questions 3

Rheumatic Heart Disease

Topic updated on 03/07/17 4:11pm

Snap Shot
  • A 13-year-old-boy presents with 3 days of malaise, painful joints, nodular swelling over his elbows, low grade fever, and a rash on his chest and left shoulder. Two weeks ago he was seen for a sore throat.
  • A sequela of pharyngeal streptococcal A infection
  • A type II hypersensitivity autoimmune disease
    • caused by cross-reactions between M protein of strep A and antigens on joint and heart tissue (epitope homology / molecular mimicry) 
  • Rheumatic fever is a systemic immune process that may or may not lead to RHD
  • RHD is a valvular abnormality secondary to rheumatic fever
    • most often mitral stenosis due to sterile vegetations
  • Rheumatic fever presents approximately two weeks post-infection with
    • migratory polyarthritis
      • no permanent damage
      • occurs at both large and small joints
    • carditis
      • endocarditis
        • more commonly affects high pressure valves first
          • sterile vegetations at closure line of the valve
        • early in disease = mitral valve prolapse
        • late in disease = mitral valve stenosis
      • myocarditis
        • contain Aschoff bodies  
          • fibrinoid necrosis surrounded by reactive histiocytes (Anitschow cells)
          • pathognomonic for acute rheumatic heart disease
      • pericarditis
        • friction rub
    • erythema marginatum
    • subcutaneous nodules on extensor surfaces
    • Sydenham's chorea
      • involuntary movements of all muscles (late in disease course)
    • note: all of these symptoms are major Jones Criterion for RF diagnosis
  • Labs
    • Positive ASO
    • positive throat culture
    • elevated ESR and CRP
    • leukocytosis increased PR interval
  • Penicillin for acute rheumatic fever


Qbank (3 Questions)

(M1.CV.75) A 38-year-old male presents to his primary care physician complaining of increasing shortness of breath over the past 2 months. He reports experiencing an extended illness of several weeks as a child that required him to miss school. He is unsure but believes it involved a sore throat followed by a fever and joint pains. He does not recall seeing a physician or receiving treatment for this. Today, on physical examination, cardiac auscultation reveals an opening snap after the second heart sound followed by a diastolic, decrescendo murmur. A follow-up echocardiogram is conducted. Which of the following best explains the pathophysiology of this patient's condition? Topic Review Topic

1. Annular calcification
2. Epitope homology
3. Myocardial ischemia
4. Atherosclerosis
5. Congenital heart defect

(M1.CV.112) A 10-year-old male presents with his mother with multiple complaints. A few weeks ago, he had a sore throat for several days that improved without specific therapy. Additionally, over the past several days he has experienced pain in his ankles and wrists and, more recently, his left knee. His mother also noted several bumps on both of his elbows, and he has also had some pain in his center of his chest. He thinks the pain is better when he leans forward. On physical examination, he is noted to be mildly febrile, and a pericardial friction rub is auscultated. Which of the following histopathologic findings is most likely associated with this patient's condition? Topic Review Topic

1. Atypical lymphocytes noted on peripheral blood smear with an initial positive heterophil antibody test
2. Plasmodium falciparum ring forms in red blood cells on peripheral blood smear
3. Needle-shaped, negatively birefringent crystal deposits surrounded by palisading histiocytes in the synovial fluid of an affected joint
4. Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin
5. Sterile vegetations on both the ventricular and atrial aspects of the mitral valve, a proliferative glomerulonephritis, and serum anti-dsDNA and anti-Sm positivity

(M1.CV.223) A 9-year-old male visited his primary care physician for a check-up three months after a throat infection. Upon examination, the patient exhibits painless subcutaneous nodules on the back of the wrist, the outside elbow, and the front of the knees, as well as inflammation in the joints of the lower extremities. Which of the following symptoms is most likely to also be present in this patient? Topic Review Topic

1. Renal failure
2. Hepatomegaly
3. Chorea
4. Pleural effusion
5. Butterfly rash


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