The Evolving Role of the Rheumatologist in ILD, With Viraj Shah, MD

Interstitial lung disease is among the most consequential complications of systemic autoimmune rheumatic diseases, occurring across a spectrum of conditions — systemic sclerosis, anti-synthetase syndrome, MDA5-associated dermatomyositis, rheumatoid arthritis, Sjögren's syndrome, and mixed connective tissue disease — with a burden of morbidity and mortality that frequently surpasses that of the primary articular or cutaneous disease itself.¹ ILD accounts for the third most common cause of death in dermatomyositis and polymyositis, ranks among the top four causes of mortality in both systemic sclerosis and Sjögren's syndrome, and contributes to approximately 8% of deaths in rheumatoid arthritis.¹

The 2023 ACR/CHEST collaborative guidelines — the first joint recommendations endorsed by both specialties for screening and monitoring ILD in patients with systemic autoimmune rheumatic diseases — established PFTs and HRCT as the preferred screening modalities, with monitoring frequencies of every 3 to 6 months for myositis and systemic sclerosis during the first year and annually for RA, Sjögren's syndrome, and MCTD.² Simultaneously, the therapeutic landscape has expanded: nerandomilast (Jascayd; Boehringer Ingelheim), a first-in-class preferential PDE4B inhibitor, received FDA approval for idiopathic pulmonary fibrosis in October 2025 and for progressive pulmonary fibrosis in December 2025, based on the FIBRONEER-IPF and FIBRONEER-ILD trials — the first novel antifibrotic mechanism to reach the market in more than a decade.³ Despite these advances, community practice implementation of guideline-concordant screening and appropriate antifibrotic use remains highly variable, and the scarcity of ILD-specialized pulmonologists in many regions has drawn rheumatologists into roles that exceed traditional specialty boundaries.

Against this backdrop, RheumatologyLive convened a panel of rheumatologists and advanced practice providers from the Pensacola, Florida region and surrounding Gulf Coast communities for an in-depth roundtable discussion on ILD screening, monitoring, and treatment in the rheumatic disease context. The forum was moderated by Viraj Shah, MD, a rheumatologist at Baptist Health Care, and included panelists from multi-physician rheumatology groups and community practices, producing a discussion that was as much about the infrastructure of rheumatology ILD practice as its clinical content.

Mycophenolate, Tocilizumab, and Rituximab: How to Select CTD-ILD Therapy

The panel reached near-universal consensus that Connective Tissue Disease (CTD)-ILD falls within the rheumatologist's scope of treatment, while non-CTD ILD and antifibrotic initiation — when a pulmonologist is accessible within a reasonable timeframe — are preferentially deferred to pulmonology. In practice, however, with pulmonologist wait times extending to months in this region, the panel described rheumatologists functioning as the de facto primary ILD managers for many patients. Mycophenolate mofetil was identified as the universal first-line agent, typically started at 500 mg twice daily for 2 weeks before escalating to 1 g twice daily, with a target of 3 g per day in appropriately tolerating patients — an approach consistent with ACR/CHEST guidelines.²

Second-line selections were disease-specific: tocilizumab was the preferred escalation for SSc-ILD, supported by the phase 2/3 faSScinate and phase 3 focuSSced trials, which demonstrated preservation of FVC at week 48 despite not meeting the primary skin endpoint — the basis for its FDA approval in SSc-ILD.⁴ Rituximab was favored for RA-ILD and myositis-ILD, particularly when combined articular and pulmonary disease made a B-cell–depleting strategy advantageous. For Sjögren's syndrome with lymphocytic interstitial pneumonia, rituximab was also endorsed as the best-supported option, with several panelists noting that emerging Sjögren's-specific agents in late-stage development may soon provide additional ILD-relevant options.

Cyclophosphamide was described as rarely used and reserved for refractory cases, though 2 panelists had initiated it recently in severely ill patients. A tocilizumab biosimilar supply shortage was raised as a live access problem forcing mid-treatment switches back to the reference drug. As one panelist summarized the broader treatment imperative: "I think the key is if you can't get them in with someone who has expertise in the antifibrotics, we got to get them on it now. We've got to put them on it because that is months to years of life versus not."

Nintedanib, Nerandomilast, and the Infrastructure Gap in Community ILD Care

Nintedanib's decade-long presence in the antifibrotic market has generated a clinical reputation defined largely by its gastrointestinal tolerability profile. Severe diarrhea was described as the dominant reason for discontinuation in this panel's experience — 1 panelist cited a single severe patient event as effectively deterring further prescribing, while another noted the drug ships with antidiarrheal medication as part of its packaging. The panel's characterization of discontinuation rates was consistently high, and at least 1 panelist reported never observing clear disease-slowing benefit before intolerance forced discontinuation.

Nerandomilast drew considerably more favorable early impressions: patients on the drug were described as "doing well," no infection concerns were noted, and its PDE4B mechanism — familiar from apremilast (Otezla) in psoriatic disease — was invoked as reassuring context for the tolerance profile. In the FIBRONEER-ILD trial, nerandomilast demonstrated a significantly smaller FVC decline versus placebo in PPF (–86 mL vs. –152 mL with placebo at 18 mg; P <.001), with permanent discontinuation rates similar to placebo across all dose cohorts — a contrast specifically noted by panelists as advantageous over nintedanib.³ One panelist referenced a publication from the preceding day showing FVC decline reduction of approximately 43 mL versus 196 mL with placebo in IPF — consistent with published FIBRONEER-IPF data — and described visible interest in the room at that data point.

Access was the principal barrier, with Boehringer Ingelheim's CareConnect4Me bridge program providing 2 months of supply with one refill as the standard workaround while prior authorization is navigated. The panel also endorsed AI-assisted PFT interpretation — specifically via tools such as DiveDeep — as an increasingly practical workflow for rheumatologists who lack institutional infrastructure for real-time spirometry interpretation, a gap that was described with candor: one panelist noted sending all PFTs directly to pulmonology rather than attempting independent interpretation, while another acknowledged defaulting to the radiologist's impression on HRCT without reviewing the images.

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 treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). 2024;76(8):1051–1069. doi:10.1002/acr.25348

2. 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

3. Maher TM, Corte TJ, Kreuter M, et al; FIBRONEER-ILD Trial Investigators. Nerandomilast in patients with progressive pulmonary fibrosis. N Engl J Med. 2025. doi:10.1056/NEJMoa2415780 [Note: per Boehringer Ingelheim press materials and FDA approval basis; confirm final DOI upon indexing]

4. 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