Acute Rheumatic Fever PDF

Introduction Acute rheumatic fever (ARF) is an autoimmune inflammatory process that develops as a sequela of streptococcal infection. ARF has Acu...

Introduction Acute rheumatic fever (ARF) is an autoimmune inflammatory process that develops as a sequela of streptococcal infection. ARF has Acute rheumatic fever extremely variable manifestations, and remains a clinical syndrome for which no specific diagnostic test exists. Persons who have experienced By Israa an episode of ARF are predisposed to recurrence following subsequent group A streptococcal infections. The most significant complication of ARF is rheumatic heart disease, which usually occurs after repeated bouts of acute illness. Pathophysiology ARF is characterized by nonsuppurative inflammatory lesions of the Molecular mimicry accounts for the tissue injury that occurs in joints, heart, subcutaneous tissue, and central nervous system. An rheumatic fever. Both the humoral and cellular host defenses of a genetically vulnerable host are involved. In this process, the patient's extensive literature search has shown that, at least in developed immune responses (both B- and T-cell mediated) are unable to countries, rheumatic fever follows pharyngeal infection with distinguish between the invading microbe and certain host tissues. rheumatogenic group A streptococci. Clinical manifestation Diagnosis The diagnosis of RF is based on JONES criteria 1. Subcutaneous nodules, Erythema marginatum (keratin) We need two major criteria or one major and two minor criteria in 2. Sydenham’s chorea (Ganglioside protein) order to diagnose RF 3. Pancarditis (laminin, tropomyosin, myosin, actin and troponin) 4. Migratory poly Arthritis (vimentin) Jones Criteria, 2015 revision, low-risk Jones Criteria, 2015 revision, low-risk populations (United States, Europe, other high- populations (United States, Europe, other high- income areas) income areas) Major criteria are as follows: Minor criteria are as follows:  Carditis (clinical or echocardiographic diagnosis)  Polyarthralgia (cannot count arthritis as a major criterion and  Polyarthritis (not monoarthritis) arthralgia as a minor criterion)  Chorea (rare in adults)  Fever exceeding 38.5°C  Erythema marginatum (uncommon; rare in adults)  Elevated ESR (>60 mm/hr) or CRP level (>3 mg/L)  Subcutaneous nodules (uncommon; rare in adults)  Prolonged PR interval Jones criteria, 2015 revision, high-risk Jones criteria, 2015 revision, high-risk populations (Oceania, Africa, South Asia, other populations (Oceania, Africa, South Asia, other lower-income areas) lower-income areas) Major criteria are as follows: Minor criteria are as follows:  Carditis (clinical or echocardiographic diagnosis)  Polyarthralgia (cannot count arthritis as a major criterion and  Polyarthritis or monoarthritis: Polyarthralgias can also be considered arthralgia as a minor criterion) but only after careful consideration of the differential diagnoses.  Fever exceeding 38°C (note lower cutoff)  Chorea (rare in adults)  Elevated ESR (>30 mm/hr; note lower ESR standard) or CRP level (>3  Erythema marginatum (uncommon; rare in adults) mg/L)  Subcutaneous nodules (uncommon; rare in adults)  Prolonged PR interval Universal criteria Scoring In both higher- and lower-risk settings, evidence of group A If supported by evidence of preceding group A streptococcal streptococcal disease is required for diagnosis, except when rheumatic infection, the presence of two major manifestations or one major and fever is first discovered after a long latent period (eg, Sydenham two minor manifestations indicates a high probability of ARF. Failure to chorea, indolent carditis), as follows: fulfill the Jones criteria makes the diagnosis unlikely but not impossible.  Evidence of preceding group A streptococcal infection - Positive Clinical judgment is required. throat culture or rapid antigen test result Recurrent ARF can be diagnosed based on 2 major, 1 major plus 2  Elevated or rising streptococcal antibody titer minor, or 3 minor criteria. Medical Care 1-Treatment of the group A streptococci Management and prevention of acute rheumatic fever (ARF) can be Treatment of the group A streptococcal infection that led to the divided into the following 4 approaches. disease Although never proven to improve the one-year outcome, this is a standard practice. It may at least serve to reduce the spread of causative strain 2-General treatment of the acute episode Anti-inflammatory agents are used to control the arthritis, fever, and Bed rest is a traditional part of ARF therapy and is especially other acute symptoms. Salicylates are the preferred agents, although important in those with carditis. Patients are typically advised to rest other nonsteroidal agents are probably equally efficacious and maybe through the acute illness and to then gradually increase activity; some preferred in children. Steroids are also effective but should probably be clinicians monitor the patient’s ESR and restart activity only as it reserved for patients in whom salicylates fail, since there is a risk of normalizes. rebound when they are withdrawn. Intravenous immunoglobulin has not been shown to reduce the risk None of these anti-inflammatory agents has been shown to reduce of rheumatic heart disease or to substantially improve the clinical the risk of subsequent rheumatic heart disease. course.  3-Cardiac management Chorea is usually managed conservatively in a quiet nonstimulatory Bedrest is essential in patients with cardiac involvement. Carditis environment; valproic acid is the preferred agent if sedation is needed. resulting in heart failure is treated with conventional measures; some Intravenous immunoglobulin, steroids, and plasmapheresis have all use corticosteroids for severe carditis, although data to support this are been used successfully in refractory chorea, although conclusive scant. Diuretics and vasodilators are the mainstays of therapy. Monitor evidence of their efficacy is limited. for development of arrhythmias in patients with active myocarditis. Some promising work suggests a possible role for hydroxychloroquine Atrial fibrillation requires aggressive management to reduce the risk of in the treatment of ARF, although no clinical data are yet available to stroke. recommend its use. 4-Prophylaxis Primary prophylaxis (treatment of streptococcal pharyngitis) secondary prevention is recommended to prevent additional dramatically reduces the risk of ARF and should be provided whenever streptococcal infections and is believed by most experts to be a critical a group A streptococcal pharyngitis is confirmed. Treatment of step in management of ARF. Patients with a history of rheumatic fever pharyngitis without proof of group A streptococcal etiology may be are at a high risk of recurrent ARF, which may further the cardiac reasonable in areas of high endemicity. damage. The exact duration of chronic antimicrobial prophylaxis remains controversial, but the WHO guidelines are commonly used. There had been concern that sustained benzathine penicillin as secondary prophylaxis would lead to the development of resistant strains of Streptococcus viridans, but a 2008 study found no support for this hypothesis.  Surgical care Rheumatic fever with carditis and clinically significant residual heart disease requires Surgical care is not typically indicated in ARF. Surgical intervention is antibiotic treatment for a minimum of 10 years after the latest episode; prophylaxis is required until the patient is aged at least 40-45 years and is sometimes continued for life. often required to treat long-term valvular cardiac sequelae of ARF Rheumatic fever with carditis and no residual heart disease aside from mild mitral including aortic and mitral regurgitation as well as mitral stenosis. regurgitation requires antibiotic treatment for 10 years or until age 25 years (whichever is longer). Rheumatic fever without carditis requires antibiotic treatment for 5 years or until the patient is aged 18-21 years (whichever is longer). For secondary prophylaxis penicillin G benzathine at a dose of 1.2 million U IM q4wk is used. Long-term administration of oral penicillin may be used in lieu of the intramuscular route. Erythromycin or sulfadiazine may be used in patients who are allergic to penicillin. Good luck