Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review

Jae Il Shin, Keum Hwa Lee, Seoyeon Park, Jae Won Yang, Hyung Ju Kim, Kwanhyuk Song, Seungyeon Lee, Hyeyoung Na, Yong Jun Jang, Ju Yun Nam, Soojin Kim, Chaehyun Lee, Chanhee Hong, Chohwan Kim, Minhyuk Kim, Uichang Choi, Jaeho Seo, Hyunsoo Jin, Bo Mi Yi, Se Jin JeongYeon Ook Sheok, Haedong Kim, Sangmin Lee, Sangwon Lee, Young Soo Jeong, Se Jin Park, Ji Hong Kim, Andreas Kronbichler

Research output: Contribution to journalReview articlepeer-review


Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multiorgan manifestations, including pleuropulmonary involvement (20–90%). The precise mechanism of pleuropulmonary involvement in SLE is not well-understood; however, systemic type 1 interferons, circulating immune complexes, and neutrophils seem to play essential roles. There are eight types of pleuropulmonary involvement: lupus pleuritis, pleural effusion, acute lupus pneumonitis, shrinking lung syndrome, interstitial lung disease, diffuse alveolar hemorrhage (DAH), pulmonary arterial hypertension, and pulmonary embolism. DAH has a high mortality rate (68–75%). The diagnostic tools for pleuropulmonary involvement in SLE include chest X-ray (CXR), computed tomography (CT), pulmonary function tests (PFT), bronchoalveolar lavage, biopsy, technetium-99m hexamethylprophylene amine oxime perfusion scan, and (18)F-fluorodeoxyglucose positron emission tomography. An approach for detecting pleuropulmonary involvement in SLE includes high-resolution CT, CXR, and PFT. Little is known about specific therapies for pleuropulmonary involvement in SLE. However, immunosuppressive therapies such as corticosteroids and cyclophosphamide are generally used. Rituximab has also been successfully used in three of the eight pleuropulmonary involvement forms: lupus pleuritis, acute lupus pneumonitis, and shrinking lung syndrome. Pleuropulmonary manifestations are part of the clinical criteria for SLE diagnosis. However, no review article has focused on the involvement of pleuropulmonary disease in SLE. Therefore, this article summarizes the literature on the epidemiology, pathogenesis, diagnosis, and management of pleuropulmonary involvement in SLE.

Original languageEnglish
Article number6714
JournalJournal of Clinical Medicine
Issue number22
Publication statusPublished - 2022 Nov

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All Science Journal Classification (ASJC) codes

  • Medicine(all)


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