Rheumatic heart disease

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Rheumatic heart disease


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Rheumatic heart disease

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A 70-year-old man comes to the emergency department for evaluation of dyspnea, dizziness, and associated chest pain. He became more concerned when he was walking up stairs and suddenly “passed out.” His medical history includes type 2 diabetes mellitus, for which he takes metformin. He smoked one pack per day for forty years but does not use excessive alcohol or illicit drugs. His temperature is 37.0°C (98.6°F), pulse is 80/min, and blood pressure is 139/75 mmHg. On physical examination, a systolic murmur is appreciated over the right sternal border, which decreases with sustained hand grip. Which of the following is the most likely underlying cause of this patient's condition? 

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rheumatic fever p. 321

Hypersensitivity reactions p. 110-111

rheumatic fever p. 320

M protein

rheumatic fever and p. 134


for rheumatic fever p. 321


prophylaxis (rheumatic fever) p. 195

Rheumatic fever p. 321

chorea with p. 537

heart murmur with p. 298

Streptococcus pyogenes p. , 134

streptolysin O p. 131

type II hypersensitivity p. 110

Type II hypersensitivity reactions

rheumatic fever p. 320


“Rheumatism” is used to describe inflammation in the joints, muscles, and the fibrous tissue, so rheumatic fever is a type of inflammatory disease that can damage the heart tissue, and lead to rheumatic heart disease.

Rheumatic fever develops after streptococcal pharyngitis, inflammation of the throat due to Streptococcus pyogenes where pyogenes literally means “makes pus”. The bacteria is sometimes referred to as “Group A beta hemolytic” streptococcus, and the infection itself is most often just called Strep throat. This particular group of streptococcus has an antigen that lumps it into a group called “group A”, and it also produces an enzyme called streptolysin, that completely lyses nearby red blood cells, or causes them to rupture—rupturing red blood cells is called hemolysis, right? And when those red blood cells rupture and are destroyed, it’s called beta-hemolysis—as opposed to alpha-hemolysis, where cells aren’t actually destroyed, they’re just damaged or bruised.

Some of these strep bacteria have a protein on their cell wall called “M protein”, and this particular protein is highly antigenic, meaning that the immune system sees it and recognizes it as a foreign molecule, and mounts an immune response, which rightfully so, produces antibodies against these proteins. Those antibodies, though, are thought to cross-react with proteins on some of our body’s own cells, like cells in the myocardium (or heart muscle) and heart valves, but also cells in the joints, the skin and the brain.

This phenomenon, where antibodies accidentally target proteins on our own cells because they look like the proteins on foreign cells, is called molecular mimicry, and is an example of what’s called a type 2 hypersensitivity reaction. Once bound to cardiac tissue, the antibodies activate nearby immune cells, which causes a cytokine-mediated inflammatory response and tissue destruction.

Obviously though, not everyone that gets strep throat gets rheumatic fever, right? And it’s actually only a small minority that get it, estimated around 3%, and it’s more likely to happen in children or people in areas of poverty and crowding.


Rheumatic heart disease is a condition that develops as a complication of rheumatic fever, which is an inflammatory disease that can occur as a complication of streptococcal infections such as strep throat. This occurs due to the antibodies against streptococcal M proteins that cross-react with proteins in the myocardium, heart valves, joints, skin, and brain.

Rheumatic heart disease is characterized by heart tissue scarring that damages the heart valves, leading to problems such as mitral stenosis, and aortic regurgitation. Symptoms of rheumatic heart disease include shortness of breath, fatigue, chest pain, and heart palpitations. Treatment may involve antibiotics to prevent further streptococcal infections, and surgery to repair or replace damaged heart valves.


  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "Acute rheumatic fever" The Lancet (2005)
  5. "Rheumatic fever & rheumatic heart disease: the last 50 years" Indian J Med Res (2013)
  6. "Rheumatic heart disease" The Lancet (2012)
  7. "Rheumatic Heart Disease: Causes, Symptoms, and Treatments" Cell Biochemistry and Biophysics (2015)

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