Hypertrophic Cardiomyopathy: Current Treatment and Future Options.

Hypertrophic cardiomyopathy (HCM) is a disease involving the cardiac sarcomere. It is associated with various disease-causing gene mutations and phenotypic expressions, managed with different therapies with variable prognoses. The heterogeneity of the disease is evident in the fact that it burdens patients of all ages. HCM is the most prevalent cause of sudden death in athletes. However, several technological advancements and therapeutic options have reduced mortality in patients with HCM to 0.5% per year. In addition, rapid advances in our knowledge of the molecular defects accountable for HCM have strengthened our awareness of the disorder and recommended new approaches to the assessment of prognosis. Despite all these evolutions, a small subgroup of patients with HCM will experience sudden cardiac death, and risk stratification remains a critical challenge. This review provides a practical guide to the updated recommendations for patients with HCM, including clinical updates for diagnosis, family screening, clinical imaging, risk stratification, and management.

The Role of Belimumab in Systemic Lupus Erythematosis: A Systematic Review.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple systems with a range of clinical presentations caused by the production of antibodies, activation of complements, and deposition of immune complexes. The exact cause of SLE is still unknown. The effectiveness of traditional treatment methods for SLE is very little. Nowadays, resistance to conventional therapy, steroids, and immunosuppressants is common among SLE patients. Patients with refractory disease and nephritis generally have severe drug-induced toxicity which contributes to organ dysfunction, despite available therapies. Different biologic agents and therapeutic antibodies have become an alternative and have been under experiment in clinical trials, enrolling patients whose disease is inadequately controlled by conventional treatment. Belimumab is the only targeted therapy approved for SLE treatment. This systematic review discusses one such biological agent for treating systemic lupus erythematosus, namely, belimumab. The systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies included randomized clinical trials (RCTs) from 2005 to 2021 on adult SLE. patients treated with monoclonal antibodies to assess the efficacy and safety. Methodological quality was assessed using PubMed, PMC, the Cochrane Risk of Bias tool, and the QUality In Prognosis Studies Tool (QUIPS) for RCTs. Two independent reviewers performed an electronic search on MEDLINE, Cochrane Library, SCIELO, Scopus, and ResearchGate. Based on a systematic review of articles we found that belimumab appears to be efficacious and generally well-tolerated in the treatment of SLE as compared to other drugs. The long-term use of belimumab combined with standard therapy showed a low incidence of organ damage. A lower incidence of organ damage was seen after initiating treatment in patients with a high risk for organ dysfunction. Patients who test for antinuclear antibody or anti-dsDNA-positive SLE, with moderate symptoms in the skin and musculoskeletal systems despite immunosuppressants, are treated with belimumab as an adjunct therapy. Patients with severe lupus nephritis or active CNS lupus cannot be treated with belimumab. Belimumab is effective in most races, as a clinical trial done in North-East Asia showed improvement in SLE symptoms and decreased dependence on prednisone. Belimumab also decreased disease activity and severe flares. Belimumab had greater efficacy in children.