Multidisciplinary Approach to Interstitial Lung Disease Associated with Systemic Rheumatic Diseases

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Background

Interstitial Lung Disease (ILD) is a common manifestation of Connective Tissue Disease and ANCA associated vasculitides, with a prevalence ranging from 3% in Systemic Lupus Erythematosus to 80% in the Scleroderma Spectrum Disorders. Moreover, ILD associated with a definite Autoimmune Rheumatic Disease (ARD) or an undifferentiated form (Interstitial Pneumonia with Autoimmune Features, IPAF) represents about 25% of total ILD patients in respiratory settings. In the last few years, a growing interest in this topic led to great advances, with the discover of some clinical and serological predictive factors and the recent introduction of antifibrotic drugs for management of disease. However, ILD-ARDs remain associated with progression and worsening in about 30% of patients, representing one of the leading causes of death.

The diagnosis of ARD underlying ILD could be difficult: actually, ILD can be the first, the prevalent, or even the sole clinical manifestation of ARD. Moreover, some rare clinical signs, although highly specific, could not be correctly recognized by non-trained physicians. Finally, despite Nonspecific Interstitial Pneumonia being the most common ILD radiologic and histological pattern, all of ILD patterns can be associated with ARD. The same management often requires a tight collaboration between pulmonologists, rheumatologists and radiologists. The Multidisciplinary Team (MDT) is currently considered the gold standard for the ILD management. This is particularly true for ILD associated with autoimmune rheumatic conditions, considering the common systemic involvement.

The scope of this research topic is to highlight the role of the collaboration of pulmonologists, rheumatologists and radiologists in the research and clinical management of ILD-ARDs patients.

We welcome submission of Original Research, review, instructive case reports and clinical trials regarding ILD associated with specific ARDs diagnosis (Rheumatoid Arthritis, Scleroderma Spectrum Disorders, Idiopathic Inflammatory Myopathies, Sjogren’s syndrome, ANCA-associated vasculitides) or IPAF.

The paper can be focused on, but not limited to, the following topics:
- Diagnosis
- Immunopathogenic mechanisms
- Development of new markers, prognostic factors, predictive algorithms, application of new technologies
- Clinical management of comorbidity, with a special interest for management of COVID-19 infection and relative vaccination
- Imaging
- Treatment

Keywords: Interstitial Lung Disease, Polymyositis, Dermatomyositis, Antisynthetase Syndrome, Vasculitis, Systemic Sclerosis, Rheumatoid Arthritis, Sjogren's Syndrome

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