatric 36% and cardiac 21%. 11 episodes required ICU admission for a median time of 8 days. Chest CT-scan revealed mostly basal consolidation and ground-glass opacities. When available, bronchoalveolar lavage mostly revealed a neutrophilic alveolitis with alveolar hemorrhage in 67% cases. Symptomatic respiratory treatments were: oxygen 81%, high-flow nasal canula oxygen 27%, non-invasive ventilation 36%, mechanical ventilation 64% and venovenous extracorporeal membrane oxygenation 18%. SLE-specific treatments were: corticosteroids 100%, cyclophosphamide 56% and plasma exchange 25%. All patients but one survived to ICU and hospital discharge. Two patients had a relapse of SLE-related ALD but none had interstitial lung disease during follow-up. </jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p> Systemic lupus erythematosus-related acute respiratory failure is a severe event, mostly occurring at SLE onset, typical harboring a basal consolidation pattern on chest CT-scan and alveolar hemorrhage on BAL pathological examination. Mortality in our cohort is lower than previously reported but these results needs to be confirmed in further larger studies. </jats:p></jats:sec>