Pulmonologists and Rheumatologists Struggle to Diagnose and Manage CTD-ILD, According to New Research from Spherix Global Insights

By: Spherix Global Insights via GlobeNewswire

June 17, 2025 at 15:01 PM EDT

EXTON, PA, June 17, 2025 (GLOBE NEWSWIRE) -- According to Spherix Global Insights’ newly published Special Topix™: Interstitial Lung Disease in Rheumatology (US) report, both rheumatologists and pulmonologists report significant challenges in diagnosing and managing connective tissue disease-associated interstitial lung disease (CTD-ILD), with systemic sclerosis (SSc) and myositis (IIM) cited as the most difficult subtypes to treat. The research, based on responses from 138 US specialists (n=68 rheumatologists and 70 pulmonologists), highlights persistent unmet needs, diagnostic ambiguity, and a call for more robust and collaborative management strategies.

The findings reflect that undiagnosed and misdiagnosed patients make up a substantial portion of the CTD-ILD population, with nearly one-third of rheumatologists and over half of pulmonologists naming differential diagnosis as a key challenge. Specialists underscore a lack of specific biomarkers, logistical biopsy issues, and overlapping symptoms as primary obstacles.

“The most definitive test (biopsy) is invasive. It is difficult to find the clinicians who are willing to do it in a timely manner, and to convince the patient it needs to be done.” – Rheumatologist

“Ensuring the correct diagnosis to avoid misdiagnosing the type of ILD or source of ILD. I usually employ the assistance of a rheumatologist.” – Pulmonologist

“Often it would be difficult to get patients biopsied for logistical reasons, and often the pathology report would not be precise enough.” – Pulmonologist

Even once diagnosed, CTD-ILD patients face a treatment landscape with few options to slow or halt disease progression. When asked about the most difficult aspects of management, specialists consistently pointed to a lack of effective and targeted therapies, with SSc-ILD and myositis-ILD receiving the highest unmet need scores.

For SSc-ILD specifically, not only is it associated with the most aggressive progression and worst long-term outcomes, but it also presents a uniquely complex therapeutic challenge. The clinical burden is especially pronounced in patients with diffuse cutaneous systemic sclerosis (dcSSc), where the use of corticosteroids is often limited due to the heightened risk of scleroderma renal crisis. This leaves physicians with a narrow band of anti-fibrotic and immunosuppressive options, many of which are perceived as only modestly effective in controlling ILD progression, let alone reversing it.

Even therapies with regulatory approval for SSc-ILD, such as Boehringer Ingelheim’s OFEV (nintedanib), garner only measured enthusiasm from treating physicians. While rheumatologists acknowledge nintedanib’s potential to slow functional decline, they note it does little to address the underlying autoimmune pathology. Similarly, mycophenolate mofetil and rituximab remain staples of clinical practice, yet their real-world performance is viewed as inconsistent and difficult to monitor. Across the board, satisfaction ratings for currently utilized agents fall short of expectations, reinforcing a treatment landscape still dominated by trial-and-error approaches.

This disconnect between disease complexity and treatment efficacy is fueling demand for dual-acting agents, therapies that can address both systemic autoimmunity and pulmonary fibrosis. Nowhere is this demand more visible than in systemic lupus erythematosus-associated ILD (SLE-ILD), where rheumatologists express growing interest in GSK’s Benlysta (belimumab). Though not currently approved for ILD, belimumab’s immunologic mechanism and expanded label for lupus nephritis have raised hopes that similar benefit may extend to pulmonary manifestations. Across interviews and survey feedback, physicians articulate a desire for treatments that reduce systemic activity while simultaneously preventing fibrotic progression—without the need for an antifibrotic add-on with limited synergy.

Looking ahead, specialists are cautiously optimistic about the pipeline. While interest in agents with antifibrotic and immunomodulatory properties is rising, physicians emphasize that what’s most needed is not just another drug, but a paradigm shift—a treatment model that reflects the multifaceted nature of CTD-ILD. As one physician summarized, the goal is to "treat the patient, not just the lungs."

In addition to tracking physician sentiment and practice patterns, the study includes target product profile evaluations for several pipeline therapies: Benlysta (GSK), BMS-986278 (Bristol Myers Squibb), Nerandomilast (Boehringer Ingelheim), and Treprostinil (United Therapeutics). These profiles offer manufacturers timely insights into clinical expectations, adoption potential, and differentiation opportunities in an increasingly complex ILD treatment landscape.

Special Topix™ is an independent service that includes access to a report or series of reports based on current events or topics of interest in specialty markets covered by Spherix.

About Spherix Global Insights 

Spherix is a leading independent market intelligence and advisory firm that delivers commercial value to the global life sciences industry, across the brand lifecycle. 

The seasoned team of Spherix experts provides an unbiased and holistic view of the landscape within rapidly evolving specialty markets, including dermatology, gastroenterology, rheumatology, nephrology, neurology, ophthalmology, and hematology. Spherix clients stay ahead of the curve with the perspective of the extensive Spherix Physician Community. 

As a trusted advisor and industry thought leader, Spherix’s unparalleled market insights and advisory services empower clients to make better decisions and unlock opportunities for growth. 

To learn more about Spherix Global Insights, visit spherixglobalinsights.com or connect through LinkedIn.  

For more details on Spherix’s primary market research reports and interactive dashboard offerings, visit or register here: https://clientportal.spherixglobalinsights.com  

NOTICE: All company, brand or product names in this press release are trademarks of their respective holders. The findings and opinions expressed within are based on Spherix Global Insight’s analysis and do not imply a relationship with or endorsement of the companies or brands mentioned in this press release.

Andy Stankus, Rheumatology Franchise Head
Spherix Global Insights
4848794284
andy.stankus@spherixglobalinsights.com

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Pulmonologists and Rheumatologists Struggle to Diagnose and Manage CTD-ILD, According to New Research from Spherix Global Insights

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