Pulmonary Manifestations of Autoinflammatory Disorders

  • Ahmadreza Jamshidi
  • Saeed Aslani
  • Mahdi MahmoudiEmail author


Respiratory complications of autoinflammatory disorders (AID) are known as a significant cause of morbidity and mortality in the world. Rheumatologic complications, particularly, have been seen in patients with interstitial lung disease in remarkable rates. Among the most frequently associated rheumatologic AIDs with pulmonary complications are systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), polymyositis-dermatomyositis (PM-DM), Sjogren’s syndrome (SjS), ankylosing spondylitis (AS), Behçet’s disease (BD), autoimmune pancreatitis, Wegener granulomatosis, Churg-Strauss syndrome, adult-onset Still’s disease (AOSD), familial Mediterranean fever (FMF), inflammatory bowel disease (IBD), Cherubism, and chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome. With respect to clinical manifestations, initiation of pulmonary involvement in these disorders is different. In some patients, the pulmonary complications occur before the AID or are a manifestation after disease development. Furthermore, there is a wide range of clinical manifestations in these diseases that can be moderate as subclinical abnormalities to more severe forms like acute respiratory failure. Regarding histopathological complications, the main specification of pulmonary involvement in AID is inflammation in one or both lungs. The inflammatory reactions can be acute to chronic, in which remodeling of the lungs by fibrosis is commonly seen. Most of the times, the inflammatory outcomes are not specific and certain, but a distinctive series of responses or anatomic involvement may be seen in particular AID. Moreover, unique histopathological manifestations such as rheumatoid nodules may be observed. Herein, we determined to have an overview on different aspects of common pulmonary pathologic complications of AID with respect to clinical and radiologic observations.


Autoimmune disorders Pulmonary manifestations 


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Ahmadreza Jamshidi
    • 1
  • Saeed Aslani
    • 1
  • Mahdi Mahmoudi
    • 1
    Email author
  1. 1.Rheumatology Research CenterTehran University of Medical Sciences (TUMS)TehranIran

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