Interstitial Lung Disease Associated With Autoimmune Rheumatic Diseases: Checklists for Clinical Practice

Abstract

Background: Interstitial lung diseases (ILDs) are often associated with rheumatic diseases. Their early diagnosis and management are not only difficult, but also crucial, because they are associated with major morbidity and mortality and can be the first cause of death in autoimmune rheumatic diseases (ARDs).Objectives: By using methodologies, such as Nominal Group Technique (NGT) and Delphi Survey, the aims of this study were (1) to measure consensus between pulmonologists, radiologists, and rheumatologists experienced in the management of ARD-ILD; (2) to highlight the importance of a multidisciplinary approach; and (3) to provide clinicians with a practical tool aimed at improving the prompt recognition and follow-up of ILD associated with ARDs and of any possible rheumatic conditions underlying ILD.Results: During the NGT round, the Steering Committee defined 57 statements to be used in the Delphi survey. A total of 78 experts participated in the Delphi survey, namely 28 pulmonologists, 33 rheumatologists, and 17 radiologists. During this round, consensus on agreement was reached in 47 statements, while disagreement was not reached in any statements. A secondary questionnaire was drafted by the Steering Committee to obtain clearer indications on ILD-ARD “red-flags” and follow-up. Delphi Panelists took part also in the second-questionnaire survey. Answers from both surveys were used to draft two checklists of “red flags” sign or symptom suggestive of ILD and ARD, respectively, and two checklists on identification and monitoring of rheumatoid arthritis (RA) and systemic sclerosis (SSc) ILD.Limitations: This study is a consensus work, which cannot produce empiric data, and is limited to the Italian scenario.Conclusions: This work showed a high level of agreement, but also shows some divergent opinions between different experts. This underlines the importance of a multidisciplinary approach. Eventually, we believe the drafted checklists can help clinicians in the diagnosis and follow-up of ILD-ARD.

Diagnosing interstitial lung disease by multidisciplinary discussion: A review

“…Although up to 20% of ILDs are associated with underlying CTDs such as systemic sclerosis, rheumatoid arthritis and idiopathic inflammatory myopathies, rheumatologists and immunologists are not routinely involved in ILD MDMs ( 37 , 38 ). In fact, an evaluation of global MDM practices showed only 37.1% of survey centers routinely involved a rheumatologist in ILD MDM discussions ( 10 ), and rheumatology opinion was otherwise only sought if the referring clinician suspected a systemic autoimmune disease based on their own assessment.…”

“…Another recent Italian study involving a Delphi survey and additional questionnaires distributed to pulmonologists, rheumatologists and radiologists demonstrated high levels of agreement regarding the importance of a collaborative approach to diagnosis of CTD-ILD ( 38 ). Results were used to generate checklists of important “red flag” signs and symptoms suggestive of ILD in CTD patients, as well important “red flag” signs and symptoms suggestive of CTD in undifferentiated ILD patients.…”

“…Importantly, the Delphi survey also addressed potential methods to improve recognition of CTD-ILD where rheumatologists are not present at the ILD MDM. These included creation of networks and collaborative research efforts between different centers across the country; and development of opportunities for clinicians to participate in multidisciplinary clinics or locoregional ILD MDMs discussing archetypal cases; with the aim of improving evidence-based approach to diagnostic formulation in challenging ILDs ( 38 ).…”

La corretta gestione del paziente con amiloidosi ereditaria da transtiretina a fenotipo misto: le raccomandazioni di un gruppo di esperti Italiani

“…Poiché l'incontro è avvenuto in modalità virtuale a causa della situazione pandemica, la metodologia è stata adattata (Figura 1). 33,34 Nominal Group Technique (NGT) (iv) ranking: ogni partecipante assegna in modo autonomo a ogni statement un punteggio tra 1 e 5 (1=non rilevante, 5=estremamente rilevante). I punteggi ottenuti per ciascuno statement vengono sommati, creando una graduatoria di idee giudicate maggiormente rilevanti sulla base di un processo di clustering.…”

Long‐term proactive management of psoriasis with calcipotriol and betamethasone dipropionate foam: an Italian consensus through a combined nominal group technique and Delphi approach

“…In particular, we used a combined approach of two different consensus methods, the nominal group technique (NGT) and the Delphi method. The combination of NGT and Delphi is increasingly used in clinical qualitative methdology, to provide a robust collection of expert opinions 11–13 …”