AB1135 THE MANAGEMENT OF INTERSTITIAL LUNG DISEASES: THE IMPORTANCE OF THE RHEUMATOLOGIC EXPERTISE IN MULTIDISCIPLINARY MEETINGS
Authors: Enrico De Lorenzis, Gerlando Natalello, Lucrezia Verardi, Silvia Laura Bosello, Elisa Gremese
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Multidisciplinary Team (MDT) meetings are the current "gold standard" in interstitial lung disease (ILD) diagnosis. Requisite participants are respiratory physicians, radiologists and pathologists. A rheumatologist physician is not routinary involved in MDT even if up to 20% of ILD are related to connective tissue disease, rheumatoid arthritis or systemic vasculitis. <h3>Objectives</h3> The aim of this study is to evaluate the prevalence and predictors of systemic rheumatological diseases in a cohort of patients with ILD, evaluated by a rheumatology specialist on the advice of MDT in a university hospital. <h3>Methods</h3> Thirty-two patients with ILD, evaluated at dedicated MDT were referred to a rheumatologist in 2018, usually for autoantibodies positivity or clinical history suspected for a rheumatological disorder. Rheumatologic evaluation included physical examination, routinary blood and urine tests, serum levels of C3 and C4, ANA, Rheumatoid Factor (RF), ANCAs, anti-Sm, anti-RNP, anti-Ro/SSA, anti-La/SSB, anti-Sm, anti-Jo1, anti-dsDNA and anti-CCP antibodies. Family history of autoimmune diseases, presence of rheumatologic red flags (Raynaud's phenomenon, photosensitivity, inflammatory skin manifestations, sicca syndrome, recurrent fever, inflammatory arthralgias, paresthesias, oral or genital aphthosis, thrombosis and recurrent miscarriages), respiratory symptoms and pulmonary function test were also evaluated. When indicated, capillaroscopy, joint imaging and salivary gland biopsy were performed. <h3>Results</h3> Twenty-one patients (65.6%) were female. At the time of rheumatological evaluation, the patients had a mean age of 64.4±12.6 years with a mean period of 21.1±39.6 months since the first identification of ILD on a CT scan. Twenty-five (78.1%) and 18 (56.3%) patients respectively complained exertional dyspnea and cough, 6 patients (18.8%) were on oxygen therapy and 13 (40.6%) had a FVC&lt;80%. The pattern on CT scan suggested a UIP, NSIP and OP respectively in 18 (56.3%), 6 (18.8%) and 2 (6.3%) patients. Ten patients (31.3%) were diagnosed with a defined rheumatological condition (4 with Sjogren Syndrome, 3 with scleroderma, 1 with Rheumatoid Arthritis, 1 with Mixed Connective Tissue Disease and 1 with Granulomatosis with Polyangiitis); 7 (21.9%) were diagnosed with Interstitial Pneumonia with autoimmune feature (IPAF). A rheumatological diagnosis was not statistically associated with any specific rheumatologic red flag, routine laboratory abnormalities or family history of systemic autoimmune disease. Three out of 13 capillaroscopies and 3 out of 9 salivary glands biopsies performed had diagnostic findings. The only immunologic abnormalities associated to a rheumatologic diagnosis were RF positivity (20.0%vs54.5%, p=0.024) and p-ANCA positivity (10.0%vs40.9%, p=0.042), both with low specificity (respectively 15.0% and 5.0%) and sensitivity (respectively 55.6% and 42.9.0%). After diagnosis, 11 patients (34.4%) started immunosuppressive therapy and 7 (21.9%) started antifibrotic therapy. <h3>Conclusion</h3> Single clinical or laboratory abnormalities are not strongly associated to a rheumatological diagnosis in patients with ILD, suggesting that only a comprehensive rheumatological evaluation allows correct classification of the disease associated with ILD and is mandatory to make or exclude a diagnosis. In this study, we evaluated only preselected patients by ILD-MDT, but the results indirectly suggest that direct participation of rheumatologist to MDT is advisable to increase accuracy and reduce delay in diagnosis and treatment. <h3>Disclosure of Interests:</h3> Enrico De Lorenzis: None declared, Gerlando Natalello: None declared, Lucrezia Verardi: None declared, Silvia Laura Bosello: None declared, Elisa Gremese Consultant for: AbbVie, BMS, Celgene, Janssen, Lilly, MSD, Novartis, Sanofi, UCB, Roche, and Pfizer, Speakers bureau: BMS, Speakers bureau: Roche, Speakers bureau: AbbVie, BMS, Celgene, Janssen, Lilly, MSD, Novartis, Sanofi, UCB, Roche, and Pfizer