Introduction: A novel highly pathogenic human coronavirus able to induce severe acute respiratory syndrome (SARS) has been recently recognized as the cause of the coronavirus disease 2019 (COVID-19) outbreak, which has spread rapidly from China to other countries. Little is known about laboratory prognostic markers in COVID-19 patients. The aim of our study was to describe the basic clotting parameters in COVID-19 patients and their prognostic role in different clinical forms of the disease. Material and Methods: We enrolled 67 COVID-19 patients admitted to the Emergency Department. A cohort of 67 age- and sex-matched non-COVID-19 patients with acute respiratory illness was used as a control group. For all patients, platelet count (PLT), prothrombin time (PT), activated thromboplastin time (aPTT), C-reactive protein (PCR), fibrinogen, and D-dimer were determined. The COVID-19 population was divided in two groups according to the presence or absence of SARS. The clotting factors values were compared between the groups. Results: At admission, the COVID-19 patients showed statistically significant increased levels of fibrinogen (601.5 (480–747) vs. 455 (352.5–588.5) mg/dL; p = 0.0000064), and a higher percentage of patients had fibrinogen levels >400 mg/dL (86% vs.58%; p = 0.0054) compared to the control group. The levels of fibrinogen were higher in COVID-19 patients with SARS compared to those without SARS (747 (600.0–834.0) vs. 567 (472.5–644.50); p = 0.0003). Conclusion: Fibrinogen seems to increase early in COVID-19 patients and may be used as a risk stratification marker for the early detection of a subgroup of COVID-19 patient at increased risk to develop SARS, who might benefit from a different and thorough clinical surveillance and treatment.

The purpose of this study was to evaluate the efficacy and safety of the Moderna-1273 mRNA vaccine for SARS-CoV-2 in patients with immune-mediated diseases under different treatments. Anti-trimeric spike protein antibodies were tested in 287 patients with rheumatic or autoimmune diseases (10% receiving mycophenolate mofetil, 15% low-dose glucocorticoids, 21% methotrexate, and 58% biologic/targeted synthetic drugs) at baseline and in 219 (76%) 4 weeks after the second Moderna-1273 mRNA vaccine dose. Family members or caretakers were enrolled as the controls. The neutralizing serum activity against SARS-CoV-2-G614, alpha, and beta variants in vitro and the cytotoxic T cell response to SARS-CoV-2 peptides were determined in a subgroup of patients and controls. Anti-SARS-CoV-2 antibody development, i.e., seroconversion, was observed in 69% of the mycophenolate-treated patients compared to 100% of both the patients taking other treatments and the controls (p < 0.0001). A dose-dependent impairment of the humoral response was observed in the mycophenolate-treated patients. A daily dose of >1 g at vaccination was a significant risk factor for non-seroconversion (ROC AUC 0.89, 95% CI 0.80–98, p < 0.0001). Moreover, in the seroconverted patients, a daily dose of >1 g of mycophenolate was associated with significantly lower anti-SARS-CoV-2 antibody titers, showing slightly reduced neutralizing serum activity but a comparable cytotoxic response compared to other immunosuppressants. In non-seroconverted patients treated with mycophenolate at a daily dose of >1 g, the cytotoxic activity elicited by viral peptides was also impaired. Mycophenolate treatment affects the Moderna-1273 mRNA vaccine immunogenicity in a dose-dependent manner, independent of rheumatological disease.

Connective tissue disease associated pulmonary arterial hypertension (CTD-PAH) has benefited from the major treatment advances that have occurred within pulmonary hypertension over the past three decades. Inclusion of CTD-PAH cases in pivotal clinical trials led to regulatory approval and drug availability. This has improved outcomes but there are additional challenges for management. First, the multifaceted co-morbidity related to the associated CTD needs treatment alongside PAH and may impact on diagnosis and evaluation of treatment response. Secondary, cardiac involvement, interstitial lung disease and predisposition to thromboembolism in CTD may lead to compound phenotypes where PH has multiple mechanisms as well as precapillary pulmonary vasculopathy of PAH. In general, especially for systemic sclerosis, CTD-PAH has worse long-term survival than idiopathic or familial PAH. However, CTD also present an opportunity for screening and early detection and treatment for associated PAH, and this may in the future be a major advantage over idiopathic disease where presentation inevitable only occurs at symptomatic stages and diagnosis may be delayed. This article reviews and summarises some of the recent developments in investigation and management of CTD-PAH.

Objectives: Many patients with systemic sclerosis (SSc) experience impaired hand function, yet the precise nature and impact of this impairment remains unclear. In this study, we explored the determinants of hand function impairment in SSc from a patient perspective and its impact on daily life. Additionally, we identified unmet care needs related to hand function impairment. Methods: Adult patients with SSc were included from the University Medical Centre Utrecht, the Netherlands, and Royal Free Hospital London, United Kingdom (UK). Face-to-face semi-structured interviews were conducted, transcribed verbatim and coded. Thematic analysis was performed to identify key themes. Hand function was evaluated using the modified Hand Mobility in Scleroderma (mHAMIS) and the Cochin Hand Function Scale (CHFS). Results: Thirty-three patients were included (n = 18 in the Netherlands, n = 15 in the UK). Three main themes were identified: symptoms, impact and (un)met needs. The symptoms theme captures the broad range of medical and functional complaints, often co-occurring and leading to significant hand function impairment. The impact theme describes how these symptoms limited daily activities, employment and leisure, and contributed to emotional distress and social isolation. The (un)met needs theme highlights varied coping strategies and experiences with care. While participants felt that patient education was sufficient when healthcare professionals addressed hand impairment, many reported a lack of tailored support and insufficient recognition of hand-related problems. Conclusion: Hand function impairment in SSc profoundly affects patients’ daily lives and well-being. Addressing this unmet need requires greater clinical awareness and more personalised and symptom-specific management strategies.

Gastric antral vascular ectasia is a frequent and potentially severe complication of systemic sclerosis. Management is presently limited to supportive care, acid suppression and endoscopic treatment. Many cases of gastric antral vascular ectasia tend to be refractory or partially responsive to standard treatment and require multiple endoscopic procedures to control the recurrent bleeding. Immunosuppression is not part of the recommended management of gastric antral vascular ectasia: limited data exist on the role of cyclophosphamide or autologous stem cell transplant in severe cases, but no prospective data or randomised controlled trial supports its routine use. Here, we present a case of an adult male patient with diffuse cutaneous systemic sclerosis complicated by arthritis and severe gastric antral vascular ectasia. The latter required multiple endoscopic procedures and remained transfusion-dependent. Due to progressive skin disease and active arthritis refractory to conventional synthetic disease-modifying antirheumatic drugs, the patient was started on tocilizumab. While he showed an early response in terms of scores related to skin involvement and arthritis, response to gastric antral vascular ectasia was unexpected. As soon as the biologic therapy was started, the patient was no longer transfusion-dependent and haemoglobin levels started to rise. Subsequent endoscopic investigations confirmed resolution of gastric antral vascular ectasia. This case is illustrative of an unexpected response to tocilizumab, and this observation is supported by the biological rationale of interleukin-6 in vascular remodelling.