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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.

ObjectivesThe aim of our work was to identify specific patterns in clinical features and nailfold capillary changes that may help in screening for pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).MethodsWe identified patients with SLE and type I PAH (n=20) without other connective tissue diseases and collected demographic, clinical and laboratory features. We selected as controls patients with SLE who underwent cardiopulmonary screening to exclude PAH (n=87): we collected demographic, clinical and laboratory features and performed nailfold videocapillaroscopy (NVC).ResultsAll patients with SLE-PAH were women; age and disease duration were not different from patients with SLE without PAH. Lupus anticoagulant (LAC)+and anti-ribonucleoprotein (RNP)+were more prevalent in patients with SLE-PAH (respectively, PAH 45.0% vs no-PAH 20.5%, p=0.042; PAH 45.0% vs no-PAH 19.5%, p=0.035). No differences were observed for anti-Sm, anti-Ro, anti-La and anti-cardiolipin and anti-beta2GPI antibodies. Among clinical features, mucocutaneous and central nervous system involvement were more prevalent in patients with SLE-PAH than in SLE controls (respectively, PAH 65.0% vs no-PAH 34.5%, p=0.024; PAH 25.0% vs no-PAH 8.0%, p=0.046). Raynaud’s phenomenon (RP) was more prevalent in patients with SLE-PAH than in SLE controls (PAH 60.0% vs no-PAH 13.8%, p<0.001). RP was a predictor of PAH in patients with SLE (OR 3.8 (0.9–14.8)). We performed NVC on nine patients with PAH and on controls: we observed a significantly higher prevalence of scleroderma pattern at NVC in SLE-PAH than controls (PAH 66.7% vs no-PAH 9.2%, p<0.001). Patients with SLE-PAH showed a lower number of capillary density and a higher frequency of giant capillaries.ConclusionsOur data showed that LAC+, RNP+, RP and a scleroderma pattern at NVC was indicative for patients with SLE-PAH. Our results pointed to generalised microvascular involvement and a hypercoagulation state in patients with SLE-PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE at risk of developing PAH.

The role of immunosuppression in SARS-CoV-2-related disease (COVID-19) is a matter of debate. We here describe the course and the outcome of COVID-19 in a cohort of patients undergoing treatment with calcineurin inhibitors. In this monocentric cohort study, data were collected from the COVID-19 outbreak in Italy up to 28 April 2020. Patients were followed at our hospital for solid organ transplantation or systemic rheumatic disorders (RMDs) and were on calcineurin inhibitor (CNI)-based therapy. Selected patients were referred from the North of Italy. The aim of our study was to evaluate the clinical course of COVID-19 in this setting. We evaluated 385 consecutive patients (220 males, 57%; median age 61 years, IQR 48–69); 331 (86%) received solid organ transplantation and 54 (14%) had a RMD. CNIs were the only immunosuppressant administered in 47 patients (12%). We identified 14 (4%) COVID-19 patients, all transplanted, mainly presenting with fever (86%) and diarrhea (71%). Twelve patients were hospitalized and two of them died, both with severe comorbidities. No patients developed acute respiratory distress syndrome or infectious complications. The surviving 10 patients are now fully recovered. The clinical course of COVID-19 patients on CNIs is generally mild, and the risk of superinfection seems low.

Objectives Systemic sclerosis (SSc) is characterised by vasculopathy, an aberrantly activated immune system and excessive extracellular matrix deposition. Inflammatory chemokines control migration of cells to sites of tissue damage; their removal from inflamed sites is essential for resolution of the inflammatory response. The atypical chemokine receptor D6 has a critical role in this physiological balance. To explore potential deregulation of this system in SSc, inflammatory chemokine and D6 expression were compared with that in healthy controls (HC). Methods Serum levels of inflammatory mediators were assessed by luminex analysis. Peripheral blood mononuclear cells (PBMCs) were used in molecular and immunocytochemical analysis. Platelet-rich plasma was collected and assessed by western blotting for D6 expression levels. Sex-matched HC were used for comparison. Results 72 patients with SSc and 30 HC were enrolled in the study. The chemokines MCP-1/CCL2, MIP-1α/CCL3, MIP-1β/CCL4 and IL-8/CXCL8 were significantly increased in patients with SSc, regardless of disease subtype and phase. Quantitative PCR analysis revealed a significant 10-fold upregulation of D6 transcripts in patients with SSc compared with controls, and this was paralleled by increased D6 protein expression in the PBMCs of patients with SSc. Platelet lysates also showed strong D6 expression in patients with SSc but not in controls. Importantly, high levels of D6 expression correlated with reduced levels of its ligands in serum. Conclusions Inflammatory chemokines and the regulatory receptor D6 are significantly upregulated in SSc and high D6 levels are associated with lower systemic chemokine levels, indicating that some patients control systemic chemokine levels using D6. These results suggest that chemokines may represent a therapeutic target in SSc.

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.

ObjectiveThe aim of our work is to analyze the clinical and demographic features and nailfold capillary changes in patients with SLE-related PAH compared to a group of SLE patients without PAH.MethodsWe identified and selected 20 patients with SLE and type I PAH and collected demographic, clinical and laboratory features from 8 rheumatology centers across Europe. We could perform NVC on 9 patients. We selected as controls 68 patients with SLE who underwent cardiopulmonary screening to exclude PAH: we collected demographic, clinical and laboratory features and performed NVC. The presence of SD pattern was assessed according to Smith et al (Autoimmunity Reviews 2019). Patients satisfied the 2019 EULAR/ACR SLE classification criteria. We excluded patients with a diagnosis of mixed tissue disease and overlap syndrome.ResultsAll patients with SLE-PAH were female; age and disease duration were not different from SLE patients without PAH. LAC+ and anti-RNP+ was more prevalent in patients with SLE-PAH. No differences were observed for anti-Sm, anti-Ro, anti-La and anti-phospholipid antibodies. Of clinical features, skin and CNS involvement were more prevalent in patients with SLE-PAH than in SLE controls. Raynaud’s phenomenon was more prevalent in patients with SLE-PAH than in SLE controls. In patients with SLE-PAH we observed a significantly higher prevalence of scleroderma pattern at NVC than in SLE controls: patients with SLE-PAH showed a lower number of capillary density and a higher frequency of megacapillaries. In multivariate analysis, Raynaud phenomenon and anti-RNP are predictors of PAH in patients with SLE. The McFadden’s R-squared for the model is 0.30.ConclusionsOur data show that LAC+, RNP+, Raynaud’s, Skin and CNS involvement and a SD pattern at NVC is more prevalent in patients with SLE PAH than in patients with SLE without PAH. Our results point to a generalized microvascular involvement and a hypercoagulation state in patients with SLE-PAH. The variables we identified could be used to implement a screening algorithm to identify patients with SLE with a high risk of developing PAH.AcknowledgementsThe study was supported by SLEuro.

Correspondence to Professor Carlomaurizio Montecucco, Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Lombardia, Italy; montecucco@smatteo.pv.it We thank Perniola et al for their interest in our paper and the circumstantial details on their outpatient activity.1 Our service is a referral rheumatology department with more than 100 outpatients evaluated every day. Since the first Italian subject has been diagnosed with COVID-19 and transferred to our Hospital on 21 February 2020, a prompt reorganisation of our department and of the entire hospital facilities has been carried out in order to create a COVID-19 hub hospital and deal with the emergency at best.2 Starting 24 February 2020, we have severely restricted the access to our outpatient clinic. [...]patients had to undergo a three-step authentication in order to access the platform and shared data were stored in protected servers of our institution and automatically destroyed after 45 days. [...]a spike in deadly manifestations such as sudden cardiac death has been reported, due to the inaccessibility of routine care or fear to appeal to medical help.5 In the light of these reports, we have still to evaluate the usefulness of telemedicine in new patients requiring prompt diagnosis and very early referral as in the detection and fast tracking of large vessel vasculitides.6 It is likely that we will be able to better balance the consequences of such delays that occurred during the COVID-19 pandemic, in the future and we should get ready to manage them in the best possible way.

[...]unlike inflammatory arthritis, in which disease flares are in most cases symptomatic and impacting on patients’ quality of life and functionality, CTDs like systemic lupus erythematosus (SLE) or systemic sclerosis may give rise to more insidious manifestations. The platform at our disposal allows both visual interaction and real-time sharing of test results.3 Following this approach, from 24 February to 17 April, we evaluated in person 47 patients against the 315 visits planned before the severe acute respiratory syndrome coronavirus 2 outbreak (15%). In order to make telemedicine even more reliable, several digital applications for monitoring disease activity are already available for RA,4 and efforts are being made in order to evaluate their impact on disease control and treatment adherence.5 6 However, a treat-to-target strategy exclusively based on PROs is debated also for RA and might be problematic especially for patients in a near-remission status or with established disease.7 8 In conclusion, we believe that the identification of the most suitable subsets of patients and the development of flexible approaches—allowing a prompt switch to inperson evaluation when necessary—are of utmost importance to provide good-quality healthcare assistance.

Objectives To assess the effects of Rituximab (RTX) on skin and lung fibrosis in patients with systemic sclerosis (SSc) belonging to the European Scleroderma Trial and Research (EUSTAR) cohort and using a nested case-control design. Methods Inclusion criteria were fulfilment of American College of Rheumatology classification criteria for SSc, treatment with RTX and availability of follow-up data. RTX-treated patients were matched with control patients from the EUSTAR database not treated with RTX. Matching parameters for skin/lung fibrosis were the modified Rodnan Skin Score (mRSS), forced vital capacity (FVC), follow-up duration, scleroderma subtype, disease duration and immunosuppressive co-treatment. The primary analysis was mRSS change from baseline to follow-up in the RTX group compared with the control group. Secondary analyses included change of FVC and safety measures. Results 63 patients treated with RTX were included in the analysis. The case-control analysis in patients with severe diffuse SSc showed that mRSS changes were larger in the RTX group versus matched controls (N=25; −24.0±5.2% vs −7.7±4.3%; p=0.03). Moreover, in RTX-treated patients, the mean mRSS was significantly reduced at follow-up compared with baseline (26.6±1.4 vs 20.3±1.8; p=0.0001). In addition, in patients with interstitial lung disease, RTX prevented significantly the further decline of FVC compared with matched controls (N=9; 0.4±4.4% vs −7.7±3.6%; p=0.02). Safety measures showed a good profile consistent with previous studies in autoimmune rheumatic diseases. Conclusions The comparison of RTX treated versus untreated matched-control SSc patients from the EUSTAR cohort demonstrated improvement of skin fibrosis and prevention of worsening lung fibrosis, supporting the therapeutic concept of B cell inhibition in SSc.