Marfans Syndrome

Skeletal disproportion (arm span greater than height), arachnodactyly

(long, thin, spider fingers), sternal depression, generalised hypermobility
of joints, lens dislocation, high arched palate, scoliosis
Mutations of fibrillin gene component of ECM
Complications: mitral valve prolapsed, aortic incompetence, aortic
dissection
Weakening o faortic media progressive dilatation of ascending aorta
aortic regurgitation and aortic dissection
Rheumatic Fever
Pathogenesis
Immune-mediated delayed response to infection with specific strains of
GAS possess Ag that cross-react with cardiac myosin and sarcolemmal
membrane protein
Ab produced against streptococcal Ag mediate inflammation in
endocardium, myocardium, pericardium, joints and skin
Microscopic feat: Fibrinoid degeneration in collagen of CT, Aschoff nodules
nodules resulting from inflammation of heart muscle & composed of
multinucleated giant cells surrounded by macrophages and T lymphocytes