Rheumatic Fever 2023-2024 PDF

Pediatrics/ Rheumatology/ lect. 2 2023-2024 Dr. Sawsan Ali Rheumatic fever Etiology:  It is caused by Group A Streptococcus upper respiratory tract infections.  The annual incidence of acute rheumatic fever in some developing countri...

Pediatrics/ Rheumatology/ lect. 2 2023-2024 Dr. Sawsan Ali Rheumatic fever Etiology:  It is caused by Group A Streptococcus upper respiratory tract infections.  The annual incidence of acute rheumatic fever in some developing countries exceeds 50 per 100,000 children, and very high rates are also seen in ethnic minority populations within Australia and New Zealand.  Worldwide, rheumatic heart disease remains the most common form of acquired heart disease in all age-groups, accounting for up to 50% of all cardiovascular disease and 50% of all cardiac admissions in many developing countries.  As many as two-thirds of patients with an acute episode of rheumatic fever have history of an upper respiratory tract infection  Patients with acute rheumatic fever almost always have serologic evidence of a recent GAS infection. Not all serotypes of GAS can cause rheumatic fever. In addition, certain serotypes of GAS (M types 1, 3, 5, 6, 18, 29) are more frequently isolated from patients with acute rheumatic fever than are other serotypes.  The incidence of both initial attacks and recurrences of acute rheumatic fever peaks in children 5-15 years of age. The onset of acute rheumatic fever (approximately 2-4 week) after GAS pharyngitis. Clinical manifestations and Diagnosis:  Because no clinical or laboratory finding is pathognomonic for acute rheumatic fever, The Jones Criteria, are intended for diagnosis of the initial attack of acute RF and recurrent attacks.  Diagnosis of RF requires {(2 major) OR (1 major + 2 minor) criteria} + evidence of preceding GAS infection.  There are 5 major criteria: 1. Migratory Polyarthritis 2. Carditis 3. Sydenham Chorea 4. Erythema Marginatum 5. Subcutaneous Nodules 1   Minor criteria involve: 1. Arthralgia (only if arthritis is not used as a major criterion) 2. Fever 3. Elevated acute phase reactants (ESR, C- Reactive protein) 4. Prolonged P-R interval on ECG (unless carditis is a major criterion).  Recent Group A Streptococcus Infection:  An absolute requirement for the diagnosis of acute RF.  Positive throat culture, rapid streptococcal antigen test (Streptozyme test), elevated or rising serum antistreptococcal antibody titers (ASOT, anti–DNase B, or anti-hyaluronidase).  The diagnosis of acute RF can be made without strict adherence to the Jones Criteria in 3 circumstances: (1) When chorea occurs as the only major manifestation of acute RF. (2) When indolent carditis is the only manifestation in patients who first come to medical attention only months after the apparent onset of acute RF. (3) In a limited number of patients with recurrence of acute RF in particularly high-risk populations. Migratory Polyarthritis: Arthritis occurs in approximately 75% of patients with acute RF and typically involves larger joints, particularly the knees, ankles, wrists, and elbows. Involvement of the spine, small joints of the hands and feet, or hips is uncommon. Rheumatic joints are classically hot, red, swollen, and exquisitely tender, However, Rheumatic arthritis is almost never deforming. The joint involvement is characteristically migratory in nature; that is, a severely inflamed joint can become normal within 1-3 days without treatment, even as 1 or more other large joints become involved. A dramatic response to even low doses of salicylates is another characteristic feature of the arthritis, and the absence of such a response should suggest an alternative diagnosis. Carditis: Occurs in 50-60% of all cases. Carditis and resultant chronic rheumatic heart disease are the most serious manifestations of acute rheumatic fever and account for essentially all of the associated morbidity and mortality. 2  Rheumatic carditis is characterized by pancarditis, with active inflammation of myocardium, pericardium, and endocardium. Endocarditis (valvulitis) is a universal finding in rheumatic carditis, whereas the presence of pericarditis or myocarditis is variable. Myocarditis and/or pericarditis without clinical evidence of endocarditis almost never is rheumatic carditis; alternate etiologies (especially viral) need to be sought. Isolated mitral valvular disease or combined aortic and mitral valvular disease are common, while isolated aortic or right-sided valvular involvement is uncommon. Carditis usually presents as tachycardia, cardiac murmurs, cardiomegaly, heart failure with hepatomegaly, peripheral and pulmonary edema. Sydenham Chorea: It occurs in 10-15% of patients with acute RF. The latent period from acute GAS infection to chorea can be months. Usually presents as an isolated, movement disorder, motional liability, incoordination, poor school performance, uncontrollable movements, and facial grimacing, all exacerbated by stress and disappearing with sleep. Chorea rarely, if ever, leads to permanent neurologic sequelae. Clinical maneuvers to elicit features of chorea include: 1. Demonstration of milkmaid’s grip. 2. Spooning and pronation of the hands when the patient’s arms are extended above the head (Pronator test). 3. Wormian movements of the tongue upon protrusion (Darting tongue). 4. Examination of handwriting to evaluate fine motor movements. Erythema Marginatum: Is a rare (approximately 1%) Characteristic rash of acute rheumatic fever. It consists of erythematous, serpiginous, macular lesions with pale centers that are not pruritic. It occurs primarily on the trunk and extremities, but not on the face, and it can be accentuated by warming the skin. Subcutaneous Nodules: Rare (≤1% of patients). Consist of firm nodules approximately 1 cm in diameter along the extensor surfaces of tendons near bony prominences. There is a correlation between the presence of these nodules and significant rheumatic heart disease. 3 Differential diagnosis of acute rheumatic fever Treatment  All patients with acute rheumatic fever should be placed on bed rest and monitored closely for evidence of carditis.  Antibiotic Therapy: regardless of the throat culture results, the patient should receive 10 days of orally administered penicillin or amoxicillin or erythromycin or a single IM injection of benzathine penicillin to ensure eradication of GAS from the upper respiratory tract. If penicillin allergic, 10 days of erythromycin, 5 days of azithromycin, or 10 days of clindamycin is indicated. After this initial course of antibiotic therapy, long-term antibiotic prophylaxis for secondary prevention should be instituted (see later).  Anti-inflammatory Therapy:  Patients with polyarthritis, and those with carditis without cardiomegaly or CHF should be treated with oral salicylates.  Patients with carditis with cardiomegaly and/or CHF should receive corticosteroids.  Supportive therapies for patients with moderate to severe carditis include digoxin, fluid and salt restriction, diuretics, and oxygen.  Sydenham Chorea; Sedatives may be helpful early in the course of chorea; such as phenobarbital, haloperidol, chlorpromazine. Some patients may benefit from corticosteroids. 4  Prevention Prevention of both initial and recurrent episodes of acute RF depends on controlling GAS infections of URT. A. Primary prevention: Appropriate antibiotic therapy instituted before the 9th day of symptoms of acute GAS pharyngitis is highly effective in preventing first attacks of acute RF. B. Secondary Prevention: Individuals who have already suffered an attack of acute RF are susceptible to recurrences of RF with any subsequent GAS URTI. Therefore, these patients should receive continuous antibiotic prophylaxis to prevent recurrences. Antibiotic prophylaxis should continue in these patients until the patient reaches 21 yrs. of age or until 5 years since the last RF attack, whichever is longer. (Sometimes lifelong prophylaxis is needed for those with carditis and residual heart disease).  The regimen of choice for secondary prevention is a single IM injection of benzathine penicillin G every 4 wk.  In compliant patients, oral Penicillin V 250 mg twice daily, are equally effective.  For patient who is allergic to both penicillin, a macrolide (erythromycin or clarithromycin or azithromycin) may be used. Duration of chemoprophylaxis: 5  Henoch-Schonlein Purpura (Anaphylactoid Purpura) HSP is a common vasculitis of the small vessels & the most common cause of non-thrombocytopenic purpura in children. HSP occurs worldwide and affects all ethnic groups but is more common in white and Asian populations. The incidence of HSP is estimated at 14-20 per 100,000 children per year and affects males more than females, with a 1.2-1.8: 1 male/female ratio. Etiology: Unknown, but may follow URTI (especially group A streptococcus) & occasionally occurs as clusters in families. Pathology: IgA-mediated vasculitis of small vessels with complement activation Clinical manifestations: Peak age is between 3-10 years. It may be acute or insidious with sequential occurrence of symptoms over a period of weeks or months; these manifestations include: 1. Skin rash: is the hallmark of HSP that occur in all cases (100%). It begins as pinkish maculopapules that initially blanch on pressure, then progress to petechiae or purpura, which is palpable & not blanchable, then it evolves from red to purple to rusty brown before they eventually fade. Occasionally, bullae and ulcerations develop. The skin lesions are usually symmetric and occur in gravity-dependent areas (lower extremities), extensor aspect of the upper extremities or on pressure points (buttocks). 2. GIT involvement occurs in up to 80%; it is due to edema and damage of blood vessels of the GIT → intermittent colicky abdominal pain. GIT bleeding may occur → hematemesis, malena, or occult blood in stool. 3. Arthritis occurs in ≈ 75% & mainly affect the knees and ankles → swelling & effusion. It is usually resolves within 2 wk without residual deformity. 4. Renal involvement occurs in up to 50%. e.g. nephritis, nephrosis, or ARF. 5. Other manifestations: low-grade fever & fatigability; during active disease, HSM & LAP may occur. 6 Diagnosis: Diagnosis is usually clinical, but the definitive test is skin biopsy which shows leukocytoclastic angiitis. Complications: GIT: intussusceptions (usually ileo-ileal), bowel infarction or perforation. Neurological involvement (seizures, paresis, or coma), Others: cardiac and eye involvement, pancreatitis, pulmonary or intramuscular hemorrhage, and testicular torsion. Treatment: Supportive therapy e.g. adequate hydration, nutrition, & analgesia. Corticosteroids are indicated for GIT & other life-threatening manifestations; however, corticosteroids neither alter the overall prognosis nor prevent renal disease. Prognosis: HSP is a self-limited disease lasting ≈ 4 wk with excellent overall prognosis, although death may rarely occur due to GIT, CNS, or renal complications. About 15-60% of children with HSP experience one or more recurrences, typically within 4-6 mo. In each relapse, symptoms are usually milder than those at presentation. Renal involvement at presentation may result in chronic renal disease & chronic hypertension. Therefore, even in children with normal renal function, they should be followed-up for 6 months by urinalysis & blood pressure measurement because persistent renal disease may develop. ***Good Luck*** 7