Other 5 types of Vasculitis

Behçet's Disease

• Definition: Vasculitis targeting small arteries and venules, of unknown origin, often associated with HLA-B51.
• Prevalence: Common in Mediterranean countries (e.g., Turkey) and Japan; more prevalent in males, typically ages 20-40.
• Etiology: Unknown; thought to have autoimmune and genetic components (HLA-B51 association).
• Clinical Features:
  • Recurrent, painful oral ulcers (10-30 days duration).
  • Recurrent genital ulcers (60-80% of cases).
  • Ocular involvement (common): anterior/posterior uveitis, retinal vasculitis (potential blindness in 25% of patients).
  • Skin manifestations: erythema nodosum, papulo-pustular rash.
  • Neurological complications: hemiparesis, cranial nerve lesions, aseptic meningitis, headache.
  • Vascular complications: aneurysm, deep vein thrombosis (DVT), thrombophlebitis.
  • Arthritis (non-deforming): affects elbows, knees, and ankles in 60% of cases.
  • Rare renal manifestations.
• Diagnosis: Recurrent oral ulcers (at least 3 times in 12 months) plus two of the following: recurrent genital ulcers, eye lesions, skin lesions, positive pathergy test (needle prick resulting in pustules within 48 hours).
• Treatment:
  • Oral/genital ulcers: colchicine and topical glucocorticoids.
  • Erythema nodosum, arthritis, arthralgia: colchicine.
  • Uveitis, recurrent venous thrombosis, neurological disease: glucocorticoids and immunosuppressants (e.g., azathioprine).
• Diagnostic Testing: HLA-B51.

Takayasu Arteritis

• Definition: Granulomatous vasculitis affecting the aorta and its major branches (occasionally pulmonary arteries).
• Prevalence: Typical age 25-30; more frequent in females (8:1 ratio).
• Clinical Features: Claudication, fever, arthralgia, weight loss.
• Diagnostic Findings: Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), normocytic anemia.
• Diagnostic Investigations: CT or MRI angiography, PET-CT.
• Treatment: High-dose glucocorticoids and immunosuppressants.

Kawasaki Disease

• Definition: Primarily affecting coronary vessels, usually in children under 5.
• Etiology: Unknown, possibly post-immune response to infection.
• Clinical Features: Fever, generalized rash (including palms and soles), inflamed oral mucosa and conjunctiva.
• Cardiovascular Complications: Coronary arteritis leading to myocardial infarction (MI), transient coronary dilatation, myocarditis, pericarditis, peripheral vascular insufficiency, and gangrene.
• Treatment: Aspirin, cyclosporine, and intravenous immunoglobulin (IVIG).

Giant Cell Arteritis

• Definition: Granulomatous arteritis affecting medium and large-sized arteries, often associated with polymyalgia rheumatica (PMR).
• Prevalence: Common age 50-70; more frequent in females (3:1 ratio).
• Association: HLA-DRB1.
• Clinical Features: Symmetrical pain and stiffness affecting the shoulder and pelvic girdle.
• Other Symptoms: Headache (temporal or occipital), scalp tenderness, jaw pain, and visual disturbance (amaurosis or blindness in one eye).
• Symptoms: weight loss, fatigue, malaise, and night sweats are common.
• Diagnostic Findings: Elevated ESR and CRP, elevated liver function tests (LFTs), normocytic anemia.
• Diagnostic Investigations: Temporal artery biopsy and ultrasound.
• Treatment: Glucocorticoids (dramatic response within 48-72 hours).

Henoch-Schönlein Purpura

• Definition: Small-vessel vasculitis caused by immune complex deposition following an infectious trigger.
• Prevalence: Predominantly affects children and young adults.
• Clinical Features: Purpura (bruising) over buttocks and lower legs accompanied by abdominal pain, gastrointestinal (GIT) bleeding, and arthralgia.
• Nephritis: May develop up to 4 weeks after the onset of other symptoms.
• Diagnosis: Biopsy of affected tissue showing vasculitis with IgA deposits in the vessel wall.
• Treatment: Typically self-limiting. Glucocorticoids and immunosuppressants may be needed for severe cases, especially with nephritis.
• Diagnostic Testing: Skin biopsy to show IgA deposits.