Background/Purpose: Interstitial lung disease (ILD) affects 40-60% of adults with systemic sclerosis (SSc) and is the leading cause of death in this population. Nintedanib, a tyrosine kinase inhibitor, was the first medication to receive Food & Drug Administration approval for the treatment of SSc-ILD, in September 2019, based on the results of the SENSCIS trial. The most common adverse event (AE) associated with nintedanib is gastrointestinal distress. Of the 288 participants who received nintedanib in the SENSCIS trial, 75.7% experienced diarrhea, 31.6% nausea, and 24.7% vomiting. Moreover, 16% of participants discontinued nintedanib due to an AE.¹ It is unknown how nintedanib is being used in clinical practice. The aims of this study were to describe the nintedanib prescribing practices at expert SSc centers in the US.

Methods: We performed a prospective cohort study of patients enrolled in the Collaborative National Quality and Efficacy Registry (CONQUER) at 13 sites in the US between June 6, 2018, and March 1, 2021. CONQUER is a US-based, prospective, multicenter cohort of adults with SSc who met 2013 ACR/EULAR Classification Criteria and had a disease duration ≤ 5 years from the 1st non-Raynaud’s symptom at enrollment. We compared baseline characteristics between participants with SSc-ILD who did and did not receive nintedanib. Descriptive data are presented due to the small sample size of subjects who received nintedanib.

Results: 459 SSc patients were enrolled in CONQUER, of whom 202 (44%) had ILD. Of the 202 participants with SSc-ILD, 14 (6.9%) had taken nintedanib at some point during their SSc-ILD disease course. Among those who received nintedanib compared to those who did not, a greater proportion were male (42.9% vs. 19.1%), Hispanic (21.4% vs. 10.8%), never smokers (78.6% vs. 67.0%), Scl-70 positive (50.0% vs. 35.6%), and had crackles on exam (71.4% vs. 32.6%), while a smaller percentage were centromere positive (0% vs. 10.1%) or RNA polymerase 3 positive (7.1% vs. 22.9%) (Table 1). The median baseline diarrhea scale score of the UCLA SCTC GIT 2.0 was in the “moderate” range for those who received nintedanib and in the “none-to-mild” range for those who did not. SSc patients who received nintedanib had lower baseline forced vital capacity, total lung capacity, and diffusion capacity for carbon monoxide than those who did not (Table 2). Of the 14 patients who received nintedanib, 10 (71.4%) have remained on it, 2 (14.3%) stopped and then restarted it, and 2 (14.3%) discontinued it. Twelve of the 14 (85.7%) patients who received nintedanib were concurrently receiving mycophenolate mofetil (MMF).

Conclusion: A relatively small percentage of patients with SSc-ILD enrolled in CONQUER have received nintedanib. The majority of patients who received nintedanib did so in combination with MMF. Most patients who received nintedanib were able to remain on this therapy. Future research should explore the tolerability of nintedanib in clinical practice, including reasons for discontinuation. Longitudinal registries such as CONQUER are critical for understanding SSc care in the US.

Reference:
1. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 2019;380:2518-28.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/use-of-nintedanib-for-the-treatment-of-systemic-sclerosis-associated-interstitial-lung-disease-at-expert-scleroderma-centers-in-the-united-states/