Management of connective tissue disease-associated interstitial lung disease
The management of connective tissue diseases (CTD)-associated interstitial lung disease (ILD) is challenging considering its myriad presentations and variable course. Evidence to guide treatment is insufficient with very few randomized trials reported till date. This review discusses the current evi...
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SAGE Publishing
2021-01-01
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| Series: | Indian Journal of Rheumatology |
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| Online Access: | http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2021;volume=16;issue=5;spage=87;epage=91;aulast=Talari |
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| _version_ | 1832569166054293504 |
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| author | Keerthi Talari |
| author_facet | Keerthi Talari |
| author_sort | Keerthi Talari |
| collection | DOAJ |
| description | The management of connective tissue diseases (CTD)-associated interstitial lung disease (ILD) is challenging considering its myriad presentations and variable course. Evidence to guide treatment is insufficient with very few randomized trials reported till date. This review discusses the current evidence guiding management of CTD-ILD with a specific focus on corticosteroids, cyclophosphamide, mycophenolate, azathioprine, calcineurin inhibitors, and pirfenidone. Corticosteroids (except in systemic sclerosis [SSc]-ILD) in combination with immunosuppressants form the mainstay of therapy in CTD-ILD. Cyclophosphamide and mycophenolate mofetil are backed by randomised trials-Scleroderma lung study I and II respectively for SSc-ILD, while calcineurin inhibitors with or without cyclophosphamide have emerged as a promising initial therapy in myositis associated ILD. Rituximab is probably beneficial for all forms of CTD-ILD even as randomized trial data are awaited. General measures such as lifestyle modification, pulmonary rehabilitation, vaccination, prophylaxis, supplemental oxygen, palliative care are also of paramount importance. Future research should focus on long-term efficacy, safety and head-to-head comparison of drugs, role of upfront combination therapies, supportive and palliative care. |
| format | Article |
| id | doaj-art-07661e7237a34d55b1a6881365237fc2 |
| institution | Kabale University |
| issn | 0973-3698 0973-3701 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Indian Journal of Rheumatology |
| spelling | doaj-art-07661e7237a34d55b1a6881365237fc22025-02-02T22:47:46ZengSAGE PublishingIndian Journal of Rheumatology0973-36980973-37012021-01-01165879110.4103/0973-3698.332982Management of connective tissue disease-associated interstitial lung diseaseKeerthi TalariThe management of connective tissue diseases (CTD)-associated interstitial lung disease (ILD) is challenging considering its myriad presentations and variable course. Evidence to guide treatment is insufficient with very few randomized trials reported till date. This review discusses the current evidence guiding management of CTD-ILD with a specific focus on corticosteroids, cyclophosphamide, mycophenolate, azathioprine, calcineurin inhibitors, and pirfenidone. Corticosteroids (except in systemic sclerosis [SSc]-ILD) in combination with immunosuppressants form the mainstay of therapy in CTD-ILD. Cyclophosphamide and mycophenolate mofetil are backed by randomised trials-Scleroderma lung study I and II respectively for SSc-ILD, while calcineurin inhibitors with or without cyclophosphamide have emerged as a promising initial therapy in myositis associated ILD. Rituximab is probably beneficial for all forms of CTD-ILD even as randomized trial data are awaited. General measures such as lifestyle modification, pulmonary rehabilitation, vaccination, prophylaxis, supplemental oxygen, palliative care are also of paramount importance. Future research should focus on long-term efficacy, safety and head-to-head comparison of drugs, role of upfront combination therapies, supportive and palliative care.http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2021;volume=16;issue=5;spage=87;epage=91;aulast=Talaricyclophosphamideconnective tissue diseaseinterstitial lung diseasemycophenolate mofetiltreatment |
| spellingShingle | Keerthi Talari Management of connective tissue disease-associated interstitial lung disease Indian Journal of Rheumatology cyclophosphamide connective tissue disease interstitial lung disease mycophenolate mofetil treatment |
| title | Management of connective tissue disease-associated interstitial lung disease |
| title_full | Management of connective tissue disease-associated interstitial lung disease |
| title_fullStr | Management of connective tissue disease-associated interstitial lung disease |
| title_full_unstemmed | Management of connective tissue disease-associated interstitial lung disease |
| title_short | Management of connective tissue disease-associated interstitial lung disease |
| title_sort | management of connective tissue disease associated interstitial lung disease |
| topic | cyclophosphamide connective tissue disease interstitial lung disease mycophenolate mofetil treatment |
| url | http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2021;volume=16;issue=5;spage=87;epage=91;aulast=Talari |
| work_keys_str_mv | AT keerthitalari managementofconnectivetissuediseaseassociatedinterstitiallungdisease |