OBJECTIVE: Systemic Sclerosis (SSc) is characterized by widespread microangiopathy and fibrosis of skin and visceral organs. Left ventricle involvement is usually subclinical, characterized by systolic and/or diastolic dysfunction. The global longitudinal strain (GLS), a validated and reliable technique for the measurement of ventricular longitudinal deformation by means of echocardiography, may detect subclinical systolic dysfunction of SSc myocardium. The improvement of myocardial perfusion by means of intravenous Iloprost administration could ameliorate the contractility of SSc heart. Therefore, we aimed to evaluate GLS in a series of SSc patients prior and after Iloprost infusion.
METHODS: Fifteen consecutive SSc patients (age: 54 ± 11 years; 12 females) treated with Iloprost because of the presence/history of digital ulcers underwent echocardiography, including GLS technique. This evaluation was conducted immediately before Iloprost administration and at the end of the 6-h infusion session.
RESULTS: Significant improvement in the mean GLS was observed after Iloprost administration (from -13.5 ± 2.5 to -15 ± 3.3; p= 0.011). The echocardiographic data obtained from the four-chamber view showed the best quality for GLS analysis and showed a highly significant improvement of the strain after Iloprost administration (from -13.4 ± 2.2 to -15.6 ± 3; p= 0.001). The degree of GLS improvement did not correlate with any SSc parameters.
CONCLUSIONS: Iloprost administration improved GLS, suggesting that the increase of myocardial perfusion allowed, at least in part, a correction of left ventricular systolic dysfunction. Further studies are needed to confirm these findings, further exploring the mid/long-term effects of Iloprost on myocardial contraction.

Scleroderma is a complex autoimmune disorder that primarily affects the connective tissue. Its key pathogenesis comprises vascular abnormalities, autoimmunity, and tissue fibrosis. While the exact etiology of the disease is unclear, patients may exhibit a wide array of symptoms. Scleroderma can rarely induce systemic effects that alter normal cervical spine anatomy. The effects on the cervical spine may be mediated through autoimmune phenomena or dystrophic calcinosis along the vertebral column. We discuss a rare case involving a 60-year-old female with a four-month history of scleroderma, who presented with cervical kyphosis, neck pain, impaired ambulation, dysphagia, edema, and reduced range of motion.

We present the case of a 63-year-old female diagnosed with atypical SSc in the setting of acute SRC. She was undergoing work-up for progressive dyspnoea in the outpatient setting when she was found to have newly diagnosed restrictive lung pathology and worsening renal function, thus prompting acute hospital admission. Given multisystem involvement of the pulmonary and renal systems, the differential diagnosis included autoimmune and connective tissue disorders. Although serologies were non-specific, renal biopsy confirmed scleroderma renal disease, and she was started on treatment with captopril. This case highlights the importance of clinical judgment and timely diagnosis, even when laboratory data might indicate otherwise.
CONCLUSIONS: Scleroderma renal crisis (SRC) remains an important cause of morbidity and mortality in systemic sclerosis (SSc), and clinicians should have a high index of suspicion to diagnose it.The absence of specific serologic markers makes SSc diagnosis challenging and necessitates reliance on clinical findings and additional diagnostic tools such as imaging studies and tissue sampling.

(1) Background: Bullous morphea is an extremely rare form of localized scleroderma, a condition that is marked by the presence of sporadic and intermittent blisters on sclerodermatous skin. This condition stands out due to its rarity and the unique manifestation of blistering, which sets it apart from other forms of localized scleroderma. Due to the infrequent presentation of bullous morphea, there is a significant gap in our understanding of its pathogenesis. The exact mechanisms that lead to the development of this condition remain largely unknown, which poses a challenge for medical professionals in terms of both diagnosis and treatment. The limited number of reported cases makes it difficult to establish a standardized approach to managing this condition, and as a result, treatment options are often limited and may vary from one patient to another. (2) Methods: In this case report, we present a rare case of bullous morphea that manifested before the onset of autoimmune hepatitis. When morphea presents unusually or is resistant to traditional immunosuppressive treatment, a comprehensive assessment of possible concurrent autoimmune illnesses provoking the rash must be conducted. (3) Results: We report a successful case of bullous morphea treated with systemic corticosteroids following a diagnosis of autoimmune hepatitis. (4) Conclusions: This case highlights the importance of considering overlapping autoimmune conditions in the management of bullous morphea and the potential efficacy of systemic corticosteroids in such scenarios. Collaborative efforts involving dermatologists, rheumatologists, and hepatologists are essential to enhance understanding and optimize treatment outcomes for patients affected by this rare and complex condition. Thus, further research is necessary to gain a deeper understanding of the pathogenesis of bullous morphea and to develop more effective and targeted treatment options for patients affected by this condition.

Excimer light is a subtype of NB-UVB that emits a 308 nm wavelength, and can provide targeted phototherapy treatment. The absorption of 308 nm light by skin cells leads to therapeutic response in various common and ultraviolet-responsive skin diseases, such as psoriasis and vitiligo, and photo-resistant skin diseases such as prurigo nodularis, localized scleroderma, genital lichen sclerosis, and granuloma annulare, cutaneous T-cell lymphomas, among others. Excimer light has few adverse reactions and overall is well tolerated by patients, furthermore, it can be performed in places that are difficult to access. This article aims to explain the therapeutic bases and applications of excimer light in current dermatology.

[This corrects the article DOI: 10.3389/fimmu.2024.1351675.].

OBJECTIVE: Raynaud\'s phenomenon (RP) is a symptom complex associated with digital vascular compromise. Our aim was to examine for clinically relevant differences between primary RP (PRP) and secondary RP (SRP) to connective tissue disease.
METHODS: We report cross-sectional results from the Patient Survey of experiences of Raynaud\'s Phenomenon (PASRAP), which aimed to explore the broad-ranging impact of RP. The survey was widely distributed online including via social medial. Participation was voluntary and responses were anonymous.
RESULTS: 1229 respondents completed PASRAP with self-reported RP: PRP 218 (17.7 %) and SRP 1011 (82.3 %) of which 903 (92.9 %) Systemic Sclerosis. The mean (SD) age was significantly lower in respondents with PRP (41.7 [11.8] vs 54.2 [12.4] years, P<0.0001). During attacks, more subjects with SRP reported cyanotic colour changes (92.2 % vs 86.5 %, P=0.0089). Patients with PRP experienced more pain (72.1 % vs 55.9 %, P<0.0001), numbness (80.3 % vs 69.4 %, P=0.0016), stinging/throbbing (93.4 % vs 80.8 %, P<0.0001), and tingling (84.0 % vs 77.5 %, P=0.0345). Only half of respondents\' symptoms were adequately controlled by their current medication(s), more commonly in SRP (55.2 % vs 45.2 %, P=0.0084). There were important differences in the triggers, number, and seasonal variation of RP attacks.
CONCLUSIONS: There are clinically relevant differences between PRP and SRP concerning the multifaceted lived patient experience of RP. Neurosensory symptoms are more common in PRP. Patients with SRP are older and present with more colour changes, overrepresented by cyanosis, and with less complete resolution of symptoms between attacks. These data provide novel insights for future RP clinical trial design.

Seven of the 11 newer medications recently or soon to be approved to treat rheumatologic diseases discussed in this article are biologic agents and reflect the current ability of science to target specific components of the immune system. The other agents are molecules that are directed against specific immune pathway targets as well. All have shown superiority to placebo and in some cases have been compared to currently accepted therapies. Safety issues are generally centered around infections due to the immune-interrupting nature of these therapies.

Selexipag is indicated for the treatment of pulmonary arterial hypertension (PAH), including PAH associated with connective tissue disease (CTD), and further insights into the management of selexipag-treated PAH-CTD patients in clinical settings are needed. These analyses of the ongoing, multicenter, prospective EXPOSURE (EUPAS19085) study describe characteristics, treatment patterns, tolerability, and outcomes of PAH-CTD patients initiating selexipag in Europe/Canada. All analyses were descriptive, with idiopathic PAH patients who typically display better prognosis included for context. Six hundred ninety-eight selexipag-treated patients had follow-up information; 178 (26%) had PAH-CTD. The median age was 68 years, patients were predominantly female (88%), and with WHO functional class III symptoms (63%); the median time since diagnosis was 1.7 years. There were 5% patients at low, 25% intermediate-low, 40% intermediate-high, and 30% high risk of 1-year mortality, according to the ESC/ERS 4-strata risk score. Most (80%) initiated selexipag as a triple oral therapy, and most of these (62%) remained on triple therapy 6 months post-baseline. Over a median (Q1-Q3) selexipag exposure period of 8.6 (2.5-17.2) months, 79 (44%) patients discontinued selexipag; 36 (20%) due to tolerability/adverse events. Sixty (34%) patients were hospitalized at least once; 120 hospitalizations occurred, with 49 (48%) deemed PAH-related. Survival at 1 year was 85%, and at 2 years was 71%; 29 (16%) patients died. These results describe the use of combination therapy with selexipag for patients with PAH-CTD. These findings suggest an opportunity to optimize the benefits of selexipag among patients with PAH-CTD by moving from escalating after years in response to clinical deterioration to escalating sooner to prevent clinical deterioration.

UNASSIGNED: Systemic sclerosis (SSc) is a rare connective tissue disease causing pain, reduced mobility and decreased health-related quality of life (HRQoL). Studies suggest that exercise is a necessary adjunct to the medical treatment.
UNASSIGNED: To examine the feasibility of a 12-week home-based exercise program for SSc patients, and evaluate changes in physical function, HRQoL and SSc-related symptoms.
UNASSIGNED: Twenty patients were enrolled in the study. Feasibility was measured by adherence, adverse events, and exercise task self-efficacy. At baseline and follow-up patients completed the 6-minute walk test, 30-second sit-to-stand test, 30-second arm curl, SSc Impact of Disease (ScleroID) and 36-item short form survey (SF-36).
UNASSIGNED: Median adherence to the intervention was 36 (interquartile range 27-36) out of a total of 36 exercise sessions. Fifteen patients completed the intervention, with three dropouts. Patients\' exercise task self-efficacy was 98%, 93% and 78%, for one, two and three weekly exercise sessions, respectively. There were no adverse events related to the exercise sessions. Improvements were observed in all physical tests, and minor improvements in HRQoL and SSc-related symptoms.
UNASSIGNED: This study suggests that a home-based exercise intervention is feasible for patients with SSc. The results suggest improvements in physical function, HRQoL and SSc-related symptoms.