Mediterranean Journal of Rheumatology最新文献

Despite advances in the management of systemic lupus erythematosus (SLE), it remains a chronic disease with frequent flares, requiring constant medical care, laboratory exams, hospitalisations, and the use of immunosuppressive drugs and corticosteroids, increasing the morbidity and mortality of these patients. The past decade of research has brought to light multiple observations on the role of interferons (IFNs) in the pathogenesis of SLE, which paved the way for the development of potential novel therapies targeting the interferon pathway. Following two phase III trials, anifrolumab, a monoclonal antibody which binds to the type I IFN receptor, blocking the activity of type I IFNs, was approved for active SLE. This review summarises the latest research on the role and mechanisms of type I IFNs in SLE and the development and advances on new therapeutic drugs based on IFN inhibition for SLE.

Systemic lupus erythematosus (SLE) has a spectrum of phenotypes. Its management should target remission or low disease activity, prevention of relapses and organ damage, minimisation of drug-related harms, and optimisation of health-related quality of life. Advances in our understanding of the disease pathophysiology have expanded the treatment armamentarium with targeted biologics that demonstrate superiority over conventional drugs in controlling activity, reducing flares and glucocorticoid exposure, and improving patient-related outcomes. In view of this, there is a critical need for real-world evidence providing insight into the spectrum of activity, the treatment landscape, and unmet needs among SLE patients. Such information can support regulatory and reimbursement decision-making. The primary objective of the NYMERIA multicentre study is to generate real-world evidence on the activity state of SLE patients treated in routine care settings in Greece. The overarching aim is to capture the disease burden based on both clinical aspects and the patient perspective.

Lupus enteritis is a rare gastrointestinal complication of systemic lupus erythematosus (SLE) associated with significant morbidity and mortality. Rituximab, a monoclonal antibody targeting CD20-positive B cells, has shown promise in refractory SLE cases. We present a case of a 45-year-old female with SLE who developed lupus enteritis and experienced an unusually rapid and remarkable response to Rituximab. The patient presented with severe abdominal pain and distension. Within two days of Rituximab treatment, the patient's abdominal pain, distension, and associated complications resolved completely. This exceptional response challenges the typical timeline of Rituximab efficacy in SLE and highlights the need for further investigation into the factors influencing treatment response. Understanding the mechanisms underlying such rapid improvement may provide insights into SLE pathogenesis and guide therapeutic strategies for optimal outcomes.

Background: Systemic sclerosis (SSc) is an autoimmune disorder characterised by skin fibrosis leading to skin tightening and disfigurement. However, there is no definite treatment for SSc and its skin complications. Fractional carbon dioxide laser has been widely used for different cutaneous pathologies. This study aims to evaluate the benefits of CO2 laser resurfacing on microstomia and peri-oral rhytids in systemic sclerosis patients.

Method and patients: 33 systemic sclerosis patients were enrolled. Four sessions of CO2 laser treatment were performed at an interval of four weeks. Patients were evaluated monthly. The interincisal distance (IID) measurement was used to evaluate maximal mouth opening capacity, and the mouth handicap in systemic sclerosis (MHISS) scale was used to assess the improvement after treatment.

Results: All of the participants were female with a mean age of 47.46 and a standard deviation of 7.11. The mean disease duration was 12.35. The mean total core of the MHISS scale was 25.24, and the mean IID was 48.11 millimetres before the treatment with CO2 laser. The MHISS score decreased, and patient satisfaction increased after treatment. However, observed differences in the results of IID were not statistically significant.

Conclusion: In conclusion, it seems that the fractional carbon dioxide laser is effective in the improvement of perioral rhytids, patient satisfaction based on the MHISS score, and mouth disability in SSc patients.

MDA5+ DM, or anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (DM), is a rare autoimmune illness that primarily affects women of Asian origin. The typical presentation of MDA5+ DM includes a variety of cutaneous lesions accompanied by either no muscular weakness (amyopathic) or hypomyopathic features. In patients with MDA5+ DM, rapid progression of interstitial lung disease is a frequent manifestation associated with poor prognosis. Pneumomediastinum, spontaneous intramuscular haemorrhage, and macrophage activation syndrome form a spectrum of rare manifestations associated with MDA5+ DM. Since these issues are uncommon but fatal, it's important to explore the approaches for diagnosis, treatments, and possible mechanisms, that are useful for prompt treatments and management of the patient.

Sjögren's syndrome (SS) is less frequently diagnosed in childhood than in adults, and central nervous system (CNS) disease is among the rarest systemic involvements. Thus, the clinical spectrum of CNS diseases and their management strategies have not been fully defined. In this article, we present the case of a 16-year-old girl who was referred for severe headache and diagnosed with SS with CNS involvement. Several immunosuppressive treatments failed to improve her neurological symptoms until the rituximab treatment. When we systematically reviewed the literature on cases of CNS involvement in childhood-onset SS, we found that CNS involvement was the presenting feature at the diagnosis of SS in the majority of published cases. While headache and fever were the most frequent complaints at presentation, most of the children displayed features of neuromyelitis optica spectrum disorder. CNS disease showed a variable response to immunosuppressives, and residual neurological deficits were not rare. Additionally, a significant number of cases required treatment with rituximab due to the treatment failures or subsequent flares. Sjögren's syndrome should be considered in children presenting with predominant neurological symptoms, and careful evaluation of glandular features might help in the prompt diagnosis of childhood-onset SS in children with CNS disease.

COVID-19, caused by a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), can lead to severe infection and has been suggested to induce autoimmune phenomena. We report three cases of rheumatoid arthritis (RA) occurring after COVID-19 infection and we present a systematic review of the literature of cases of RA post COVID-19. Our findings suggest that RA may be trigged by COVID-19 infection in genetically predisposed individuals.