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

Silvia Laura Bosello; Lorenzo Beretta; Nicoletta Del Papa; Sergio Harari; Stefano Palmucci; Alberto Pesci; Gilda Rechichi; Francesco Varone; Marco Sebastiani

doi:10.3389/fmed.2021.732761

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

Front Med (Lausanne). 2021 Oct 15:8:732761. doi: 10.3389/fmed.2021.732761. eCollection 2021.

Authors

Silvia Laura Bosello¹, Lorenzo Beretta², Nicoletta Del Papa³, Sergio Harari^{4

5}, Stefano Palmucci⁶, Alberto Pesci⁷, Gilda Rechichi⁸, Francesco Varone⁹, Marco Sebastiani¹⁰

Affiliations

¹ Rheumatology Unit, A. Gemelli University Hospital Foundation - Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Catholic University of the Sacred Heart, Rome, Italy.
² Scleroderma Unit, Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS) Ca' Granda Foundation Ospedale Maggiore Policlinico di Milan, Milan, Italy.
³ Rheumatology Department, Scleroderma Clinic, ASST Pini-CTO, Milan, Italy.
⁴ Department of Medical Sciences, San Giuseppe Hospital MultiMedica Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Milan, Italy.
⁵ Department of Clinical Sciences and Community Health, Università Degli Studi di Milano, Milan, Italy.
⁶ Radiology Unit I, Department of Medical Surgical Sciences and Advanced Technologies GF Ingrassia, University Hospital Policlinico "G. Rodolico-San Marco", University of Catania, Catania, Italy.
⁷ Pneumological Clinic of the University of Milan-Bicocca, ASST-Monza, Monza, Italy.
⁸ Radiology Unit, University Hospital San Gerardo, ASST-Monza, Monza, Italy.
⁹ Pneumology Unit, A. Gemelli University Hospital Foundation Istituto di Ricerca e Cura a Carattere Scientifico (IRCCS), Rome, Italy.
¹⁰ Rheumatology Unit, University of Modena and Reggio Emilia, Italy.

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.

Keywords: ARD-ILD; Delphi panel survey; autoimmune rheumatic diseases; consensus; interstitial lung disease; multidisciplinary team; nominal group technique; red flags and referral indications.