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
![HME Hero image](/sites/default/files/2021-11/Hero-Rheums-desktop-1280x495px%20HME_1.jpg)
DISCOVER HOW AND WHY IDENTIFYING FIBROTIC CTD-ILDs MATTERS
![Word cloud of terms associated with ILD identification.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9%402x_9.png)
Screening for ILD in CTDs
![mdt](/sites/default/files/2021-11/MDT%20placeholder%20image%20.jpg)
Diagnosis of CTD-ILDs
![Increased mortality rates](/sites/default/files/2021-11/01-elements-placeholder-card-16-9%403x_1.jpg)
Increased mortality rates
![Screening for ILD in RA](/sites/default/files/2021-11/01-elements-placeholder-card-16-9%403x_13.jpg)
Screening for ILD in RA
![Management of SSc-ILD](/sites/default/files/2021-11/01-elements-placeholder-card-16-9%403x_19.jpg)
Management of SSc-ILD
![Spikes](/sites/default/files/2021-11/01-elements-placeholder-card-16-9%403x_18.jpg)
SPIKES model: breaking bad news to your patients
![The first step to slowing progressive CTD-ILDs is identification of ILD](/sites/default/files/2021-11/01-elements-placeholder-card-16-9%403x_4.jpg)
Early and regular monitoring for ILD progression in CTD-ILDs
![card image](/sites/default/files/2021-11/01-elements-placeholder-card-16-9.jpg)
CTD-ILD patient cases for screening and diagnosis
![Progressive CTD-ILD](/sites/default/files/2021-11/Card-Image-Progressive%20fib%20CTD-ILDs%402x.png)
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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Esposito AJ, Chu SG, Madan R, et al. Thoracic manifestations of rheumatoid arthritis. Clin Chest Med. 2019;40(3):545–560.
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Fischer A and Distler J. Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases. Clin Rheumatol. 2019;38(10):2673–2681.
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Mathai SC and Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ. 2016;352:h6819.
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Wallace B, Vummidi D, Khanna D. Management of connective tissue diseases associated interstitial lung disease: a review of the published literature. Curr Opin Rheumatol. 2016;28(3):236–245.
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Spagnolo P, Cordier JF, Cottin V. Connective tissue diseases, multimorbidity and the ageing lung. Eur Respir J. 2016;47(5):1535–1558.
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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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Doyle TJ, Hunninghake GM, Rosas IO. Subclinical Interstitial Lung Disease. Am J Respir Crit Care Med. 2012;185:1147–1153.
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Maher TM, Wuyts W. Management of fibrosing interstitial lung diseases. Adv Ther. 2019;36(7):1518–1531.
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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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Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254
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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.
Resources for patients
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![Life with Pulmonary Fibrosis website logo.](/sites/default/files/2021-07/logo-primary_0.png)
![Scleroderma female patient looking into the window.](/sites/default/files/2021-07/image_0.png)
![More then scleroderma website logo.](/sites/default/files/2021-07/logo-primary_1.png)