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Abstract
Interstitial lung disease (ILD) encompasses a wide range of parenchymal lung pathologies with different clinical, histological, radiological, and serological features. Follow-up, treatment, and prognosis are strongly influenced by the underlying pathogenesis. Considering that an ILD may complicate the course of any connective tissue disease (CTD) and that CTD's signs are not always easily identifiable, it could be useful to screen every ILD patient for a possible CTD. The recent definition of interstitial pneumonia with autoimmune features is a further confirmation of the close relationship between CTD and ILD. In this context, the multidisciplinary approach is assuming a growing and accepted role in the correct diagnosis and follow-up, to as early as possible define the best therapeutic strategy. However, despite clinical advantages, until now, the pathways of the multidisciplinary approach in ILD patients are largely heterogeneous across different centers and the best strategy to apply is still to be established and validated. Aims of this article are to describe the organization of our multidisciplinary group for ILD, which is mainly focused on the early identification and management of CTD in patients with ILD and to show our results in a 1 year period of observation. We found that 15% of patients referred for ILD had an underlying CTD, 33% had interstitial pneumonia with autoimmune feature, and 52% had ILD without detectable CTD. Furthermore, we demonstrated that the adoption of a standardized strategy consisting of a screening questionnaire, specific laboratory tests, and nailfold videocapillaroscopy in all incident ILD proved useful in making the right diagnosis.
Highlights
Interstitial lung disease (ILD) includes a heterogeneous group of parenchymal lung pathologies with different clinical, histological, radiological, and serological features [1]
Eight patients entered the GIILD multidisciplinary discussion, but a definite diagnosis was not yet established at the end of the period considered for the present study, so they were excluded from analysis (STROBE diagram, Figure 3)
The CTD patients were classified as rheumatoid arthritis in four (3%), systemic sclerosis in three (3%), undifferentiated connective tissue disease in three (2%), and antisynthetase syndrome in two (2%) cases, whereas six patients (5%) were classified one each as polymyositis, dermatomyositis, Sjogren syndrome, scleromyositis, amyopathic dermatomyositis, and granulomatosis with polyangiitis
Summary
Interstitial lung disease (ILD) includes a heterogeneous group of parenchymal lung pathologies with different clinical, histological, radiological, and serological features [1]. The inclusion of the rheumatology assessment is an added value for patients [9, 17, 18], and the possibility to start the multidisciplinary pathway from a screening tool seems to be effective in terms of health-care resources optimization. Despite these observations, the best strategy to apply in the multidisciplinary evaluation still has to be defined and validated [19]. We want to describe the organization, and share the first results, of our Multidisciplinary Group for Interstitial Lung Disease (GI-ILD), focusing on the early identification of CTDs in ILD patients referring to our clinics
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