Overview:

• Vasculitides are characterized by blood vessel inflammation with possible necrosis, ischemia, and organ damage.
• The vessels and organs affected vary by the specific vasculitic disorder.
• General symptoms and signs are due to systemic inflammation,which can cause fever, arthritis, arthralgia, fatigue, and weight loss.
• Skin lesions are associated with small and medium vessel vasculitides.
• Treatments include corticosteroids and immunosuppressants.

MEDIUM VESSEL VASCULITIDES

Polyarteritis nodosa is characterized by necrotizing inflammation that most commonly involves the medium muscular arteries, especially at their branch points.

• The resulting ischemia can affect multiple organ systems:
  – Nervous system involvement often produces mononeuritis multiplex or asymmetric polyneuropathy; sensory and motor deficits of the median, ulnar, and fibular nerves are common.
  – Renal involvement can lead to hypertension, oliguria, and even renal failure.
  – Gastrointestinal involvement can produce pain and malabsorption; when larger vessels, such as the celiac trunk or its branches are affected, fatal aneurysms can develop.
  – Skin manifestations can take various forms, including livedo reticularis (which presents as a purplish lace-like pattern), ulcers, subcutaneous nodules, or even gangrene.
  – Heart failure can occur due to coronary artery obstruction.
• Polyarteritis nodosa can be systemic or cutaneous.
• Some secondary forms are related to hepatitis B and C infections, or to rheumatoid arthritis.
• Most commonly affects men over 50 years of age.

Kawasaki disease, aka, mucocutaneous lymph node syndrome

• Characterized by proliferative inflammation.
• Tends to involve the medium muscular arteries.
• Predilection for the coronary arteries, which are affected in approximately 20% of cases
  – In fact, it is a key cause of acquired heart disease in children.
  – Large coronary aneurysms can be fatal, as they can cause myocarditis, cardiac tamponade, or, as we’ve illustrated here, coronary artery thrombosis.
• Typically presents as a self-limiting, acute febrile illness in children:
  – Fever
  – Conjunctivitis
  – Erythematous macular rash
  – Edema and desquamation, particularly of the limbs
  – Cervical lymphadenopathy
  – A red, inflamed throat
  – Strawberry tongue with dry, fissured lips.

VARIABLE VESSEL SIZE VASCULITIDES

Behçet disease

• A chronic, relapsing vasculitis that affects vessels of all sizes.
• Mucosal inflammation is a common manifestation of the disorder, and often causes oral and genital lesions; ocular inflammation can produce uveitis or hypopyon.
• Cutaneous manifestations vary, and include pus-filled bumps, palpable purpura, erythema nodosum, and superficial thrombophlebitis.
• Typical age of onset is around 20 years old.
• Behçet disease is equally common in men and women, though it’s often worse in men.

Buerger disease

Don’t confuse Buerger disease with Berger’s disease, a nephropathy caused by IgA accumulation.

• Characterized by inflammation and occlusive thrombosis of the medium and small arteries and veins
• Most commonly affects the extremities; signs and symptoms typically begin distally and move proximally.
• Ischemia from vessel occlusion often produces numbness, coldness, or tingling in the extremities, with claudication, then, pain at rest.
• Affected limbs are often cold, sweaty, and cyanotic, and ulcers form that progress to gangrenous.
• Smoking is a major cause of Buerger disease, and smoking cessation is key to remission.