International multidisciplinary team discussions on the diagnosis of idiopathic non-specific interstitial pneumonia and the development of connective tissue disease. Issue 1 (1st January 2021)

• Record Type: Journal Article
• Authors: Møller, Janne; Altraja, Alan; Sjåheim, Tone; Rasmussen, Finn; Madsen, Line Bille; Bendstrup, Elisabeth
• Is Part Of: European clinical respiratory journal. Volume 8:Issue 1(2021)
• Publication Date: 2021-01-01
• Subjects: Non-specific interstitial pneumonia -- multidisciplinary team discussion -- connective tissue disease -- idiopathic pneumonia with autoimmune features; Lungs -- Diseases -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Lung Diseases; Lungs -- Diseases; Respiratory organs -- Diseases; Europe; Periodicals; Electronic journals; Periodicals; 616.24
• DOI: 10.1080/20018525.2021.1933878
• Languages: English
• ISSNs: 2001-8525

Abstract

ABSTRACT: Background: Idiopathic Non-Specific Interstitial Pneumonia (iNSIP) is a rare interstitial lung disease, diagnosed, by definition, on the basis of a multidisciplinary team discussion (MDD). Association with an autoimmune background has been suggested in iNSIP. Aims: To test the feasibility of conducting a multinational MDD to review the diagnosis in iNSIP cases and to estimate the emergence of connective tissue disease (CTD) during follow-up. Methods: Investigators from three expert centers (Denmark, Estonia and Norway) met and discussed cases of biopsy-proven iNSIP at an international MDD. The cases were previously diagnosed at a national level between 2004 and 2014. Based on clinical, radiographic and pathological data, the diagnosis of iNSIP was re-evaluated and a consensus diagnosis was made. Cases incompatible with iNSIP were excluded. Relevant data were registered comprising any development of CTD. Results: In total, 31 cases were discussed and 23 patients were included with a diagnosis of iNSIP. The mean follow-up time was 57 months. None of the patients developed CTD according to the rheumatologic criteria during the follow up period. Four patients (17.4%) met the criteria for interstitial pneumonia with autoimmune features. Conclusion: We found that an international MDD was a feasible and valuable tool in the retrospective diagnostic evaluation of iNSIP. Diagnosis was changed in a statistically significant number of patients by our international MDD team. None of the patients developed CTD during follow-up.