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. 2021 Jun 10;8(1):1933878.
doi: 10.1080/20018525.2021.1933878.

International multidisciplinary team discussions on the diagnosis of idiopathic non-specific interstitial pneumonia and the development of connective tissue disease

Janne Møller  1 , Alan Altraja  2   3 , Tone Sjåheim  4 , Finn Rasmussen  5 , Line Bille Madsen  5 , Elisabeth Bendstrup  6
Affiliations

International multidisciplinary team discussions on the diagnosis of idiopathic non-specific interstitial pneumonia and the development of connective tissue disease

Janne Møller et al. Eur Clin Respir J. .

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.

Keywords: Non-specific interstitial pneumonia; connective tissue disease; idiopathic pneumonia with autoimmune features; multidisciplinary team discussion.

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Conflict of interest statement

Janne Møller has received lecture fees from Roche and participated in advisory boards of Roche. Alan Altraja has received lecture fees and sponsorships from Boehringer Ingelheim and Roche and research support from Boehringer Ingelheim, and has participated in advisory boards of Boehringer Ingelheim and Roche. Elisabeth Bendstrup reports fees and grants from Boehringer Ingelheim, Hoffman la Roche, Galapagos, and Bristol-Myers-Squibb.

Figures

Figure 1.
Figure 1.
High-resolution computed tomography (HRCT) finding of one of the patients with non-specific interstitial pneumonia (NSIP)
Figure 2.
Figure 2.
Surgical lung biopsy finding of one of the patients: Cellular and fibrotic non-specific interstitial pneumonia (NSIP) with interstitial inflammation and uniform collagen deposition. HE x 40
See this image and copyright information in PMC

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