Closing the Gap: Screening and Treatment Ownership in CTD-Associated ILD

The past decade has brought meaningful change to the management of interstitial lung disease (ILD) arising in the context of connective tissue diseases (CTDs), driven in large part by the development of effective pharmacologic therapies and the publication of the first joint screening guidelines from the American College of Rheumatology (ACR) and American College of Chest Physicians (CHEST) in 2024.¹˒² Antifibrotic agents — nintedanib, pirfenidone, and most recently nerandomilast (Jascayd) — have expanded the treatment armamentarium for progressive pulmonary fibrosis (PPF), with nerandomilast notable as the first agent combining antifibrotic and anti-inflammatory properties through selective phosphodiesterase 4B (PDE4B) inhibition.³ In the phase 3 FIBRONEER-ILD trial, nerandomilast significantly slowed FVC decline versus placebo in patients with PPF, with a more favorable gastrointestinal side effect profile than nintedanib when used without concomitant antifibrotic background therapy.⁴ The recognition that ILD affects a substantial subset of patients across multiple CTDs — carrying meaningful mortality implications, particularly in myositis and systemic sclerosis — has sharpened the urgency of early identification. Yet despite this progress, substantial variation persists in how rheumatologists screen for, monitor, and co-manage ILD across disease states and practice settings.

To explore these gaps, RheumatologyLive convened a peer-to-peer clinical forum in San Diego in March 2026. The discussion was moderated by Ara Dikranian, MD, a rheumatologist at Cabrillo Center for Rheumatic Disease with extensive experience in systemic sclerosis and lupus, and brought together a diverse group of community and academic rheumatologists from the San Diego region — representing practices ranging from large academic systems including UCSD and Scripps to solo and small-group community settings. The forum was intentionally discussion-driven, with an emphasis on capturing real-world practice patterns, clinical judgment, and the challenges that guidelines alone do not resolve.

RheumatologyLive caught up with Dikranian after the forum to go over some of the main takeaways discussed. View part of the conversation below:

The forum convened at a moment of active guideline evolution in CTD-ILD. The 2024 ACR/CHEST screening and treatment guidelines established a formal framework for the first time, but most recommendations remain conditional and rest on low-quality evidence.¹˒² The ERS/EULAR CTD-ILD clinical practice guidelines, published simultaneously in the European Respiratory Journal and Annals of the Rheumatic Diseases in September 2025, introduced updated, disease-specific recommendations on screening, monitoring, and treatment sequencing — including support for combination immunosuppressant and antifibrotic therapy in patients with PPF — and drew direct discussion during the forum.⁵˒⁶ The timing also coincided with the December 2025 FDA approval of nerandomilast for PPF, adding a therapeutic option of particular interest to rheumatologists given its anti-inflammatory mechanism.³˒⁴ Against this backdrop, the group's discussion addressed three broad domains: who to screen and how frequently, how to assess and monitor for progression, and how to structure treatment and multidisciplinary co-management in an era where both the options and expectations for rheumatologists have expanded.

The forum's most substantive discussions centered on 2 interconnected challenges: inconsistency in ILD screening across CTD subtypes, and ambiguity about the rheumatologist's role in ongoing management. Panelists agreed that systemic sclerosis has the most clearly defined and consistently applied screening protocol, and that evidence supports improved mortality and morbidity outcomes from systematic annual surveillance. Less consensus existed — and greater practice variation was admitted — regarding MCTD, Sjögren's disease, and anti-CCP–positive RA patients. Multiple panelists acknowledged not currently screening all high-risk RA patients who lack respiratory symptoms, and at least one explicitly identified anti-CCP positivity as a gap in his own practice he intended to address. In Sjögren's specifically, the forum surfaced an underappreciated distinction: SSA-52 positivity, rather than SSA-60, carries the stronger ILD association, and this subtype is frequently not differentiated in community practice. Discussion of the 2024 ACR/CHEST algorithm included practical emphasis on the importance of ordering ILD-protocol HRCT correctly — thin-slice, unenhanced, full inspiration and expiration — and on basic PFT interpretation as a skill rheumatologists should develop without over-relying on pulmonology for every read. Panelists were candid about their confidence limits in CT pattern classification, and generally felt that distinguishing UIP from NSIP, excluding infectious mimics, and initiating antifibrotics warranted ILD-specialist pulmonology involvement. However, the quality and accessibility of that subspecialty expertise varied substantially across practice settings, with one panelist describing a 2-year wait at a local institution due to infighting over clinic leadership.

Treatment discussion reflected a field in transition. Mycophenolate remains the most widely used first-line immunosuppressant for CTD-ILD and was broadly accepted as falling within the rheumatologist's domain, while antifibrotic initiation was more often deferred to pulmonology — though with notable exceptions in practices with limited access. Tocilizumab was the preferred biologic for SSc-ILD, consistent with the focuSSced trial evidence demonstrating lung function preservation on the secondary FVC endpoint,⁷ though panelists registered clear frustration with guideline hierarchies that continue to sequence non-biologics ahead of FDA-approved agents without adequate supporting evidence. Nerandomilast generated substantive interest: the moderator shared early prescribing experience describing a favorable GI profile relative to nintedanib and noted that secondary endpoint data from FIBRONEER-ILD — including a nominally significant reduction in acute ILD exacerbations and a survival trend with the 18 mg dose — support the rationale for combination use with nintedanib in select patients.⁴ The forum closed with a strong convergence around multidisciplinary care as a clinical standard. The Scripps bimonthly ILD conference was cited as a working model — bringing rheumatology, pulmonology, radiology, and pathology together on a shared virtual platform every eight weeks — with evidence that rheumatologist involvement in traditional MDT changes a meaningful proportion of presumed IPF diagnoses to more specific CTD-related entities and avoids invasive diagnostic procedures. For practices without formal MDT infrastructure, direct phone or text contact with ILD-specialist pulmonologists was endorsed as a practical minimum. Panelists identified ongoing unmet needs including the absence of longitudinal management data for IPAF patients followed by rheumatology, a lack of validated biomarkers for individualized progression risk, and the anticipated but not yet widely available AI-based quantification of CT fibrosis burden.

References

1. Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the screening and monitoring of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). 2024;76(8):1070-1082. doi:10.1002/acr.25347

2. Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). Published online July 8, 2024. doi:10.1002/acr.25348

3. Jascayd (nerandomilast) [prescribing information]. Ridgefield, CT: Boehringer Ingelheim Pharmaceuticals, Inc; 2025.

4. Maher TM, Assassi S, Azuma A, et al. Nerandomilast in patients with progressive pulmonary fibrosis. N Engl J Med. 2025;392:2203-2214. doi: 10.1056/NEJMoa2503643

5. Antoniou KM, et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. Eur Respir J. 2025. doi:10.1183/13993003.02533-2024

6. Antoniou KM, et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. Ann Rheum Dis. 2026;85(1):22-60. doi:10.1016/j.ard.2025.08.021

7. Khanna D, Lin CJF, Furst DE, et al; focuSSced investigators. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020;8(10):963-974. doi:10.1016/S2213-2600(20)30318-0