Other 5 types of Vasculitis

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Questions and Answers

Which of the following is most commonly associated with Behçet's disease?

HLA-A1

HLA-B51 (correct)

HLA-B27

HLA-DRB1

What is the most common ocular manifestation in Behçet's disease?

Corneal ulcer

Scleritis

Optic neuritis

Retinal vasculitis (correct)

What is the main diagnostic criterion for Behçet's disease?

Skin lesions

Pathergy test

Recurrent oral ulcers (correct)

Neurological symptoms

Which of the following drugs is primarily used for treating oral and genital ulceration in Behçet's disease?

Colchicine Signup and view all the answers

A positive pathergy test in Behçet's disease involves which of the following findings?

Pustule formation within 48 hours Signup and view all the answers

Takayasu arteritis most commonly affects which vessels?

Aorta and its branches Signup and view all the answers

Which of the following is NOT a common clinical feature of Takayasu arteritis?

Generalized rash Signup and view all the answers

What is the investigation of choice for diagnosing Takayasu arteritis?

CT or MRI angiography Signup and view all the answers

Which of the following laboratory findings is typical in Takayasu arteritis?

Elevated ESR and CRP Signup and view all the answers

What is the primary treatment for Takayasu arteritis?

High-dose glucocorticoids Signup and view all the answers

Kawasaki disease most commonly affects:

Children under 5 years Signup and view all the answers

Which of the following is a major cardiovascular complication of Kawasaki disease?

Myocardial infarction Signup and view all the answers

Kawasaki disease is characterized by all EXCEPT:

Recurrent oral ulcers Signup and view all the answers

What is the main treatment for Kawasaki disease?

Aspirin and IV immunoglobulin Signup and view all the answers

Which of the following is a hallmark of Kawasaki disease?

Strawberry tongue Signup and view all the answers

Giant cell arteritis is commonly associated with which of the following conditions?

Polymyalgia rheumatica Signup and view all the answers

The most common symptom of giant cell arteritis is:

Temporal headache Signup and view all the answers

What is the diagnostic investigation of choice for giant cell arteritis?

Temporal artery biopsy Signup and view all the answers

Which of the following is a common laboratory finding in giant cell arteritis?

Normocytic anemia Signup and view all the answers

What is the initial treatment for giant cell arteritis?

High-dose glucocorticoids Signup and view all the answers

Henoch-Schönlein purpura is predominantly associated with:

Children and young adults Signup and view all the answers

The hallmark skin manifestation of Henoch-Schönlein purpura is:

Purpura over the buttocks and lower legs Signup and view all the answers

What is the best investigation for confirming Henoch-Schönlein purpura?

Skin biopsy showing IgA deposits Signup and view all the answers

Which of the following organs is least commonly affected in Henoch-Schönlein purpura?

Lungs Signup and view all the answers

Which of the following treatments is required in severe cases of Henoch-Schönlein purpura with nephritis?

Glucocorticoids and immunosuppressants Signup and view all the answers

Study Notes

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.

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Description

This quiz takes an in-depth look at Behçet's Disease, a type of vasculitis characterized by recurrent painful ulcers and various systemic manifestations. It covers its prevalence, clinical features, and potential complications in affected individuals. Test your knowledge on this complex condition and its implications.