Differential Diagnosis
Here we discuss differential diagnosis of two cardinal features, Polyarthritis and Rash.
I)Polyarthritis
1) Gonococcal – Therapeutic trial of pencillin may help in diagnosis of gonococcal infection.
2) Viral infections such as rubella and hepatitis B may have polyarthritis.
3) Septic arthritis – Blood cultures may grow organisms.
4) Tuberculosis – It is usually mono-articular.
5) Juvenile rheumatoid arthritis – Here there is small joint involvement which lasts for 6 to 12 weeks and valvular involvement is rare. However, pericarditis alone may be seen.Eventually deformities occur. Rheumatoid factor may be positive.
6) Serum sickness due to drug allergy, e.g., after penicillin injection may present as polyarthritis.
7) Infective endocarditis may mimic ARF as arthritis and carditis are common features.Joint involvement is usually mono-articular affecting large joints. Blood cultures if positive confirm the diagnosis.
8) Henoch Schonlein purpura, inflammatory bowel disease (ulcerative colitis, crohn’s diseases), blood disorders (sickle cell anaemia, haemophilia, leukaemias), seronegative arthritis, Takayasu’s arteritis may mimick ARF.
II)Rash
Diseases such as Lyme disease and SLE which present with rash may be mistaken for ARF.Lyme disease presents with characteristic rash and arthritis (which appears 1 to 2 months after onset). Juvenile SLE is differentiated by typical skin rash, multiple organ involvement and presence of anti-nuclear antibodies.
Diagnosis
Diagnosis of rheumatic fever is made on basis of various symptoms, signs and results of work up in a case of rheumatic fever (Table ). According to the revised Duckett- Jones criterier, the diagnosis is based upon two or more major clinical manifestations; or and major and two or more minor menifestations. In both cases evidence of previous streptococcal infection is required.
Here we discuss differential diagnosis of two cardinal features, Polyarthritis and Rash.
I)Polyarthritis
1) Gonococcal – Therapeutic trial of pencillin may help in diagnosis of gonococcal infection.
2) Viral infections such as rubella and hepatitis B may have polyarthritis.
3) Septic arthritis – Blood cultures may grow organisms.
4) Tuberculosis – It is usually mono-articular.
5) Juvenile rheumatoid arthritis – Here there is small joint involvement which lasts for 6 to 12 weeks and valvular involvement is rare. However, pericarditis alone may be seen.Eventually deformities occur. Rheumatoid factor may be positive.
6) Serum sickness due to drug allergy, e.g., after penicillin injection may present as polyarthritis.
7) Infective endocarditis may mimic ARF as arthritis and carditis are common features.Joint involvement is usually mono-articular affecting large joints. Blood cultures if positive confirm the diagnosis.
8) Henoch Schonlein purpura, inflammatory bowel disease (ulcerative colitis, crohn’s diseases), blood disorders (sickle cell anaemia, haemophilia, leukaemias), seronegative arthritis, Takayasu’s arteritis may mimick ARF.
II)Rash
Diseases such as Lyme disease and SLE which present with rash may be mistaken for ARF.Lyme disease presents with characteristic rash and arthritis (which appears 1 to 2 months after onset). Juvenile SLE is differentiated by typical skin rash, multiple organ involvement and presence of anti-nuclear antibodies.
Diagnosis
Diagnosis of rheumatic fever is made on basis of various symptoms, signs and results of work up in a case of rheumatic fever (Table ). According to the revised Duckett- Jones criterier, the diagnosis is based upon two or more major clinical manifestations; or and major and two or more minor menifestations. In both cases evidence of previous streptococcal infection is required.
Work-up in a Case of Acute Rheumatic Fever |
Course and Prognosis
The course and ultimate prognosis of ARF is usually directly related to the severity of carditis.The course and prognosis also depends upon recurrence of rheumatic fever. In pre-penicillin era,recurrences of ARF were seen in upto 70 per cent of patients. There is always a tendency to develop rheumatic fever with repeated GAS infections. There is greater chance for recurrence in young children and in the first 3 years after the first attack and in patients with established rheumatic heart disease. If patient had carditis in first attack of ARF, there is always a tendency to have carditis in subsequent attacks. With each recurrence, there is progressive deterioration in valvular lesions and myocardial function. However, if patient is managed well after first attack of ARF and proper prophylaxis is carried out, the recurrent attacks can be prevented. In the UK-USA collaborative study, only 6 per cent of patients with no carditis during first attack of ARF were having murmur after 10 years of first attack. But in patients who developed carditis during first attack of ARF as evidenced by apical systolic murmur, basal diastolic murmurs, CCF,pericarditis, heart disease was present in 30 to 68 per cent at follow up.Patient is advised bed rest preferably in the hospital. Patient must take bed rest till fever, leucocytosis, ESR, CRP are settled (see Table for guidelines of bedrest). If patient develops heart failure due to acute carditis, he should be given digitalis and diuretics with low salt diet. GAS infection should be treated even if throat culture is negative by either single IM injection of benzathine penicillin (1.2 mega units) or oral penicillin for 10 days. If patient is allergic to penicillin, Macrolides or cephalosproins should be given for 10 days. For polyarthritis, high doses of salicylates are used. Aspirin in the dose of 100 mgm/kg/day to maintain a serum level of 20 mg per cent are required. Gradually the dose should be tapered as clinical and laboratory features of inflammation (ESR, CRP) subside.
The course and ultimate prognosis of ARF is usually directly related to the severity of carditis.The course and prognosis also depends upon recurrence of rheumatic fever. In pre-penicillin era,recurrences of ARF were seen in upto 70 per cent of patients. There is always a tendency to develop rheumatic fever with repeated GAS infections. There is greater chance for recurrence in young children and in the first 3 years after the first attack and in patients with established rheumatic heart disease. If patient had carditis in first attack of ARF, there is always a tendency to have carditis in subsequent attacks. With each recurrence, there is progressive deterioration in valvular lesions and myocardial function. However, if patient is managed well after first attack of ARF and proper prophylaxis is carried out, the recurrent attacks can be prevented. In the UK-USA collaborative study, only 6 per cent of patients with no carditis during first attack of ARF were having murmur after 10 years of first attack. But in patients who developed carditis during first attack of ARF as evidenced by apical systolic murmur, basal diastolic murmurs, CCF,pericarditis, heart disease was present in 30 to 68 per cent at follow up.Patient is advised bed rest preferably in the hospital. Patient must take bed rest till fever, leucocytosis, ESR, CRP are settled (see Table for guidelines of bedrest). If patient develops heart failure due to acute carditis, he should be given digitalis and diuretics with low salt diet. GAS infection should be treated even if throat culture is negative by either single IM injection of benzathine penicillin (1.2 mega units) or oral penicillin for 10 days. If patient is allergic to penicillin, Macrolides or cephalosproins should be given for 10 days. For polyarthritis, high doses of salicylates are used. Aspirin in the dose of 100 mgm/kg/day to maintain a serum level of 20 mg per cent are required. Gradually the dose should be tapered as clinical and laboratory features of inflammation (ESR, CRP) subside.
Guidelines for Bed Rest |
For carditis, also salicylates are beneficial but if there is severe carditis, one may consider corticosteroids. Prednisolone in a dose of 1-2 mgm/kg/day is given. When corticosteroids are tapered, salicylates should be given and then continued for 2 to 4 weeks to prevent rheumatic rebound. Chorea is managed by diazepam or haloperidol.
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