Systemic vasculitis

Disease class: Vasculitis

Vasculitis, the inflammation of a blood vessel, can be a local response to common pathological processes such as infections.

However, there are a variety of diseases called systemic vasculitides, which involve vasculitis throughout the entire circulatory system. Systemic vasculitis can cause a wide range of signs and symptoms. It is often destructive and life-threatening.

 

 Examples

• Large vessel vasculitis
• Giant cell arteritis (GCA) 
• Takayasu's arteritis (TA)

• Medium vessel vasculitis
• Polyarteritis nodosa (PAN)
• Mucocutaneous lymph node syndrome (Kawasaki disease) (KD)

• Small vessel vasculitis
• Anti-glomerular basement membrane disease (Goodpasture's syndrome)
• Granulomatosis with polyangiitis (Wegener's granulomatosis)
• Eosinophilic granulomatosis with polyangiitis (Churg Strauss Syndrome) 
• Variable vessel vasculitis
• Behçet's disease (BD)

 

Classification

In 1994, the first international Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides proposed a classification system for vasculitis.
In 2012, a second conference developed a revised classification system.

Classification of systemic vasculitides according to CHCC 2012

Large vessel vasculitis

These disease may present as occlusion of large vessels, for example, stroke (cerebrovascular infarct), heart attack (cardiovascular infarct), limb claudication, gangrene.

• Giant cell arteritis (GCA) 
• Takayasu's arteritis (TA)

Medium vessel vasculitis

A large number of medium vessels supply the gut, nerves and kidneys.

• Polyarteritis nodosa (PAN) 
• Mucocutaneous lymph node syndrome (Kawasaki disease) (KD)

Small vessel vasculitis

Small vessels perfuse the majority of tissues in the body. These often present as disturbance in microvascular function, for example renal failure (kidneys) or asymmetric neuropathy (nerves).

• Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) 
• Microscopic polyangiitis (MPA)
• Granulomatosis with polyangiitis (GPA) (Wegener’s granulomatosis)
• Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome)
• Immune complex SVV
• Anti-glomerular basement membrane disease (Anti-GBM) (Goodpasture's syndrome)
• Cryoglobulinemic vasculitis (CV)
• IgA vasculitis (Henoch-Schönlein) (IgAV)
• Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)

Variable vessel vasculitis

• Behçet's disease (BD)
• Cogan’s syndrome (CS)

Single-organ vasculitis

• Cutaneous leukocytoclastic angiitis 
• Cutaneous arteritis
• Primary central nervous system vasculitis 
• Isolated aortitis

Vasculitis associated with systemic disease

• Lupus vasculitis
• Rheumatoid vasculitis
• Sarcoid vasculitis
• Others

Vasculitis associated with probable etiology

• Hepatitis C virus–associated cryoglobulinemic vasculitis
• Hepatitis B virus–associated vasculitis
• Syphilis-associated aortitis
• Drug-associated immune complex vasculitis
• Drug-associated ANCA-associated vasculitis
• Cancer-associated vasculitis
• Others

 

Investigations

Initial immunology screening

• ANA
• ANCA
• C3, C4
• CRP
• RF
• Immunoglobulins and Cryoglobulins