Background/Purpose: Interstitial lung disease (ILD) is a leading cause of mortality in systemic sclerosis (SSc). Early detection and treatment of SSc-ILD may lead to improved outcomes, however no formal recommendation exists to guide management in subclinical SSc-ILD. We undertook an international survey to understand current screening and treatment practices in subclinical SSc-ILD.

Methods: An electronic REDCap survey was distributed to 611 general rheumatologists, 348 national and international SSc experts, 285 respirologists and 15 experts in ILD.

Results: Up until April 8th, 2021, 178 participants responded to the survey, including 131 (76%) general rheumatologists and 32 (19%) respirologists. The estimated response rate was 23% among general rheumatologists, 25% among SSc experts, 8% among respirologists and 23% among ILD experts. The majority saw SSc-ILD patients at least monthly (79%) and had been in practice for at least 5 years (80%).

Overall, 59% of respondents reported routinely ordering HRCTs in all newly diagnosed SSc patients, whereas 40% ordered HRCTs only in the presence of symptoms, crackles or abnormal pulmonary function tests or chest X-ray (35%) and/or in the presence of risk factors for severe or progressive ILD (17%). There was significant regional heterogeneity in screening practices, with HRCTs being routinely ordered by 48% (40/84) in North America, 83% (29/35) in Europe, 70% (7/10) in Asia, 40% (2/5) in Australia and 100% (4/4) in Latin America.

Six-minute walk tests (6MWT) were ordered by 34% of respondents in the baseline evaluation of subclinical SSc-ILD, and more frequently by respirologists (72%) than general rheumatologists (24%). 6MWT were more rarely used in the routine follow-up of SSc-ILD (33%), with most (57%) respondents using 6MWT only if there was disease progression. Factors that influenced 6MWT use included access/availability, symptoms, pulmonary function tests and extent of ILD on CT, risk factors for progressive ILD, suspicion of hypoxemia on ambulation or concomitant pulmonary hypertension, and presence of other SSc manifestations that could affect test validity (including severe Raynaud, skin or musculoskeletal disease).

Nearly half (47%) of participants responded that they would not treat subclinical lung disease, 17% would treat and 36% responded that it would depend. Factors that would influence their decision included risk factors for progressive lung disease, other SSc manifestations requiring treatment (e.g. progressive/diffuse skin disease, myositis), disease trajectory over close follow-up, comorbidities/life expectancy, discussions with the treating respirologist, and shared decision-making with the patient regarding risks and benefits. The majority (75%) of respirologists would not treat subclinical lung disease.

Conclusion: There is considerable variability in screening and treatment practices in subclinical SSc-ILD. Further studies are required to define the impact of screening and treatment strategies on clinical outcomes in subclinical SSc-ILD, including controlled trials.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/survey-on-treatment-practices-in-subclinical-interstitial-lung-disease-in-systemic-sclerosis/