Expert Opinion Guidance on the Detection of Early Connective Tissue Diseases in Interstitial Lung Disease

Abstract

There is a significant variation in symptoms and clinical presentation of connective tissue disorders (CTD) associated with interstitial lung disease (ILD) (CTD-ILD). This presents difficulties in the diagnosis and treatment of CTD-ILD. Early detection and treatment of CTD-ILD using a multidisciplinary approach have been shown to enhance patient outcomes. This exercise aims to explore clinical components to develop a screening tool for pulmonologists for early detection of CTD in ILD and to provide a framework for a multidisciplinary approach in managing CTD-ILD. This in turn will lead to early treatment of CTD-ILD in collaboration with rheumatologists. A panel of 12 leading rheumatologists from the Middle East and North Africa (MENA) region met virtually to select the most relevant clinical findings to aid in identifying CTD-ILD. Twelve panellists opted to investigate seven of the most common inflammatory autoimmune disorders. The panel discussed how to improve the early detection of CTD-ILD. Clinical characteristics were categorized, and a nine-item questionnaire was created. A biphasic algorithm was developed to guide early referral to a rheumatologist based on the presence of one of nine clinical features of CTD (Phase 1) or the presence of CTD-specific antibodies (Phase 2). A brief questionnaire has been developed to serve as a simple and practical screening tool for CTD-ILD detection. Additional research is needed to validate and evaluate the tool in longitudinal cohorts.

Keywords:

• connective tissue disease-associated interstitial lung disease
• CTD-ILD
• rheumatologists
• pulmonologists
• screening
• multidisciplinary team

Statement of Ethics and Consent

This consensus paper was developed in compliance with the Declaration of Helsinki.²⁷ For research that uses publicly available data where identities of patients cannot readily be ascertained, US FDA regulation 45 CFR part 46.104 grants exemption from 1) IRB review and 2) taking patient consent.²⁸ Regulations of UK, Australia and Netherlands too exempt research involving pre-existing nonidentifiable data in public domain from IRB review.²⁹

To develop this guideline, research methodology was limited to review of existing literature available in public domain and the panelists’ own expertise and experience. No patient information was accessed or exposed. No multiple datasets were reviewed or cross-referenced so there is no risk of inadvertent patient identification. As no patient was contacted in any capacity, no informed consent was sought. As per the regulations quoted above, IRB review of development of this consensus and guideline was deemed unnecessary. No IRB review was conducted. The Figures 2A, 2C and 3B and 4A presented in the manuscript was taken from Rheumatology Image Library, American College of Rheumatology (ACR) image bank with prior permission.

Acknowledgments

Boehringer Ingelheim (BI) was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations. Writing, editorial support, and/or formatting assistance were provided by Cognizant technology solutions India Private Ltd, which was contracted and funded by Boehringer Ingelheim (BI).

Disclosure

The authors report no conflicts of interest in this work.