B. Polyarteritis Nodosa

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B. Polyarteritis Nodosa

• - Is a systemic vasculitis of small- or
  medium-sized muscular arteries.
• - Typically involving renal and visceral vessels
  but sparing the pulmonary circulation.
• - Vessels of the kidneys, heart, liver, and
  gastrointestinal tract are involved in
  descending order of frequency.

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• - Impaired perfusion with ulcerations, infarcts,
  ischemic atrophy, or hemorrhages may be the first
  sign of disease
• - A classic presentation can involve some
  combination of
• a. Rapidly accelerating hypertension due to renal
  artery involvement
• b. Abdominal pain and bloody stools caused by
  vascular gastrointestinal lesions

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• - Renal involvement is often prominent and a
  major cause of mortality.
• - Untreated, PAN is typically fatal however,
  immunosuppression can yield remissions or cures
  in 90 of cases.

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Kawasaki Disease

• -Kawasaki disease is an acute febrile, usually
  self-limited illness of infancy and childhood
  (80 of patients are 4 years old or younger)
• - Its clinical significance stems primarily from
  a predilection for coronary artery involvement
  that

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• can cause aneurysms that rupture or thrombose,
  resulting in acute myocardial infarctions.
• - Is is the leading cause of acquired heart
  disease in children.

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• Clinical Features.
• - Kawasaki disease typically presents with
  conjunctival and oral erythema and blistering,
  edema of the hands and feet, erythema of the
  palms and soles, a desquamative rash, and
  cervical lymph node enlargement (hence its other
  name, mucocutaneous lymph node syndrome).

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• - Approximately 20 of untreated patients
  develop cardiovascular sequelae, ranging from
  asymptomatic coronary arteritis, to coronary
  artery ectasia, to giant coronary artery
  aneurysms (7 to 8 mm) leading to rupture or
  thrombosis, myocardial infarction, and sudden
  death.

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Microscopic Polyangiitis

• - Microscopic polyangiitis is a necrotizing
  vasculitis that generally affects capillaries,
  small arterioles and venules.
• - Depending on the vascular bed involved, major
  clinical features include

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• hemoptysis, hematuria and proteinuria, bowel
  pain or bleeding, muscle pain or weakness, and
  palpable cutaneous purpura

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Behçet Disease

• - Behçet disease is a small- to medium-vessel
  neutrophilic vasculitis that classically presents
  as a clinical triad of
• 1.recurrent oral aphthous ulcers,
• 2.genital ulcers,
• 3. and uveitis.

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• 4.Granulomatosis with Polyangiitis Previously
  called Wegener granulomatosis,
• - Is characterized by a triad of
• 1. Necrotizing granulomas of the upper
  respiratory tract (ear, nose, sinuses, throat) or
  the lower respiratory tract (lung) or both.

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• 2. Necrotizing or granulomatous vasculitis
  affecting capillaries, venules, arterioles, and
  arteries) most prominent in the lungs and upper
  airways
• 3.Focal necrotizing, often crescentic,
  glomerulonephritis

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• Clinical Features.
• - Classic features include
• 1.persistent pneumonitis with bilateral nodular
  and cavitary infiltrates (95),
• 2.chronic sinusitis (90),
• 3. evidence of renal disease (80).

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Thromboangiitis Obliterans (Buerger Disease)

• - Is characterized by
• 1. Segmental, thrombosing, acute and chronic
  inflammation of medium-sized and small arteries,
  principally the tibial and radial arteries, with
  occasional secondary extension into the veins and
  nerves of the extremities.

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• 2. It is a distinctive disease that often leads
  to vascular insufficiency, typically of the
  extremities.
• 3. It occurs almost exclusively in heavy
  cigarette smokers, usually before age 35.

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• Clinical Features.
• 1. Leg pain induced by exercise that is relieved
  on rest (intermittent claudication),
• 2. instep foot pain induced by exercise (instep
  claudication),
• 3. and a superficial nodular phlebitis (venous
  inflammation).

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• - The vascular insufficiency of Buerger disease
  tends to be accompanied by severe paineven at
  restdue to the neural involvement.
• - Chronic extremity ulcerations develop,
  progressing over time (occasionally
  precipitously) to frank gangrene.

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• - Smoking abstinence in the early stages of the
  disease can often ameliorate further attacks
  however, once established, the vascular lesions
  typically do not respond to smoking
• abstinence.

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Ischemic heart disease
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Ischemic Heart Disease

• Ischemic heart disease (IHD) represents a group
  of pathophysiologically related syndromes
  resulting from myocardial ischemiaan imbalance
  between myocardial supply (perfusion) and cardiac
  demand for oxygenated blood.

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Angina Pectoris

• - Angina pectoris is characterized by paroxysmal
  and usually recurrent attacks of substernal or
  precordial chest discomfort caused by transient
  (15 seconds to 15 minutes) myocardial ischemia
  that is insufficient to induce myocyte
  necrosis.

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• 1-stable angina (occur after certain levels of
  exertion) and caused by
• - is episodic chest pain associated with
  exertion or some other form of increased
  myocardial oxygen demand (e.g., tachycardia or
  hypertension due to fever, anxiety, fear).

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• - pain? a crushing or squeezing substernal
  sensation,
• - radiate down the left arm or to the left jaw
  (referred pain).
• -lesion obstructing 70 to 75 or more of a
  vessel lumen critical stenosis ? cause angina
  only in the setting of increased demand

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• The pain is relieved by rest (reducing demand)
  or by administering agents such as nitroglycerin
  
• such drugs cause peripheral vasodilation and thus
  reduce venous blood delivered to the heart ?
  reducing cardiac work.
• - in larger doses, nitroglycerin also increases
  blood supply to the myocardium by direct coronary
  vasodilation

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• 2-variant angina or Prinzmetal angina
• ( due to vessel spasm ) completely normal vessels
  can be affected.
• Treatment administration of vasodilators such as
  nitroglycerin or calcium channel blockers.