SUSPECT PULMONARY FIBROSIS
Interstitial lung disease (ILD) is a common early manifestation of CTDs which shares common pathogenic pathways to fibrogenesis.1-7 You may see patients with connective tissue diseases (CTDs) who develop ILD, including pulmonary fibrosis.

DISCOVER WHY AND HOW IDENTIFYING FIBROTIC CTD-ILDs MATTERS

Screening for ILD in CTDs
Screening for CTD-ILD helps identify early-stage disease1

Diagnosis of CTD-ILDs
Any patient with known or suspected CTD should undergo workup including chest HRCT3

Increased mortality rates
ILD is a key driver of early mortality in patients with CTDs1-5,8

Screening for ILD in RA
RA patients with pulmonary symptoms and/or risk factors for ILD should be screened for ILD3,9

Management of SSc-ILD
Treatment initiation should aim for prevention of progression to avoid irreversible lung damage10-12

SPIKES model: breaking bad news to your patients
Professor Walter Baile explains through the SPIKES model on how to structure difficult conversations

Early and regular monitoring for ILD progression in CTD-ILDs
It is important to detect the progressive fibrosing phenotype as soon as possible13,14

CTD-ILD patient cases for screening and diagnosis
Download the patient cases: medical histories of representative patients

Management of progressive fibrosing CTD-ILDs
Treatment of progressive CTD-ILD should not wait15,16
Footnotes
- 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.
- Fischer A and Distler J. Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases. Clin Rheumatol. 2019;38(10):2673–2681.
- Mathai SC and Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ. 2016;352:h6819.
- 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.
- Spagnolo P, Cordier JF, Cottin V. Connective tissue diseases, multimorbidity and the ageing lung. Eur Respir J. 2016;47(5):1535–1558.
- 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
- 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.
- Doyle TJ, Hunninghake GM, Rosas IO. Subclinical Interstitial Lung Disease. Am J Respir Crit Care Med. 2012;185:1147–1153.
- Maher TM, Wuyts W. Management of fibrosing interstitial lung diseases. Adv Ther. 2019;36(7):1518–1531.
- Esposito AJ, Chu SG, Madan R, et al. Thoracic manifestations of rheumatoid arthritis. Clin Chest Med. 2019;40(3):545–560.
- 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.
- 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.
- 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.
- 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
- Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.
- Geerts S, Wuyts W, de Langhe E, et al. Connective tissue disease associated interstital pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Dif. 2017;34:326–335.
- 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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