Clinical Presentations
A patient comes into clinic with abrupt involuntary movements along with some muscular weakness and emotional disturbances with history of Group A strep infection. The uncoordinated movements is also known as...
What is Sydenham Chorea?
Typically a late manifestation (1-7 months after infection) of acute rheumatic fever in 30% of patients.
Inability to sustain contraction (milkmaid grip), and flexion of the wrist and extension of the digits when the arms are extended (choreic hand)
What two titers will be elevated for confirmation of GAS infection?
Anti-streptolysin O
Anti-streptococcal DNAse B
Rheumatic fever is the most frequent cause of what valvular disorder?
What is Mitral Stenosis?
This occurs later in the course after mitral regurgitation.
What hypersensitivity type is Rheumatic Fever/Rheumatic Heart Disease?
Type II
Molecular Mimicry with M Protein aka antibody-cross reactivity
Learning Objectives:
By the end of this game, we should be able to
1) Recognize the clinical presentation of a patient presenting with rheumatic fever
2) Recognize the lab values and findings associated with the diagnosis of rheumatic fever
3) Understand the progression of acute rheumatic fever to rheumatic heart disease
4) Answer a few Amboss questions pertaining to rheumatic fever
Literature/Resources:
UpToDate - Acute rheumatic fever: Clinical manifestations and diagnosis
Arvind, B., & Ramakrishnan, S. (2020). Rheumatic Fever and Rheumatic Heart Disease in Children. Indian journal of pediatrics, 87(4), 305–311. https://doi.org/10.1007/s12098-019-03128-7
Lahiri, S., & Sanyahumbi, A. (2021). Acute Rheumatic Fever. Pediatrics in review, 42(5), 221–232. https://doi.org/10.1542/pir.2019-0288
Majmundar VD, Nagalli S. Erythema Marginatum. [Updated 2022 Jul 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK557835/
Karacan, M., Ceviz, N., & Olgun, H. (2012). Heart rate variability in children with acute rheumatic fever. Cardiology in the young, 22(3), 285–292. https://doi.org/10.1017/S1047951111001429
Amboss - Practice questions
A common type of joint pain someone with acute rheumatic fever may have.
What is Migratory Polyarthritis?
What are the two types of histological findings you would find in acute rheumatic fever?
Aschoff Bodies and Anitschkow cell
Aschoff bodies are associated with myocarditis | granulomas with giant cells
Anitschknow cells --> enlarged macrophages with ovoid, wavy, rod-like nucleus
The most important prognostic factor is.....
Cardiac Involvement
Early death is typically due to myocarditis rather than valvular defects
After a Group A Streptococcus infection, how does the infection progress to acute rheumatic fever (why doesn't all GAS infections causes ARF in everyone that gets the infection)?
Infection is left untreated
Goal is eradication so prompt treatment with penicillin at diagnosis/suspected diagnosis is essential, may need a different bacteriocidal antibiotic if patient has penicillin allergy.
How does this related to our patient?
Our patient shared all the symptoms and lab findings as someone who has acute rheumatic fever and rheumatic heart disease.
What are the two skin manifestation of rheumatic fever?
Subcutaneous Nodules - occurs 6-8 weeks after acute event, typically firm and nontender associated with carditis (in primary resources, only occurs <10% of the time but "harbinger" for involvement of cardiac valves)
Erythemia marginatum --> rash is evanescent, pink, and nonpruritic with a white center | reactive inflammatory reaction to rheumatic fever
What two lab finding may be elevated in acute rheumatic fever?
CRP and ESR
CRP + ESR --> acute systemic inflammation (they are acute phase markers in clinical practice)
Although, they are considered non-specific tests.
The first line drug therapy is Penicillin V for GAS, what drug should we use if the patient is allergic to penicillin?
Cephalosporin or Macrolides (at high doses can be bactericidal)
Goal is eradication of GAS -> bactericidal drugs
Fun fact: There has been no confirmed reports of GAS resistance to penicillin
What causes the progression from acute rheumatic fever to rheumatic heart disease?
After initial rheumatic fever, there is possible post-inflammatory scarring and calcification leading to development of valvular heart defects + Recurring episode --> Rheumatic Heart Disease
What cardiac abnormality is typically involved in the early lesions of acute rheumatic fever?
Mitral valve is most commonly involved --> Mitral regurgitation
What EKG findings are typically seen in acute rheumatic fever?
Prolonged PR intervals
Our results indicated that in the acute period of rheumatic fever, sympathetic dominance is apparent; in patients with prolonged PR interval, sympathetic dominance is relatively lower when compared with the patients with normal PR interval.
List 3 symptoms you would expect someone with rheumatic heart disease to come into clinic with complaints of:
Fatigue/lethargy/Malaise, Increased work of breathing, Joint Pain, Chest Pain, Swelling of ankle/knee
Recurrent episodes of fever, SOB, fatigue
Sore throat several weeks ago
Abnormal movements of limbs
Irritable
JVD and b/l ankle edema --> carditis
What is Rheumatic heart disease two clinical entities?
A 27-year-old woman comes to the physician because of a 3-day history of a sore throat and fever. Her temperature is 38.5°C (101.3°F). Examination shows edematous oropharyngeal mucosa and enlarged tonsils with purulent exudate. There is tender cervical lymphadenopathy. If left untreated, which of the following conditions is most likely to occur in this patient?
A. Toxic Shock Syndrome
B. Polymyalgia rheumatica
C. Rheumatoid arthritis
D. Dilated Cardiomyopathy
E. Erythema Multiforme
D. Dilated Cardiomyopathy
Can results from myocarditis or the mitral regurgitation --> more fluid into the LA --> volume overload --> dilated cardiomyopathy
A 14-year-old girl is brought to the physician because of a 1-week history of malaise and chest pain. Three weeks ago, she had a sore throat that resolved without treatment. Her temperature is 38.7°C (101.7°F). Examination shows several subcutaneous nodules on her elbows and wrist bilaterally and a new-onset early systolic murmur best heard at the apex in the left lateral position. An endomysial biopsy is most likely to show which of the following?
A - Coagulative necrosis with neutrophilic infiltrate
B - Fibrinoid necrosis with histiocytic infiltrate
C - Deposits of misfolded protein aggregates
D - Myocardial infiltration with eosinophilic proteins
Fibrinoid necrosis with histiocytic infiltrate
Aka Aschoff bodies --> granulomas that form in the myocardium due to inflammation
Anitschkow cells --> mononuclear cardiac histiocytes
A 12 year old girl is brought to the physician by her mother because of a 2-day history of high fever and swelling of left ankle and knee. She had a sore throat 3 weeks ago. There is no family of serious illness. Her immunizations are up-to-date. She developed an episode of breathlessness and generalized rash when she received dicloxacillin for a skin infection 2 years ago. She appears ill. Her temperature is 102.3F, pulse is 87/min, andBP is 98/62 mmHg. Exam shows swelling and tenderness of left ankle and knee, range of motion is limited. Lungs are clear to auscultation. A grade 3/5 holosystolic murmur is heard best at the apex. Which of the following is the most appropriate pharmacotherapy?
A. Vancomycin
B. Clarithromycin
C. High Dose Glucocoricoids
D. Amoxicillin
E. Doxycycline
F. Methotrexate
G. Ciprofloxacin
Clarithromycin
A 14-year-old boy is brought to the physician because of fever, malaise, and severe right knee joint pain and swelling for 3 days. He has also had episodes of abdominal pain and epistaxis during this period. Five days ago, he had swelling and pain in his left ankle joint which has since resolved. He reports having a sore throat 3 weeks ago while he was camping in the woods, for which he received symptomatic treatment. His immunizations are up-to-date. His temperature is 38.7°C (101.6°F), pulse is 119/min, and blood pressure is 90/60 mm Hg. Examination shows a swollen, tender right knee; range of motion is limited. There are painless 3 to 4-mm nodules over the elbow. Cardiopulmonary examination is normal. His hemoglobin concentration is 12.3 g/dL, leukocyte count is 11,800/mm3, and erythrocyte sedimentation rate is 63 mm/h. Arthrocentesis of the right knee joint yields clear, straw-colored fluid. Analysis of the synovial fluid shows a leukocyte count of 1350/mm3 with 17% neutrophils; no organisms are identified on Gram stain. Which of the following is the most likely diagnosis
A. Septic Arthritis
B. Acute Lymphoblastic leukemia
C. Infective Endocarditis
D. Acute Rheumatic Fever
E. Lyme Disease
F. Kawasaki Disease
G. Juvenile idiopathic arthritis
D. Acute Rheumatic Fever