Autoimmune Disease

Systemic Vasculitis

Inflammation of blood vessel walls — a heterogeneous group of diseases classified by vessel size

Overview

What is Vasculitis?

Vasculitis encompasses a heterogeneous group of autoimmune disorders characterised by inflammation and necrosis of blood vessel walls. The resulting vessel wall damage causes luminal narrowing, thrombosis, aneurysm formation, and end-organ ischaemia. Vasculitis is classified by the size of predominantly affected vessels (large, medium, or small), the presence of ANCA antibodies, and specific clinicopathological features. It may be primary (idiopathic autoimmune) or secondary to infection, malignancy, drugs, or other connective tissue diseases. Accurate classification is essential as treatment strategies differ significantly between subtypes.

Clinical Presentation

Signs & Symptoms

Symptoms are typically symmetrical and involve small joints of the hands and feet first, progressing to larger joints.

Currently Licensed States:

Systemic Involvement

Organs Affected

Cardiovascular

Pericarditis, accelerated atherosclerosis, increased MI risk

Pulmonary

Pleuritis, pneumonitis, pulmonary hypertension

Ocular

Episcleritis, scleritis, keratoconjunctivitis sicca

Haematological

Anaemia of chronic disease, Felty syndrome

Diagnosis

ACR/EULAR 2010 Criteria

Score ≥6/10 confirms RA. Applies to patients with at least 1 swollen joint not explained by another disease.

Laboratory & Imaging

Key Investigations

Pathophysiology

Disease Mechanism

1


Genetic susceptibility

HLA-DR2/DR3, complement deficiencies (C1q, C4), IRF5, STAT4 gene polymorphisms increase risk.

2


Triggering factors

UV light, infections (EBV), hormonal changes, and drugs (procainamide, hydralazine) can precipitate disease.

3


Autoantibody formation

B cells produce ANA, anti-dsDNA, and other autoantibodies. T helper cells and type I interferons amplify the response.

4


Immune complex deposition

Autoantibody-antigen complexes deposit in tissues — kidneys, skin, vessels — activating complement and causing inflammation.

5


End-organ damage

Persistent inflammation leads to fibrosis and functional impairment of target organs if disease remains uncontrolled.

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