SUSPECT PULMONARY FIBROSIS
You may see patients with connective tissue disorders (CTDs) who develop ILD, including pulmonary fibrosis. Interstitial lung disease (ILD) is a common early manifestation of CTDs which shares common pathogenic pathways to fibrogenesis.2–8
DISCOVER HOW AND WHY IDENTIFYING FIBROTIC CTD-ILDs MATTERS
Screening for ILD in CTDs
Diagnosis of CTD-ILDs
Increased mortality rates
Screening for ILD in RA
Management of SSc-ILD
SPIKES model: breaking bad news to your patients
Early and regular monitoring for ILD progression in CTD-ILDs
CTD-ILD patient cases for screening and diagnosis
Management of progressive fibrosing CTD-ILDs
Footnotes
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CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.
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Vacchi C, Sebastiani M, Cassone G, et al. Therapeutic options for the treatment of interstitial lung disease related to connective tissue diseases. A narrative review. J Clin Med. 2020;9(2):407. doi: 10.3390/jcm9020407
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Koo SM, Kim SY, Choi SM, et al. Korean guidelines for diagnosis and management of interstitial lung diseases: part 5. Connective tissue disease associated interstitial lung disease. Tuberc Respir Dis (Seoul). 2019;82(4):285–297.
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Hoffmann-Vold AM, Allanore Y, Alves M, et al. Progressive interstitial lung disease in patients with systemic sclerosis- associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2020. Epub ahead of print: doi:10.1136/annrheumdis-2020-217455.
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Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheum. 2020;2 e71–e83.
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Distler O, Volkmann ER, Hoffmann-Vold AM, et al. Current and future perspectives on management of systemic sclerosis-associated interstitial lung disease. Expert Rev Clin Immunol. 2019;15:1009–1017.
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Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.
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Wells AU, Denton CP. Interstitial lung disease in connective tissue disease—mechanisms and management. Nat Rev Rheumatol. 2014;10:728–739.
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Geerts S, Wuyts W, De Langhe E, et al. Connective tissue disease associated interstitial pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(4):326–335.