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ObjectivesPleuroparenchymal fibroelastosis (PPFE) is characterized by predominantly upper lobe pleural and subjacent parenchymal fibrosis; PPFE features were described in patients with rheumatic autoimmune diseases (RAID). A systematic literature review was performed to investigate the prevalence, prognosis and potential association of PPFE with previous immunosuppression in RAID.MethodsEMBASE, Web of Science and PubMed databases were questioned from inception to 1 September 2019. Articles published in English and addressing PPFE in patients with RAID were selected.ResultsTwenty out of 794 papers were selected with a total of 76 cases of RAID-PPFE patients (20 SSc, 9 RA, 6 IIM6 primary SS, 5 overlap syndromes, 3 ANCA-associated vasculitides, 2 granulomatosis with polyangiitis, 1 microscopic polyangiitis, 1 UCTD, 1 SLE, 1 GCA and 21 patients with non-specified RAID). Dyspnoea was the most frequently reported symptom (37/48 patients, 77%). Patients frequently presented with a restrictive pattern and decline in diffusing lung capacity for carbon monoxide. During the follow-up, 7/12 patients had progression at imaging, 22/39 presented a generic clinical worsening, 19/38 had a functional deterioration and 15/43 remained stable.ConclusionThe present systematic literature review confirms that PPFE features are present in RAID. Rheumatologists should be aware of this new radiological pattern that holds a bad prognosis.

Background: In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 pneumonia from a progression of interstitial lung disease (ILD) secondary to Systemic sclerosis (SSc). The aim of the present study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia. Methods: This multicentric study included 22 international readers divided in the radiologist group (RAD) and non-radiologist group (nRAD). A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study. Findings: Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT features most frequently associated with SSc-ILD. The CT features most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p

Introduction: Severe morning stiffness with painful involvement of the girdles are often referred by patients with Interstitial Lung Disease (ILD), but the association between ILD and Polymyalgia Rheumatica (PMR) is rarely reported. The purpose of the work is to describe a series of patients classified as having PMR with ILD. Material and methods: We retrospectively enrolled patients with a diagnosis of PMR referred to our center during the previous year for respiratory symptoms. Data concerning clinical and serological manifestations suggesting Connective Tissue Disease (CTD), High-Resolution Chest Tomography (HRCT), and Pulmonary Function Tests (PFTs) were systematically collected in order to verify the diagnosis. Results: Fifteen out of seventeen PMR patients had ILD. Ten patients had a confirmed diagnosis of PMR, while in five patients a CTD was discovered. Seven patients showed a severe restrictive pattern at PFTs requiring oxygen supplementation (five with PMR and two with CTD). In thirteen patients pulmonary symptoms started before or together with muscular symptoms. Regarding HRCT patterns, patients showed a Nonspecific Interstitial Pneumonia in nine cases, Usual Interstitial Pneumonia (UIP) and possible UIP in two and three cases, and a single case of Organizing Pneumonia and Combined Pulmonary Fibrosis and Emphysema Syndrome. Conclusions: Lung involvement should be evaluated in PMR patients, especially if asthenia is poorly responsive to low doses of steroids. In these cases, the diagnosis should be re-evaluated in depth, looking for a seronegative Rheumatoid Arthritis, a clinically amyopathic myositis or Interstitial Pneumonia with Autoimmune features. Keywords: Interstitial lung disease, Polymyalgia rheumatica, Connective tissue disease, Antisynthetase syndrome, Myositis, Interstitial pneumonia with autoimmune features

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7–23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients’ survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

Objectives Quantitative computed tomography (QCT) is a promising tool for objective assessment of interstitial lung disease (ILD) related to connective tissue diseases (CTD). However, its validity was never investigated. The aim of this study was to assess QCT feasibility, face, and content validity evaluation concerning CTD-ILD. Methods A rheumatologist and a chest radiologist conceived an online survey with nine statements: Two about general issue involving CTD-ILD, one for the face validity, three both for content validity and feasibility. Each statement had to be rated with a score from 0 to 100, respectively, (complete disagreement and agreement). We considered a statement supported by the experts if the median score was ≥75.0. Results A panel of 14 experts (11 from Europe, three from America) with a nine years median experience was enrolled. All the statements about feasibility, face and content validity were supported, except for QCT capability to recognize elementary lesions. Conclusions The panel of experts supported feasibility, face, and content validity of QCT assessment concerning CTD-ILD. This may stimulate a greater use in clinical practice and further studies to confirm its discriminative properties and its construct validity.

This study aims to assess the peripheral blood cell count &ldquo, signature&rdquo, of Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) to discriminate promptly between COronaVIrus Disease 19 (COVID-19) and community-acquired pneumonia (CAP). We designed a retrospective case-control study, enrolling 525 patients (283 COVID-19 and 242 with CAP). All patients had a fever and at least one of the following signs: cough, chest pain, or dyspnea. We excluded patients treated with immunosuppressants, steroids, or affected by diseases known to modify blood cell count. COVID-19 patients showed a significant reduction in white blood cells (neutrophils, lymphocytes, monocytes, eosinophils) and platelets. We studied these parameters univariately, combined the significant ones in a multivariate model (AUROC 0.86, Nagelkerke PSEUDO-R2 0.5, Hosmer&ndash, Lemeshow p-value 0.9) and examined its discriminative performance in an internally-randomized validation cohort (AUROC 0.84). The cut-off selected according to Youden&rsquo, s Index (&minus, 0.13) showed a sensitivity of 84% and a specificity of 72% in the training cohort, and a sensitivity of 88% and a specificity of 73% in the validation cohort. In addition, we determined the probability of having COVID-19 pneumonia for each Model for possible Early COvid-19 Recognition (MECOR) Score value. In conclusion, our model could provide a simple, rapid, and cheap tool for prompt COVID-19 diagnostic triage in patients with CAP. The actual effectiveness should be evaluated in further, prospective studies also involving COVID-19 patients with negative nasopharyngeal swabs.

Nailfold videocapillaroscopy (NVC) is an easy tool used for the assessment of patients with Raynaud&rsquo, s phenomenon (RP) as possibly associated with systemic sclerosis (SSc). Recent insights have also highlighted its role in the diagnostic assessment of idiopathic inflammatory myopathies (IIMs). The aim of this study is to describe the diagnostic role of NVC in a series of 361 consecutive patients with interstitial lung disease (ILD). All the patients were assessed by clinical pulmonary and rheumatic examinations, blood exams, high-resolution computed tomography and NVC. NVC was considered positive only in the presence of avascular areas or giant capillaries, but also, the presence of bushy capillaries (BCs) was recorded. NVC was positive in 17.7% of ILD patients and in 78.1% of ILD patients associated with a diagnosis of connective tissue disease (CTD). In 25% of SSc-ILD patients, NVC proved necessary for a correct diagnosis. The presence of BCs and/or NVC positivity in ILD patients with normal levels of creatine phosphokinase is associated with amyopathic IIM, regardless the presence of RP. In conclusion, NVC is useful for the diagnostic assessment of incomplete forms of CTD and in amyopathic IIMs. NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of RP.

The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD.

Objective To evaluate the prevalence, clinical presentation, serological and morphological features of, and therapeutic options for Interstitial Lung Disease (ILD) in primary Sjogren's Syndrome (pSS). Methods Pubmed was searched between February 1996 and December 2018 using a combination of MESH terms related to pSS and ILD. Selected works were subjected to blind evaluation by two authors and a senior author in case of disagreement. The work followed PRISMA guidelines and was registered on PROSPERO (CRD42018118669). Results About 20% of pSS patients have ILD, with a 5-y survival of 84% and a need for supplemental oxygen in the 11–33% range. A significant proportion of ILD patients are seronegative without sicca syndrome. ILD seems to be associated with higher levels of Lactic Dehydrogenases and positivity for Anti-Ro52k. The prevalent pattern in High Resolution Computed Tomography is Nonspecific Interstitial Pneumonia (NSIP), but all other patterns can be present. No difference in mortality was found between patients with NSIP and Usual Interstitial Pneumonia patterns. Amyloidosis and primary lung lymphoma can be observed in about 10% of pSS patients. Conclusion The recognition of pSS underlying an ILD can be challenging in seronegative patients with no or mild sicca symptoms. A complete diagnostic assessment, including minor salivary glands and, in some cases, lung biopsy, should be performed on all patients at risk. A better recognition of the clinical or serological markers of ILD progression in these patients is warranted to drive the physicians to an early diagnosis and an effective treatment.

Background:Prevalence of anti-neutrophil cytoplasmic antibody (ANCA) in patient with idiopathic pulmonary fibrosis (IPF) ranges from 1 to 35%, mainly anti-MPO. The presence of ANCA positivity seems to be a poorer prognostic factor in patient with IPF, and some of these patients will develop clinical vasculitis (7-23%).Unfortunately, the majority of the available studies on this topic are retrospective and the real natural history of the disease remains poorly understood.Objectives:Aim of the study was to investigate the clinical, serological and radiologic features of patients with interstitial lung disease (ILD) and positivity for anti-MPO, and to evaluate the survival of this population compared with IPF patients.Methods:We retrospectively analysed 30 patients with ILD and anti-MPO antibodies, without diagnosis of vasculitis, from 3 different rheumatology-pulmonology Italian Center.For each patient, clinical, radiologic and serological data were evaluated. Treatments were also collected, both immunosuppressants or antifibrotic agents.Finally, survival of ILD-MPO patients and of 90 unselected idiopathic pulmonary fibrosis (IPF) patients was compared.Results:Thirty patients were enrolled in the study (see table for the characteristics of the patients).Fibrosing pneumonia was described in 73.3% of patients (usual interstitial pneumonia [UIP] in 19 patients), and 10 patients (33.3%) received antifibrotic drugs, all with UIP pattern. Of interest, 7 patients were treated with immunosuppressants (azathioprine, cyclophosphamide, mycophenolate mofetil), independently by the ILD pattern and 21 (70%) low dosage of steroids.After a median period of 23.5 months (range 11-111), 7 patients developed an ANCA associated vasculitis, while other 3 developed other rheumatic diseases.Finally, when compared with IPF, ILD-MPO patients had a better survival (81.2%±0.9 vs 54.7±0.7 for ILD-MPO and IPF, respectively; p=0.045)Conclusion:ILD positive for anti-MPO antibodies are still a not definite condition. We need larger population to identify possible markers for the evolution in an ANCA associated vasculitis, to define the prognosis of disease and the better therapeutic approach.References: :[1]Mohammad AJ, et al. Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype. J Rheumatol. 2017;44:1458-67Table.Serological, clinical and radiological features of anti-MPO + interstitial lung diseaseNumber30Males/female15/15Median age (years + IQR)68 (17)Median follow-up (months + IQR)39.5 (61)Smoke36.70%ILD pattern Usual interstitial pneumonia63.30% Nonspecific interstitial pneumonia16.70% Hipersensitivity pneumonia10% Other fibrosing pneumonia10%Median FVC (% + IQR)83 (23)Median DLCO (% + IQR)53 (28)Clinical features Raynaud’s phenomenon7.70% Sicca syndrome0 Arthralgias20% Arthritis3.40%Serology Antinuclear antibodies30.80% Anti-extractable nuclear antibodies (ENA)8% Anti-SSA4% Rheumatoid factor21.40%Therapy Immusuppressants23.30% Anti-fibrotic drugs33.30%Disclosure of Interests:None declared

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

Introduction: Connective Tissue Diseases (CTDs) are systemic autoimmune conditions characterized by frequent lung involvement. This usually takes the form of Interstitial Lung Disease (ILD), but Obstructive Lung Disease (OLD) and Pulmonary Artery Hypertension (PAH) can also occur. Lung involvement is often severe, representing the first cause of death in CTD. The aim of this study is to highlight the role of Pulmonary Function Tests (PFTs) in the diagnosis and follow up of CTD patients. Main body: Rheumatoid Arthritis (RA) showed mainly an ILD with a Usual Interstitial Pneumonia (UIP) pattern in High-Resolution Chest Tomography (HRCT). PFTs are able to highlight a RA-ILD before its clinical onset and to drive follow up of patients with Forced Vital Capacity (FVC) and Carbon Monoxide Diffusing Capacity (DLCO). In the course of Scleroderma Spectrum Disorders (SSDs) and Idiopathic Inflammatory Myopathies (IIMs), DLCO appears to be more sensitive than FVC in highlighting an ILD, but it can be compromised by the presence of PAH. A restrictive respiratory pattern can be present in IIMs and Systemic Lupus Erythematosus due to the inflammatory involvement of respiratory muscles, the presence of fatigue or diaphragm distress. Conclusions: The lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respiratory disease related to each disease.

Background: Interstitial lung disease (ILD) is a frequent manifestation of Sjögren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard for diagnosis. Semiquantitative CT (SQCT) based on visual assessment (Goh and Taouli scoring) can estimate ILD extent, although it is burdened by relevant intra- and interobserver variability. Quantitative chest CT (QCT) is a promising alternative modality to assess ILD severity. Aim: To determine whether QCT assessment can identify extensive or limited lung disease in patients with SS and ILD. Methods: This multi-center, cross-sectional and retrospective study enrolled patients with SS and a chest CT scan. SQCT assessment was carried out in a blinded and centralized manner to calculate both Goh and Taouli scores. An operator-independent analysis of all CT scans with the open-source software platform Horos was used to evaluate the QCT indices. Patients were classified according to the extent of ILD and differences in QCT index distribution were investigated with non-parametric tests. Results: From a total of 102 consecutive patients with SS, the prevalence of ILD was 35.3% (36/102). There was a statistically significant difference in QCT index distribution between the SS with ILD and SS without ILD groups (p20% (by Goh score) had a QCT index statistically different from those with limited ILD extent (p

Background:COVID-19 pandemic is a global emergency which may overlap on the clinical and radiological scenario of ILD in SSc. In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 superinfection from a progression of SSc-ILD.Objectives:The aim of our study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia.Methods:22 international readers were included and divided in the radiologist group (RAD) and non-radiologist group (nRAD). The RAD group included non-chest RAD and chest-RAD. A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study.Results:Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT parameters most frequently associated with SSc-ILD. The CT parameters most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p Conclusion:The CT differential diagnosis between COVID-19 Pneumonia and SSc-ILD is possible and may be fostered in practice by the use of a radiological score. In the case where an overlap of both diseases is suspected, the presence of consolidation in the lower lobes may suggest a COVID-19 pneumonia while the presence of fibrosis inside GGO may indicate a SSc-ILD.References:[1]Orlandi M, Landini N, Bruni C, et al. Infection or autoimmunity? The clinical challenge of interstitial lung disease in systemic sclerosis during COVID 19 pandemic. J Rheumatol. 2020 Dec 1: jrheum.200832[2]Simpson S, Kay FU, Abbara S, et al. Radiological Society of North America Expert Consensus Statement on Reporting Chest CT Findings Related to COVID-19. Endorsed by the Society of Thoracic Radiology, the American College of Radiology, and RSNA [published online ahead of print, 2020 Apr 28]. J Thorac Imaging. 2020;10.1097/RTI.0000000000000524.[3]Cheng C, Li C, Zhao T, et al. COVID-19 with rheumatic diseases: a report of 5 cases. Clin Rheumatol. 2020;39(7):2025-2029.[4]Mariano RZ, Rio APTD, Reis F. Covid-19 overlapping with systemic sclerosis. Rev Soc Bras Med Trop. 2020 Sep 21;53:e20200450.Disclosure of Interests:None declared

Background:Lung involvement remains the main cause of morbidity and mortality in SSc. In 1 year clinical trials to assess lung involvement, FVC is usually evaluated and changes while the DLCO usually remains unchanged. In longer term observational studies, the DLCO often changes more than the FVC.Objectives:To examine, through a systematic literature review(SLR) and meta-analysis, whether DLCO%pred or FVC%pred (both to be designated solely as DLCO and FVC henceforth), responds more in assessing SSC interstitial lung disease in first year and longer term follow-up(FU).Methods:PubMed, EMBASE and COCHRANE databases were searched for english language articles on SSc published between 1960 and 31st October 2018. Any study that made reference to FVC and DLCO evaluation in SSc and reported their changes over the years was included. Reviewers double extracted articles to obtain agreement on>95% of pre-defined critical outcomes:DLCO and FVC at baseline,1 yr of FU and at the last assessment interstitial lung disease by HRCT of the lungs study design, duration of FU. Other variables included demographics. In all cases I^2 test for heterogeneity was used and a result from 75 to 100% was considered as high heterogeneity.A random effects meta-analysis was used. Differences in the degree of change in FVC and DLCO were tested with t-test without compensation for repeated analysis.Results:1870 articles were screened for eligibility and 21 were selected for the SLR and meta-analysis The analysis used 21 studies to evaluate changes of FVC and DCLO at 1 yr of FU. Only 5 studies were eligible to evaluate changes during longer FU (24.7 months(SD=20.4)). Heterogeneity was high at baseline and FU, (at 1 yr and >1 yr),both for the 21 studies at 1 yr (FVC:I^2 99.74%, 99.78%; DLCO: I^2=99.91%99.92%) and the 5 studies at > 1 year (FVC:I^2 92.86%;DLCO:I^2=99.54%), diminishing the confidence in the results. Table 1 gives the results of the random effects meta-analysis both for 21 and for the 5 studies evaluated. Regarding changes over 1 yr in the 21 studies, change of mean FVC was 2.7 (78.8vs81.5) while change in mean DLCO was 3.07(SE 8.20) (59.7vs 63.4). Considering the 5 studies during long term follow up, change in mean FVC was 2.0(7.36)(81.1vs83.1), and change in mean DLCO was -0.96 (16.95)(55.6vs54.7). Comparing the 1 yr changes in the 21 studies, change in mean FVC was 2.7(5.2)and change in mean DLCO was 3.07(8.2); difference in changes was not statistically significant (p=0.5791). During long term FU (24.7 months), change in mean FVC was 2.0(7.36)and change in mean DLCO was -0.96 (16.95); difference in changes was not statistically different (p=0.4698).Table 1.Results from Random Effect Meta-Analysis21 studies5 studiesFVC % predmean valueSEDLCO % pred mean valueSEFVC % predmean valueSEDLCO % pred mean valueSEBASELINE78.83.4359.75.5281.15.6855.611.591 YEAR FOLLOW UP81.53.8563.46.07---->1 YEAR FOLLOW UP----83.44.6854.712.37Conclusion:Our data are limited by great heterogeneity. Given this limitation, this SLR and meta-analysis indicates that there is no difference in the changes comparing FVC to DLCO at either 1 yr or during longer term follow-up. Corroboration of these results in prospective studies and in registries to make clear, comparable comparisons will be needed.Disclosure of Interests:Silvia Bellando Randone: None declared, Eleonora Cappellini: None declared, Letizia Nidiaci: None declared, Gemma Lepri: None declared, Maria Grazia Lazzaroni: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Gianluca Bagnato: None declared, Domenico Sambataro: None declared, Gianluca Sambataro: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim, Daniel Furst Grant/research support from: AbbVie, Actelion, Amgen, BMS, Corbus Pharmaceuticals, the National Institutes of Health, Novartis, Pfizer, and Roche/Genentech, Consultant of: AbbVie, Actelion, Amgen, BMS, Cytori Therapeutics, Corbus Pharmaceuticals, the National Institutes of Health, Novartis, Pfizer, and Roche/Genentech, Speakers bureau: CMC Connect (McCann Health Company)

Background:Nailfold Videocapillaroscopy (NVC) is an essential tool for the assessment of Raynaud’s Phenomenon (RP) among the Scleroderma Spectrum Disorders (SSDs). Recently, NVC abnormalities have been associated with a diagnosis of Idiopathic Inflammatory Myopathies (IIMs), independently of the presence of RP (1). Moreover, both SSDs and IIMs are commonly associated with Interstitial Lung Disease (ILD), which is the main cause of mortality in these conditions.Objectives:To verify whether NVC may allow a better diagnostic classification in a cohort of patients with ILD followed by pulmonologists.Methods:361 patients affected by ILD were prospectively enrolled in a 30-months observational study. All these patients were clinically evaluated by rheumatologists and pulmonologists together and performed general blood tests, autoantibody research, chest High-Resolution Computed Tomography and NVC. The latter was considered positive in the presence of avascular areas or giant capillaries, and also the presence of Bushy Capillaries (BCs) was recorded.Results:NVC was positive in 17.7% of ILD patients, and a third of these patients did not present RP. Patients with NVC abnormalities had a diagnosis of definite Connective Tissue Disease in 78.1% of cases. NVC resulted decisive in 25% of patients with a final diagnosis of Systemic Sclerosis according to the ACR/EULAR 2013 criteria. The presence of BCs and/or NVC+ in ILD patients with normal serum levels of muscular enzymes was associated with amyopathic IIM (BCs= OR 3.9, 95CI 1.05-14.38, p=0.04; NVC+= OR 5, 95CI 1.29-19.3 p=0.02; BCs and/or NVC+= OR 5.41 95CI 1.24-23.48 p=0.02), regardless the presence of RP.Conclusion:NVC proved to be a valid tool in the correct assessment of ILD patients secondary to SSDs and amyopathic IIMs. Therefore, it could be considered in the diagnostic evaluation of patients affected by ILD, regardless of the presence of RP.References:[1]Sebastiani M et al. Nailfold Capillaroscopy Characteristics of Antisynthetase Syndrome and possible clinical associations: results of a multicenter International Study.J Rheumatol2019; 46: 279-284Table 1.Comparison between ILD patients with and without NVC positivity..ItemsNVC+ patientsNVC- patientspNumber64297Mean Age (±SD)61.4±13.367.8±9.80.001Female%65.649.10.01RP%68.717.1HRCT patterns%NSIP51.536.80.02OP3.18n.s.UIP-like31.1544.8n.s.DIP1.53.4n.s.LIP1.50.4n.s.Indeterminate10.96.7n.s.Final Diagnosis%SSDs45.31IIMs256.4Other CTDs6.213.5n.s.IPAF10.919.5n.s.IPF7.827.90.0007Legend:DIP: Desquamative Interstitial Pneumonia; HRCT: High Resolution Computed Tomography; IIMs: Idiopathic Inflammatory Myopathies, Poly/dermatomyositis, antisynthetase syndrome; IPAF: Interstitial Pneumonia with Autoimmune Features; IPF: Idiopathic Pulmonary Fibrosis; LIP: Lymphocytic Interstitial Pneumonia; NSIP: Nonspecific Interstitial Pneumonia; OP: Organising Pneumonia; RP: Raynaud’s Phenomenon; SSDs: Scleroderma Spectrum Disorders, Systemic Sclerosis + Mixed Connective Tissue Disease; UIP: Usual Interstitial Pneumonia.Disclosure of Interests:Gianluca Sambataro: None declared, Domenico Sambataro: None declared, Francesca Pignataro: None declared, Nicoletta Del Papa: None declared, Michele Colaci: None declared, Lorenzo Malatino: None declared, Alessandro Libra: None declared, Fabio Pino: None declared, Sebastiano Emanuele Torrisi Speakers bureau: Boehringer Ingelheim; F. Hoffmann-La Roche Ltd., Stefano Palmucci Consultant of: Boehringer Ingelheim, Speakers bureau: Boehringer Ingelheim, Delphi International Srl, and F. Hoffmann-La Roche Ltd., Lorenzo Cavagna: None declared, Carlo Vanchieri Grant/research support from: F. Hoffmann-La Roche Ltd., Consultant of: AstraZeneca, Boehringer Ingelheim, Chiesi, F. Hoffmann-La Roche Ltd., and Menarini, Speakers bureau: AstraZeneca, Boehringer Ingelheim, Chiesi, F. Hoffmann-La Roche Ltd., and Menarini

Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc) patients. Usually, patients have lung involvement characterized by ground glass opacities (GGOs), but honeycombing (HC) is also possible. The Wells score is a semi-quantitative index, which is able to assess ILD by distinguishing its main components. The aim of this work is to evaluate the Wells score in relation to the disease activity (DA) index. We enrolled 40 consecutive SSc-ILD patients (26 diffuse cutaneous form, dcSSc, and 14 limited form, lcSSc). All patients were evaluated by the European Scleroderma Study Group (ESSG) index, high-resolution computed tomography, transthoracic echocardiogram, pulmonary function tests (PTSs), and nailfold videocapillaroscopy for the number of microhemorrhages (NEMO) score. In our study, the total extent of ILD (TE-ILD), fibrosis and GGOs correlated with dyspnea (p = 0.03, 0.01 and 0.01 respectively), but not with the ESSG index. Considering only the dcSSc patients, TE-ILD and GGOs correlated with the ESSG index (r = 0.5 p = 0.009), while fibrosis grade correlated with disease duration and systolic pulmonary artery pressure. In conclusion, our data suggest that GGO correlates with DA, while fibrosis may be a sign of disease damage. The quantification of pulmonary involvement using the Wells score can be a useful tool for assessing the appropriate treatment in SSc patients.

Background: Mortality rate in patients infected by severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) can be related to the presence of comorbidities like diabetes, cardiovascular and pulmonary diseases. On the contrary, few data exist on the impact of CoronaVirus Disease 2019 (COVID-19) on patients with rheumatic disorders, namely in those having pulmonary involvement and treated with immunosuppressive agents. The present survey is aimed at knowing the impact of COVID-19 in a cohort of patients with systemic sclerosis (SSc). Methods: Telephone interviews were carried out during the COVID-19 outbreak in patients with SSc followed in a Rheumatic Disease Unit in Italy. Patients were asked for confirmed SARS-CoV-2 infection, symptoms suggestive of COVID-19, and modification of their therapy. Results: A total number of 526 patients with SSc were contacted and interviewed. Of them, 270 and 256 had limited cutaneous and diffuse cutaneous SSc, respectively. Interstitial lung disease (ILD) was present in 45% of patients and most of them (68.2%) were treated with immunosuppressive therapy. Only two patients were hospitalized for COVID-19-related pneumonia, and one of them died despite invasive ventilator support. An additional 11 patients reported flu-like symptoms compatible with a mild form of COVID-19. Nobody modified the therapy during the COVID-19 outbreak. Conclusion: Despite the large prevalence of ILD and immunosuppressive therapies, which can be considered risk factors for the occurrence and severity of incidental viral infections, the impact of COVID-19, in terms of mortality rate and morbidity, does not appear particularly severe in this large cohort of patients with SSc. Possible mechanisms influencing this figure are discussed.

Background The term Interstitial Pneumonia with Autoimmune Features (IPAF) describes patients with Interstitial Lung Diseases (ILDs) and clinical or serological features of autoimmune diseases insufficient to reach a specific classification of a Connective Tissue Disease (CTD). Currently, retrospective studies on IPAF patients have proven to be heterogeneous in general characteristics, outcomes and High-Resolution Computed Tomography (HRCT) pattern. This study aims to describe for the first time the clinical, serological and radiological features of a prospective cohort of IPAF patients. This cohort is then compared to a group of patients with Idiopathic Pulmonary Fibrosis (IPF). Material and methods From 626 consecutive ILD patients evaluated, 45 IPAF and a comparison cohort of 143 IPF patients were enrolled. All patients underwent clinical assessment with rheumatologic and respiratory evaluation, HRCT, Pulmonary Function Tests and Nailfold Videocapillaroscopy. Results The IPAF patients had a predominance of female gender (62.12%) with a median age of 66 years. The most common findings were: Nonspecific Interstitial Pneumonia (NSIP, 68.89%), Antinuclear Antibody positivity (17.77%) and Raynaud Phenomenon (31.11%). In comparison with IPF, IPAF patients showed younger age, better performances in Pulmonary Function Tests, less necessity of O2 support and predominance of female sex and NSIP pattern. Discussion This is the first report of a prospective cohort of IPAF patients. IPAF patients seem to have a less severe lung disease than IPF. IPAF criteria probably need to be revisited and validated, but their capacity to recruit patients with incomplete forms or early onset of CTD could be useful for further research.

Introduction: The term Interstitial Pneumonia with Autoimmune Features (IPAF) has been proposed to classify patients with Interstitial Lung Disease (ILD) associated with autoimmune signs not classifiable for a specific Connective Tissue Disease (CTD). Currently, no prospective IPAF cohort are reported. Aims and Objectives: To describe a prospective cohort of IPAF patients. Methods: The study was conducted according to the declaration of Helsinki. A total of 170 consecutive ILD patients with at least one IPAF criteria were considered in the last year (Fischer A, Eur Respir J 2015; 46 (4): 976-987). From these, 58 patients were able to satisfy classification criteria for CTDs, while 33 patients were classified as IPAF. Results: IPAF patients (69.7% females) referred a history of smoke in 51.5% of cases and reported a restrictive pattern in Pulmonary Function Tests in 66.7% of cases. A clinical domain was present in 72.7% of patients, whereas serological and morphological domains respectively in 72.7% and 87.9% of cases. The most frequent item from each domain were arthritis (42.4%), Antinuclear Antibodies (ANA) (30.3%) and Nonspecific Interstitial Pneumonia (75.8%) as HRCT pattern. No cases of ulcers, telangiectasia and mechanic’s hand were reported. Only ANA, Rheumatoid Factor, anti-SSA, anti DsDNA and Anti Citrullinated Protein Antibodies were found as serological domain. Conclusions: This study suggests that most of IPAF patients have a clinically relevant ILD without serological and clinical items highly specific for CTDs. When these items were present, patients were generally able to satisfy specific CTD criteria. Probably, some of IPAF patients can have an early form of CTDs.

The term interstitial pneumonia with autoimmune features (IPAF) has been proposed to define patients with interstitial lung disease (ILD) associated with autoimmune signs not classifiable for connective tissue diseases (CTDs). This new definition overcomes previous nomenclatures and provides a uniform structure for prospective studies through specific classification criteria.This work evaluates the characteristics of IPAF patients reported in the literature, to highlight potential limits through a comparative analysis and to suggest better performing classification criteria.Four retrospective studies on the IPAF population have been considered. The study subjects differed in age, sex, smoking habit, ILD pattern and outcomes. Another important difference lies in the diverse items considered in the classification criteria. The retrospective design of the studies and the absence from some of them of a rheumatologist clearly involved in the diagnosis may have influenced the data, but current IPAF criteria seem to include a rather heterogeneous population. To overcome these discrepancies, this review suggests a limitation in the use of single items and the exclusion of extremely specific CTD criteria. This should avoid the definition of IPAF for those diseases at different stages or at early onset. The investigation of a functional or morphological cut-off of pulmonary involvement would be useful.