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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Rheumatic heart disease (RHD) management can be divided into two main strategies: prevention and long-term management.Primary PreventionPrimary prevention focuses on timely diagnosis and management of group A streptococcal pharyngitis to prevent acute rheumatic fever. The most widely used antibiotic for treating this condition is intramuscular benzathine penicillin G.Acute Rheumatic Fever TreatmentThe primary treatment goal for a patient diagnosed with acute rheumatic fever is to suppress the...
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AssessmentA comprehensive assessment is essential in managing a patient with rheumatic heart disease (RHD). Begin with obtaining a detailed medical history, including recent streptococcal infections, a history of rheumatic fever, or previously diagnosed rheumatic heart disease. Assess the patient for symptoms such as fever, chest pain, widespread joint pain (arthralgia), tachycardia, pericardial friction rub, muffled heart sounds, heart murmurs, peripheral edema, subcutaneous nodules, and...
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Rhupus syndrome: current knowledge and future perspectives.

Luis M Amezcua-Guerra1,2,3, Jessica Roldán-Ortega4, Mauricio Mora-Ramírez2,5

  • 1Department of Immunology, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico.

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Summary

Rhupus syndrome, a mix of rheumatoid arthritis and lupus, presents unique autoantibodies and joint issues. Further research is needed for better classification and management of this complex autoimmune condition.

Keywords:
Autoantibodiesautoimmune diseaseserosive arthritisoverlap syndromerheumatoid arthritisrhupus syndromesystemic lupus erythematosus

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Area of Science:

  • Rheumatology
  • Immunology
  • Autoimmune Diseases

Background:

  • Rhupus syndrome is characterized by overlapping features of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE).
  • Despite being recognized for decades, its natural history, immunopathogenesis, and optimal management require further definition.
  • This review synthesizes current understanding up to 2025.

Purpose of the Study:

  • To outline the historical evolution and current understanding of rhupus syndrome.
  • To emphasize the hybrid phenotype, including dual autoantibody profiles, RA-like arthropathy, and variable SLE involvement.
  • To discuss genetic and immunophenotypic findings.

Main Methods:

  • Narrative review utilizing the PICo framework.
  • Analysis of historical evolution and current understanding of rhupus.
  • Synthesis of genetic studies and immunophenotyping data.

Main Results:

  • Rhupus exhibits dual autoantibody profiles (rheumatoid factor, anti-citrullinated/carbamylated protein antibodies, antinuclear/extractable nuclear antigen antibodies).
  • Patients present with RA-like erosive arthropathy and milder SLE manifestations.
  • Shared autoimmune susceptibility loci (e.g., HLA-DRB1, PTPN22) and immune cell expansions (Th1, CD4+ CD28null T-cells) are implicated.
  • Observational evidence suggests benefits from conventional DMARDs, B-cell depletion, abatacept, and targeted synthetic agents.

Conclusions:

  • Standardized classification criteria and international registries are crucial for rhupus research.
  • Longitudinal multi-omic and imaging studies are needed.
  • A coordinated research agenda will advance rhupus management towards a precision-based approach.