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

IF 1.8 Q3 RESPIRATORY SYSTEM European Clinical Respiratory Journal Pub Date : 2021-06-10 DOI:10.1080/20018525.2021.1933878
Janne Møller, Alan Altraja, Tone Sjåheim, Finn Rasmussen, Line Bille Madsen, Elisabeth Bendstrup
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引用次数: 1

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.

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国际多学科小组讨论特发性非特异性间质性肺炎的诊断和结缔组织病的发展。
背景:特发性非特异性间质性肺炎(iNSIP)是一种罕见的间质性肺病,根据多学科团队讨论(MDD)的定义进行诊断。iNSIP中已提出与自身免疫背景有关。目的:测试进行多国MDD检查iNSIP病例诊断的可行性,并评估随访期间结缔组织疾病(CTD)的出现。方法:来自三个专家中心(丹麦、爱沙尼亚和挪威)的研究人员在国际MDD。这些病例之前是在2004年至2014年期间在国家层面诊断的。根据临床、放射学和病理学数据,对iNSIP的诊断进行了重新评估,并达成了一致诊断。排除与iNSIP不兼容的病例。相关数据包括CTD的任何发展。结果:总共讨论了31例病例,23名患者被诊断为iNSIP。平均随访时间57个月。在随访期间,根据风湿病标准,没有一名患者出现CTD。4名患者(17.4%)符合具有自身免疫特征的间质性肺炎的标准。结论:我们发现国际MDD在iNSIP的回顾性诊断评估中是一种可行且有价值的工具。我们的国际MDD团队在统计上显著数量的患者中改变了诊断。随访期间,没有患者出现CTD。
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CiteScore
3.80
自引率
0.00%
发文量
15
审稿时长
16 weeks
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