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Background: The term “Interstitial Pneumonia with Autoimmune Features (IPAF)” is used to describe patients with Interstitial Lung Disease in combination to clinical, serological and/or pulmonary features that are suggestive, but insufficient to satisfy classification criteria of a specific Connective Tissue Disease (CTD). Some retrospective studies available in literature described patient cohorts with IPAF heterogenous in terms of clinical, serological, and radiographic manifestations Objectives: To prospectively recruit a cohort of consecutive ILD patients classified as IPAF or as affected by Idiopathic Pulmonary Fibrosis (IPF); to describe their clinical, serological, and radiological features by a multi-disciplinar team composed by Pulmonologists, Radiologists, and Rheumatologists, comparing IPAF and IPF patients. Methods: In the lasts 2 years, they were enrolled 45 patients with IPAF and 143 with IPF among a total of 506 patients with Interstitial Lung Disease (ILD). All patients were evaluated clinically by both rheumatologists and pulmonologists, also by means of chest high resolution computed tomography (hrCT), pulmonary function tests (PFT), and nailfold videocapillaroscopy. Results: In IPAF cohort the most common characteristics from the clinical, serological and morphological domain were Raynaud’s phenomenon (RP) (31.1%) antinuclear antibodies positivity with titre ≥1/320 or any titre for centromeric or nucleolar pattern (17.7%) and nonspecific interstitial pneumonia (68.8%) respectively. The majority of patient (88.9%) had the minimum of 2 criteria at the recruitment. Female gender was more common in IPAF compared to IPF (62.2% vs 23%, p Conclusion: IPAF criteria recruit a rare cohort of patients with a disease probably less severe than IPF, even though clinically relevant. Further prospective studies may better define the long-term prognosis of ILD in IPAF. The follow-up of these patients by a multi-disciplinary team may be useful in order to early recognize and treat the new cases of CTDs. Some IPAF criteria probably are useless due to their high specificity for definite CTD and the classification need a revision in the light of the prospective experiences. Nevertheless, IPAF classification can recruit incomplete form or early onset of CTD that can allow a timely treatment in patients without a structured damage. References: [1] Sambataro G, Sambataro D, Torrisi SE, Vancheri A, Pavone M, Rosso R, Schisano M, Crimi C, Pignataro F, Fischer A, Del Papa N, Vancheri C. State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances. Eur Respir Rev2018:27(148) Disclosure of Interests: None declared

Background Interstitial lung disease (ILD) is the most frequent pulmonary impairment in Sjogren’s syndrome (SS). The diagnosis is challenging, as there are not specific tests (i.e. autoantibodies or pulmonary function tests) or symptoms. Chest CT is the gold standard. Semiquantitative visual scores (SQCT) estimate ILD extent, though burdened by relevant intra-, inter-rater variability. Quantitative chest CT (QCT) is a promising method to assess ILD severity Objectives: to verify if QCT assessment could identify SS subjects affected by ILD and to identify those SS patients with an extensive lung disease. Methods: in this multi-center, cross-sectional, and retrospective study, subjects affected by SS and with a chest CT were enrolled. A blind and centralized SQCT assessment was carried out in order to calculate both Goh and Taouli scores. An operator-independent analysis of all CTs with an open-source software (i.e. Horos), provided the QCT indexes. Patients were clustered in cohorts according to ILD extent. Differences in QCT indexes distribution were analyzed using non-parametric tests. Results 102 consecutive SS patients were enrolled. ILD prevalence was 36% (36/102). There was a difference in QCT indexes’ distribution in SS-ILD versus SS without ILD (p 20% (according to Goh score) had QCT indexes statistically different from those with a limited ILD extension (p 0.7; the best QTC index was: tSkew AUC 0.87 (95CI 0.78-0.95), p Conclusion QCT indexes are a serious alternative to visual scorings in ILD related to autoimmune diseases. This innovative tool will open up a potential research area in SS as it appears able to select ILD patients with an extensive lung impairment and likewise a worse prognosis. We are confident that in the next future, QCT will have a pivotal role in the diagnosis, monitoring and treatment pathway of ILD associated to SS. References [1] Silva M, Milanese G, Seletti V, Ariani A, Sverzellati N. Pulmonary quantitative CT imaging in focal and diffuse disease: current research and clinical applications. Br J Radiol2018; 91: 20170644. Disclosure of Interests None declared