Systemic lupus erythematosus (SLE) is characterized by multisystemic clinical manifestations ranging from a relatively mild involvement to potentially life-threatening complications. Due to this complexity, a multidisciplinary (MD) approach is the best strategy for optimizing patients’ care. The main aim of this systematic literature review (SLR) was to scrutinize the published data regarding the MD approach for the management of SLE patients. The secondary objective was to evaluate the outcomes of the MD approach in SLE patients. The Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were used. We performed an SLR to retrieve articles available in English or Italian listed in PubMed, Embase, Cinahl, and Cochrane Library concerning the MD approach used in observational studies and clinical trials. Four independent reviewers performed the study selection and data collection. Of 5451 abstracts evaluated, 19 studies were included in the SLR. The MD approach was most frequently described in the context of SLE pregnancy, reported in 10 papers. MD teams were composed of a rheumatologist, except for one cohort study; a gynecologist; a psychologist; a nurse; and other health professionals. MD approaches had a positive impact on pregnancy-related complications and disease flares and improved SLE psychological impact. Although international recommendations advise an MD approach for managing SLE, our review highlighted the paucity of data supporting this strategy, with most of the available evidence on the management of SLE during pregnancy.

The current coronavirus disease 2019 (COVID-19) pandemic is a global challenge with strong medical and socioeconomic implications. Hopes have been placed in the development of various vaccines. As the vaccination campaign is in progress, adverse effects need to be monitored closely. Possible side effects range from minor events to more serious manifestations. In this article, we describe two cases of erythema nodosum (EN) after COVID-19 vaccination in two previously healthy female patients of 59 and 51 years, respectively. Most of the usual etiologies of EN were excluded by laboratory testing. EN was successfully treated with corticosteroids. Remarkably, in the first case, a relapse occurred 48 hours after the second dose of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine. In this case series, we describe two unusual occurrences of EN after vaccination with an mRNA COVID-19 vaccine and a viral vector vaccine, respectively, and we discuss the available related literature.

Background: Systemic sclerosis is associated with an increased incidence of malignancies, in particular solid neoplasms. Hematological cancers have been also observed in autoimmune diseases, though rarely present with lung involvement. The latter may be misdiagnosed in systemic sclerosis patients, due to the frequent concomitant interstitial lung disease. Case description: Here, we present the case of a 63-year-old man affected by systemic sclerosis presenting with an atypical lung imaging and splenomegaly, who was diagnosed with splenic marginal zone lymphoma, thus raising the suspicion of lung secondarism. We discuss the diagnostic challenge of differential diagnosis in interstitial lung presentation and briefly review the available literature on this topic. Conclusion: Several reports have demonstrated an increased risk of malignancy in patients with systemic sclerosis. Still, the lack of concretely defined guidelines for systemic sclerosis, along with systemic sclerosis multifaceted organ involvement at presentation, may challenge diagnosis and management. Here, we remark the importance of clinical work-up and a multidisciplinary approach in systemic sclerosis, to early detect and treat concomitant hematological malignancies, especially during the first years of the disease.

BackgroundSystemic Sclerosis (SSc) is a complex autoimmune disease characterized by vascular damage, immune activation and fibrosis of skin and internal organs 1. Raynaud phenomenon (RP) is frequently the first symptom of the disease and growing evidences are supporting the hypothesis the SSc may be a vascular disease, with a pivotal role of endothelial cells, particularly in the very early phase2,3. Robust data support the use of vascular active drug to treat RP and to prevent vascular complication4–7.ObjectivesThe use of prostacyclin analog (PA) is evertything but standardized, with different regimen used all around the Country. We report data on the use of PA in a multicentric regional reality to understand which regimen are prevalent (and why) and if there is the opportunity to standardized them.MethodsWe collected data from an online survey exploring different items related to the use of PA.ResultsSurvey was fullfilled by 12 sites: 5 university hospital and 7 local hospitals, 7 driven by Rheumatologist and 5 from internal medicine specialists with/without concomitant rheumatologists. PA are ubiquitarly used for SSc-related digital ulcers (SSc-DU) and secondary RP but only a half of sites use it for primary RP. Seventy-five percent of sites (9/12) dispense PA at least once a month, but some other (1 each one respectively) on weekly basis, every other month or every 7 weeks. Drug administration may last from 2 to 5 consecutive days (mean 1.91+/- 1.5SD) with drug dose ranging from 0.5 to 2 ng/Kg/min with a minimum variability from site to site. Our regional hospitals may count on overall 68 spots, some available as beds (outpatient or inpatient), some as reclining chair or chair (outpatients only). University centers have usually more assigned personnel than local hospital (on average: 2 versus 1.5 physicians, 2 versus 1.2 nurse). Sites are able to offer meals (except one) and are able to accomodate from 1 to 12 patients at the same time (mean 3.45, +/- 3.2SD).ConclusionPA has known benefit in vascular involvement in SSc patients. Despite a multicenter palcebo-control study8 defining time and dose of this drugs and subsequent data based on the same regimen9, there is no homogeneity in treatment administration. The unequal treatment, based on our data, seems due to limited resources and personnel. High variability has been found in regimen duration and administration frequency.References[1]Ferri, C. et al. Systemic sclerosis evolution of disease pathomorphosis and survival. Our experience on Italian patients’ population and review of the literature. Autoimmunity Reviews vol. 13 1026–1034 (2014).[2]Mulligan-Kehoe, M. J. et al. Antiangiogenic plasma activity in patients with systemic sclerosis. Arthritis Rheum.56, 3448–58 (2007).[3]Wigley, F. M. Vascular disease in scleroderma. Clin. Rev. Allergy Immunol.36, 150–75 (2009).[4]Brueckner, C. S. et al. Effect of sildenafil on digital ulcers in systemic sclerosis: Analysis from a single centre pilot study. Ann. Rheum. Dis.69, 1475–1478 (2010).[5]Kowal-Bielecka, O. et al. EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann. Rheum. Dis.68, 620–628 (2009).[6]Matucci-Cerinic, M. et al. Bosentan treatment of digital ulcers related to systemic sclerosis: Results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann. Rheum. Dis.70, 32–38 (2011).[7]Herrick, A. L. & Wigley, F. M. Raynaud’s phenomenon. Best Practice and Research: Clinical Rheumatology (2020) doi:10.1016/j.berh.2019.101474.[8]Wigley, F. M. et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis: A multicenter, placebo-controlled, double- blind study. Ann. Intern. Med.120, 199–206 (1994).[9]Cappelli, L. & Wigley, F. M. Management of Raynaud Phenomenon and Digital Ulcers in Scleroderma. Rheumatic Disease Clinics of North America vol. 41 419–438 (2015).Disclosure of InterestsNone declared