Rambam Maimonides Medical Journal最新文献

The coronavirus 2019 (COVID) pandemic has highlighted the ways in which municipal, state, and Federal agencies in the USA have failed to address the inequities of present-day health systems. As alternative organizing centers outside these agencies, local communities are potentially situated to redress the inequities of present-day health systems in a collaborative manner that demonstrates solidarity by supplementing a purely scientific model of medicine and healthcare. In the mid-twentieth century, the Black Panthers, a revolutionary African American nationalist organization that focused on socialism and self-defense, introduced highly influential free clinics, which sought to bring expertise to the Black community on their own terms. This required bringing the benefits of biomedicine to those who customarily had not seen them. By extension, their approach raises questions regarding community- and expertise-centered approaches for the Jewish community: how is it engaged in healthcare for itself (in its diverse subcategories) and for others? Moreover, understanding how the Jewish community has been ill-served by present-day health-care systems might spur Jewish institutions to reimagine how healthcare should work.

Giant cell arteritis (GCA) is the most prevalent subtype of vasculitis in adults. In recent years, there has been substantial improvement in the diagnosis and treatment of GCA, mainly attributed to the introduction of highly sensitive diagnostic tools, incorporation of modern imaging modalities for diagnosis and monitoring of large-vessel vasculitis, and introduction of highly effective novel biological therapies that have revolutionized the field of GCA. This article reviews state-of-the-art approaches for the diagnosis, monitoring, and treatment options of GCA.

Glucocorticosteroid-induced osteoporosis (GIO) is the most common cause of secondary osteoporosis but is underdiagnosed and undertreated. Our aim in this communication is to review the literature on the implementation of current GIO prevention practices such as calcium and vitamin D supplementation with emphasis on the rheumatologists' perspective relating to the need for development of novel GIO educational prevention measures.

Idiopathic inflammatory myopathies (IIM) are a rare group of disorders that feature progressive immune-mediated skeletal muscle destruction along with skin, lung, and joint involvement. Management of IIMs necessitates glucocorticoid therapy followed by conventional steroid-sparing agents to control disease activity. In the settings of refractory myositis or life-threatening manifestations, e.g. lung involvement or oropharyngeal dysphagia, second-line therapies are needed to minimize disease burden, avoid end-organ damage and steroid toxicity, and decrease mortality. These therapies may include biological disease-modifying antirheumatic drugs (bDMARDs), and to a lesser extent, targeted synthetic disease-modifying antirheumatic drugs (TSD). This article reviews the current use of bDMARDs, e.g. intravenous immunoglobulin and rituximab, and a TSD-Janus kinase inhibitors (JAKI)-along with their indications, efficacy, and safety in managing IIM.

Increasing evidence points towards mitochondria as crucial players in the initiation and progression of auto-immune and degenerative disorders, to which impaired cell metabolism is but a facet of the subjacent etiopathogenesis. This review aims to introduce the reader to essential concepts of mitochondrial abnormalities in idiopathic inflammatory myopathy (IIM), underscoring inclusion-body myositis and dermatomyositis. Far surpassing the initial simplistic view of being responsible for energy generation, mitochondria have gathered attention regarding their role in inflammatory processes, being able to fuel autoimmunity, as shown by the presence of anti-mitochondrial antibodies (AMAs) in up to 10% of IIM patients. As cellular respiration takes place, mitochondrial metabolites might help to shape the pro-inflammatory milieu in affected muscle, beyond generating reactive oxygen species, which are well-recognized inducers of damage-associated molecular patterns. A series of mitochondrial components might facilitate the sterile activation of pro-inflammatory cells and the production of several cytokines responsible for enhancing auto-immune responses. Marked variation in the mitochondrial genome has also been reported in IIM patients. As such, we summarize key historical and recent advances linking aberrations and instabilities of mitochondrial DNA to impaired muscle function. Besides discussing mitochondrial dysfunction as an essential part of IIM development, we also highlight possible associations between presence of AMAs and a particular phenotype of IIM, with its own characteristic clinical and radiological pattern. Finally, we present promising treatment approaches targeting mitochondria, while briefly discussing experimental models for gaining deeper insight into the disease process, and ultimately leading to novel drug development.

Treatment with biological agents has become standard of care in treatment of immune-mediated diseases (IMD), including rheumatoid arthritis and psoriatic arthritis. Yet, a significant proportion of patients experience loss of response to biologics, need treatment escalation, or develop side effects. During the past decade, new biologic agents with different targeted molecular pathways have been approved for treatment of IMD, introducing the possibility of concomitant dual biologic therapy. The role of dual biologic therapy targeting different inflammatory pathways has become an area of great interest in the field of IMD, addressing the unmet clinical need of patients with refractory diseases and treatment of comorbidities, such as osteoporosis, asthma, atopic dermatitis, and urticaria. Despite the increasing use of biologics as a dual therapy across different indications, there is a paucity of data concerning the safety of the simultaneous use of more than one biological agents. The purpose of this review is to summarize the current literature on the use of dual biologics in patients with rheumatoid arthritis and psoriatic arthritis, addressing the potential adverse effects associated with combination therapy, and highlighting future directions in the use of this novel therapeutic modality.

Objective: Idiopathic eosinophilic vasculitis has been described in previous case series as a possible manifestation of hypereosinophilic syndrome (HES) in asthma-free patients. A rare disease, it can be classified as an eosinophilic-rich, necrotizing, systemic form of vasculitis that affects vessels of various sizes in these patients. This report shares our experience with the treatment of a patient with eosinophilic vasculitis.

Case presentation: We present the case of a 45-year-old man who suffered from idiopathic HES manifesting as digital ulcers and peripheral ischemia of both the upper and lower limbs without the involvement of other systems. Diagnosis was made after excluding the primary and secondary causes of eosinophilia. The patient responded well to both corticosteroids and mepolizumab, an interleukin-5 inhibitor, as a corticosteroid-sparing therapy.

Conclusion: Our case of HES-associated vasculitis in an asthma-free patient supports previous reports describing this rare diagnosis of idiopathic eosinophilic vasculitis in recent years. We describe a good response to mepolizumab (interleukin-5 inhibitor) in our patient.

Objective: In patients with acute hepatic porphyria (AHP), prolonged fasting is a known trigger of AHP attacks. Despite this, some Jewish AHP patients-mainly hereditary coproporphyria (HCP) and variegate porphyria (VP) patients-fast for 25 consecutive hours during the traditional Jewish holy day known as Yom Kippur. In this study, we evaluated the effect of the fast on these patients.

Methods: A retrospective study and survey of AHP patients in Israel was carried out. Patients were asked whether they have fasted and whether any symptoms were induced by this fast. Patients' medical records were reviewed for an emergency department (ED) visit following Yom Kippur between 2007 and 2019. Only 3 acute intermittent porphyria (AIP) patients reported fasting; they were excluded from analysis.

Results: A total of 21 HCP patients and 40 VP patients completed the survey; 30 quiescent patients reported they fast, while 31 did not fast. The majority of fasting patients (96.67%) reported no symptoms following a fast. We found no statistically significant association between ED visits 1 week (0.26% in both fasting and non-fasting patients) or 1 month (2.1% visits in non-fasting versus 0.78% in fasting patients) following Yom Kippur. Of the symptomatic ED visits following a fast, none were defined as severe attacks.

Conclusion: A 25-hour fast in stable HCP and VP patients did not increase the risk of an acute attack and can probably be regarded as safe.