Mediterranean Journal of Rheumatology最新文献

We report the case of a 67-year-old male with a two-year history of inflammatory polyarthritis, fatigue, and low back pain. He also had a history of biopsy proven neutrophilic dermatosis in the past. On admission and examination, he had pallor. Laboratory evaluation showed macrocytic anaemia, elevated erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). MRI of Sacroiliac joints showed presence of bilateral sacroiliitis. Bone marrow examination showed the presence of cytoplasmic vacuolisation in myeloid and erythroid precursor cells. Genetic analysis confirmed a diagnosis of VEXAS syndrome. He improved with prednisolone and Sulfasalazine with no further relapse on follow up. This case report highlights the importance of considering VEXAS syndrome in older adults with presentation of spondyloarthritis and macrocytic anaemia. Early diagnosis and treatment with corticosteroids and steroid-sparing agents can lead to significant improvement in symptoms and are important for a good outcome.

In the context of holistic therapeutic practices, the cardiovascular risk of patients with rheumatoid arthritis (RA) needs to be addressed as a major factor of compromised disease prognosis and increased mortality. The elevated prevalence of cardiovascular disease (CVD) by more than twofold in RA has been attributed, inter alia, to chronic inflammation exacerbating arterial stiffness, increased onset of hypertension, dyslipidaemia and diabetes mellitus, sedentary lifestyle, and antirheumatic drug complications. CVD risk in RA can be currently assessed by practitioners through accessible adapted calculators, but it remains problematic as their diagnostic accuracy is not superior to calculators designed for the general population. Implementation of guideline-oriented personalised interventions remains the cornerstone for cardiovascular risk management in RA. Remarkably, there is lack of a consortium that brings together different health care providers engaged in the care of patients with RA (e.g., rheumatologists, cardiologists, general practitioners, etc), to guide cardiovascular risk assessment and management. This narrative review aims at providing an overview of current CVD risk assessment and management options, highlighting their pivotal role in the comprehensive treatment of RA patients.

Limited studies summarise the immunomodulatory effects of SGLT2 inhibitors and Metformin in managing rheumatic diseases. The present narrative review aims to fill this knowledge gap by gathering information based on existing clinical evidence. A narrative review was conducted in November 2023 to identify studies investigating the impact of SGLT2 inhibitors and Metformin on rheumatic diseases. A literature search was performed across primary databases, including Medline/PubMed, Scopus, and Web of Science. Supplementary sources like Google Scholar, PubMed Central, Cochrane Library, and ScienceDirect were also consulted. Studies were screened for relevance, and those lacking pertinent outcome data were excluded. The review corroborates the multifaceted potential of Metformin as an adjunctive therapy in autoimmune rheumatologic conditions, offering avenues for further exploration and clinical application to enhance patient outcomes. However, limited literature exists on the clinical effects of SGLT2 inhibitors in rheumatic diseases.

Objective/aim: Systemic Lupus Erythematosus (SLE), is common in women of childbearing age and is associated with obstetric complications. The aim of this study was to evaluate the course of pregnancy and its results in SLE patients with a history of pregnancy.

Methods: Pregnant patients with SLE who applied to the Rheumatology outpatient clinic of between 2010 and 2020 were retrospectively screened.

Results: Fifty-five pregnancies of 31 SLE patients were included in the study. Spontaneous abortion was observed in 27.3% (n:15) and foetal loss in 18.2% (n:10). Neonatal loss or maternal death was not observed in any of the patients. The rate of patients with renal involvement was 34.5% (n:19), and the rate of exacerbation was higher in pregnant women with kidney involvement (26% vs 0% (p:0.006)). Antiphospholipid antibody syndrome (APS) was present in 32.7% (n:18) of the cases, and there was a history of foetal loss before diagnosis in 50% (n:9) of the cases with APS. The foetal loss was seen only in pregnant women with APS (55.6% (n:10) vs. 0% (n:0) (p<0.001)). Six (10.9%) of 55 pregnancies in our study were unplanned pregnancies. Of these, five resulted in abortion. Exacerbation was observed in 3 of the unplanned pregnancies.

Conclusion: Pregnancy complications were significantly lower in patients who were in remission since contraception. Exacerbations were more common in unplanned pregnancies and in patients with renal involvement. The presence of APS was associated with increased pregnancy morbidity.

Introduction: The 2018 published World Health Organisation (WHO) Europe physical activity factsheet reports, specify agreed targets for physical activity and articulate the need to improve the education of medical doctors and healthcare practitioners in order to increase physical activity and reduce sedentary time in people at risk and/or living with Noncommunicable Diseases (NCDs). Given the dearth of relevant initiatives and the continuous need to increase physical activity participation towards better health management of NCDs, the aim of this study is to embed physical activity in the undergraduate curricula of future frontline healthcare professionals (medical doctors and allied health professions) in European countries.

Methods: The Virtual Advice, Nurturing, Guidance on Universal Action, Research and Development for physical activity and sport engagement (VANGUARD) project consists of a collaborative partnership Consortium between six European Universities, WHO Europe and Ministry representatives that has been developed to implement physical activity in the curricula of medical schools and healthcare professions. The methodology of the VANGUARD project is informed by the WHO implementation guidance and the Reach, Effectiveness, Adoption, Implementation and Maintenance (RE-AIM) framework.

Discussion: Through a carefully planned implementation process and via using established appropriate implementation evaluation tools, the end result of the VANGUARD project will be the a) implementation of a physical activity module in six different European Universities (five medical schools and one physiotherapy department) and b) development of a toolkit/guide, in order to assist other healthcare systems and European Universities to develop relevant grass-root innovations for addressing the decline in physical activity levels.

Sulfasalazine (SSZ) remains a valuable treatment option for Rheumatoid Arthritis (RA), especially in women of childbearing age, and is considered safe in pregnancy and lactation. However, the adverse effects in the form of allergic reactions, rashes, feverishness, and gastrointestinal symptoms are not uncommon and usually resolve on discontinuation of the drug. Despite the potential adverse effects, the occurrences are infrequently reported. Agranulocytosis (ANC < 500 cell/cumm) is a rare complication of SSZ that may be potentially life-threatening. We report two cases of SSZ-induced agranulocytosis after 6 weeks of initiation of treatment for RA despite normal leucocyte counts in the initial phase of treatment. There was complete recovery of the counts following discontinuation, along with the institution of colony-stimulating factors and antibiotics for febrile neutropenia. Notably, Granulocyte Colony-Stimulating Factor (G-CSF) did not produce any adverse effects, and the patients were discharged after their ANC levels returned to normal. It is, therefore, essential to regularly monitor blood counts following the initial treatment.

Neuropsychiatric systemic lupus erythematosus (NPSLE) presents a significant diagnostic and therapeutic challenge due to its varied clinical manifestations. The prevalence of NPSLE ranges widely, reported between 37% and 95% in different case series, reflecting this condition's complex and heterogeneous nature. Here we report three cases of juvenile systemic lupus erythematosus (SLE) presenting with catatonia as a rare neuropsychiatric manifestation. Case 1 is a 15-year-old male with fever, and pancytopenia, diagnosed with SLE and subsequent development of catatonia. Case 2 is a 14-year-old female with a history of SLE presenting with altered sensorium, restlessness, and catatonia. Case 3 is a 15-year-old male with SLE exhibiting abnormal behaviour and catatonia. Treatment strategies for these cases include high-dose steroids, immunosuppression, and benzodiazepines. This case series emphasises the importance of a multidisciplinary approach, prompt diagnosis, aggressive treatment, and vigilant follow-up to optimise outcomes in these vulnerable paediatric patients. In conclusion, this case series contributes to the literature on catatonia in paediatric SLE, emphasising the need for expanded awareness, early recognition, and comprehensive management strategies. Further research is warranted to refine predictive factors and establish optimal maintenance protocols for this complex neuropsychiatric manifestation.

Multicentric Osteolysis Nodulosis and Arthropathy (MONA) is a rare skeletal disorder driven by mutations in the MMP2 gene, leading to bone and joint degradation. This case series presents three unique MONA cases, highlighting clinical, radiological, and genetic aspects. These insights shed light on the complexities of MONA, aiding early diagnosis and multidisciplinary management. Case 1 is a 13-year-old male, born to consanguineous parents, presented with a 5-year history of progressive joint deformities, pain, and difficulty walking. Initially diagnosed as juvenile idiopathic arthritis (JIA), despite treatment, his symptoms persisted. Examination revealed multiple clinical findings, including joint contractures and nodules. Genetic analysis identified a pathogenic variant in the MMP2 gene, confirming MONA. Case 2 and Case 3 were two siblings, aged 12 and 17 years respectively, who presented progressive joint contractures in their hands and feet since early childhood. Clinical examinations revealed contractures and subcutaneous nodules. Genetic analysis confirmed MONA with a shared homozygous pathogenic MMP2 variant, emphasising the genetic basis of this rare disorder.

Approval of anifrolumab for the treatment of moderate-to-severe systemic lupus erythematosus (SLE) in 2021 marked the success of a long quest to target the interferon system, in a disease wherein the latter has long been considered to play a pivotal role. Prior to anifrolumab, a number of agents had been tested in early phase clinical trials in patients with SLE, with equivocal results. Following its approval and marketing in several countries, the first reports regarding efficacy and safety in real-life clinical settings have been published, which suggest remarkable efficacy in skin manifestations of the disease, even after prior failure to multiple immunosuppressive therapies. In this report, we provide a short overview of IFN inhibitors that have been used in clinical trials of SLE, with a focus on anifrolumab; we also review all available evidence to date regarding its real-world efficacy and safety.