Systemic Vasculitis: What It Is and What Types of Vasculitis Exist

Vasculitis is not a single disease but a group of conditions in which the immune system mistakenly attacks the body's own blood vessels. Inflammation makes the vessel wall swollen and narrows the lumen, and which organs are left without normal blood flow determines both the name of the form and the pattern of symptoms.

Systemic Vasculitis: What It Is and Why Vessels Are Affected

Imagine the circulatory system as a network of pipes of different calibers: from major arteries down to capillaries that reach every cell. In vasculitis, the damage begins not in the organ but in the pipe itself: an inflamed vessel narrows, may close completely, or may stretch and weaken, forming an aneurysm whose rupture causes dangerous internal bleeding.

Arteries, veins, and capillaries are affected. A trigger is sometimes an infection, another disease, or a medication, but often the cause remains unknown; a combination of genetic predisposition with environmental factors, the involvement of Staphylococcus aureus or hepatitis virus is discussed. Primary vasculitis arises from the vessel itself; secondary vasculitis is a reaction to another condition. General symptoms are nonspecific: fever, swelling, a feeling of general malaise.

Classification of Vasculitides: By Vessel Size

The basis of the nomenclature was set by the Chapel Hill Consensus Conference (CHCC) in its 2012 revision. The main principle is vessel caliber: it suggests which organs are at risk.

• Large vessels: giant cell (temporal) arteritis and Takayasu arteritis.
• Medium: polyarteritis nodosa and Kawasaki disease.
• Small: ANCA-associated and immune complex vasculitides.
• Variable-vessel and single-organ: Behçet's disease, primary CNS angiitis, cutaneous leukocytoclastic angiitis, isolated aortitis.

ANCA-Associated Vasculitides and Pulmonary Vasculitis

ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener's disease), eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), and microscopic polyangiitis. The first affects the vessels of the nose, sinuses, ears, lungs, and kidneys: fever, night sweats, sinusitis, nosebleeds, hemoptysis; without treatment, organ failure is possible. Microscopic polyangiitis most often occurs in middle age and affects the lungs, kidneys, and nerves — rash, hemoptysis, numbness. Eosinophilic granulomatosis occurs in adults aged 38–54 and begins with asthma and allergic rhinitis; later the nerves, kidneys, and heart muscle are involved.

Cutaneous Vasculitides: Leukocytoclastic and Urticarial

Cutaneous leukocytoclastic angiitis is classified as a single-organ vasculitis: it affects a single organ — the skin. Hypocomplementemic urticarial vasculitis (anti-C1q) belongs to the group of immune complex small-vessel lesions. The skin is also involved in systemic forms — in 25–35% of patients with granulomatosis with polyangiitis and in 70% with microscopic polyangiitis. Immunoglobulin A vasculitis (Henoch-Schönlein purpura) affects the skin, kidneys, or intestines, is more common in children, and usually resolves without treatment: a rash in the form of small bruises, joint and abdominal pain, blood in the urine or stool.

Cerebral, Cryoglobulinemic, and Other Rare Forms

Primary CNS angiitis — cerebral vasculitis — is classified as single-organ involvement. Cryoglobulinemic vasculitis is an immune complex form of small-vessel disease associated with hepatitis C virus. Polyarteritis nodosa targets the arteries of the intestines, kidneys, and nerves and is sometimes triggered by hepatitis B: muscle and joint pain, abdominal pain after eating, rash, numbness, intestinal ulcers. Takayasu arteritis occurs in young women, Kawasaki disease in children under 5, and Behçet's disease causes ulcers in the mouth and on the genitals.

How Vasculitides Are Diagnosed and When a Rheumatologist Is Needed

The diagnosis is assembled piece by piece: testing for antineutrophil cytoplasmic antibodies (ANCA), C-reactive protein, ESR, CT and MR angiography, and in some cases cerebral or aortic angiography. Temporal arteritis requires a rapid response: after age 50 — tenderness in the temples, pain in the jaw muscles while eating, headache, double vision, or vision loss; without treatment, stroke and blindness are possible.

The name of a vasculitis in your report is not in itself a reason to call an ambulance; 103 or 112 — for signs of stroke, sudden vision loss, or hemoptysis with shortness of breath.

This material is for reference: it explains the mechanics of the disease but does not replace a doctor's consultation — it is best to go over your report with a specialist.

This content is for reference and does not replace a doctor's consultation. Medical disclaimer