RT Journal Article
SR Electronic
T1 Lung complications of Sjogren syndrome
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 200021
DO 10.1183/16000617.0021-2020
VO 29
IS 157
A1 Fabrizio Luppi
A1 Marco Sebastiani
A1 Nicola Sverzellati
A1 Alberto Cavazza
A1 Carlo Salvarani
A1 Andreina Manfredi
YR 2020
UL http://err.ersjournals.com/content/29/157/200021.abstract
AB Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations, including those regarding the lung. Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders.Patients with pSS-ILD report impaired health-related quality of life and a higher risk of death, suggesting the importance of early diagnosis and treatment of this type of pulmonary involvement. In contrast, airway disease usually has little effect on respiratory function and is rarely the cause of death in these patients.More rare disorders can be also identified, such as pleural effusion, cysts or bullae.Up to date, available data do not allow us to establish an evidence-based treatment strategy in pSS-ILD. No data are available regarding which patients should be treated, the timing to start therapy and better therapeutic options. The lack of knowledge about the natural history and prognosis of pSS-ILD is the main limitation to the development of clinical trials or shared recommendations on this topic. However, a recent trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various ILDs, including those in pSS patients.Primary Sjogren syndrome is a systemic autoimmune disease with a possible lung involvement, that it appears as polymorphic, including interstitial lung disease, airway disease and lymphoproliferative disorders with different degree of severity https://bit.ly/3akjk4b