Tuberc Respir Dis.  2019 Oct;82(4):285-297. 10.4046/trd.2019.0009.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease

Affiliations
  • 1Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea.
  • 2Division of Pulmonology, Department of Internal Medicine, Severance Hospital, Institute of Chest Diseases, Yonsei University College of Medicine, Seoul, Korea.
  • 3Division of Pulmonary and Critical Care Medicine, Department of Internal Meidicine, Seoul National University Hospital, Seoul, Korea. sunmich81@gmail.com
  • 4Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Korea. goodoc@gmail.com

Abstract

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the co-occurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjögren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.

Keyword

Lung Disease, Interstitial; Connective Tissue Disease; Asian Continental Ancestry Group; Guidelines as Topic; Diagnosis; Disease Management; Lung

MeSH Terms

Arthritis, Rheumatoid
Asian Continental Ancestry Group
Autoantibodies
Connective Tissue Diseases*
Connective Tissue*
Diagnosis*
Disease Management
Guidelines as Topic
Humans
Idiopathic Interstitial Pneumonias
Incidence
Lung
Lung Diseases, Interstitial*
Myositis
Prognosis
Scleroderma, Systemic
Thorax
Autoantibodies
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