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Vasculitis – Causes, Symptoms and Treatment

Vasculitis refers to inflammation of the blood vessel walls. It can affect multiple organs and requires early diagnosis and targeted medical treatment.

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Things worth knowing about "Vasculitis"

Vasculitis refers to inflammation of the blood vessel walls. It can affect multiple organs and requires early diagnosis and targeted medical treatment.

What is Vasculitis?

Vasculitis is an umbrella term for a group of disorders characterised by inflammation of blood vessel walls. It can affect arteries, veins, or capillaries – spanning small, medium, or large vessels. The inflammation can involve a single organ or spread throughout the body, often narrowing or blocking blood flow and causing damage to tissues and organs.

Causes

The exact causes of vasculitis are not always fully understood. A distinction is made between primary and secondary forms:

Primary vasculitis: Inflammation occurs without an identifiable underlying disease. The immune system mistakenly attacks the walls of blood vessels (autoimmune reaction).
Secondary vasculitis: It arises as a consequence of another condition, such as infections (hepatitis B or C), autoimmune diseases (lupus erythematosus, rheumatoid arthritis), or as a reaction to certain medications.

Classification and Types

Vasculitides are classified according to the size of the affected vessels:

Large-vessel vasculitis: e.g. giant cell arteritis (temporal arteritis), Takayasu arteritis
Medium-vessel vasculitis: e.g. polyarteritis nodosa, Kawasaki disease
Small-vessel vasculitis: e.g. ANCA-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis), IgA vasculitis (Henoch-Schonlein purpura)

Symptoms

The symptoms of vasculitis are highly variable and depend on which vessels and organs are affected. Common general symptoms include:

Fever, fatigue, and a general sense of illness
Unintentional weight loss
Muscle and joint pain
Skin changes (redness, bleeding into the skin, palpable purpura)
Headaches (especially in giant cell arteritis)

Organ-specific symptoms may include:

Kidneys: Blood in the urine, reduced kidney function
Lungs: Cough, coughing up blood, shortness of breath
Nerves: Numbness, tingling, or paralysis
Eyes: Visual disturbances or sudden vision loss

Diagnosis

Diagnosing vasculitis requires a thorough medical evaluation. Typical diagnostic steps include:

Laboratory tests: Full blood count, inflammatory markers (CRP, ESR), ANCA antibodies, kidney and liver function tests, urinalysis
Imaging: Ultrasound, MRI, CT angiography to visualise affected vessels
Tissue biopsy: A sample of affected tissue or vessel wall examined under a microscope – often the most important diagnostic step
PET-CT: Used to assess disease activity in large-vessel vasculitis

Treatment

Treatment depends on the type and severity of vasculitis and the organs involved. The main goals are to suppress inflammation and prevent organ damage.

Medication

Corticosteroids (cortisone): Often the first-line treatment to rapidly reduce inflammation
Immunosuppressants: e.g. cyclophosphamide, methotrexate, azathioprine, or mycophenolate mofetil for long-term disease control
Biologics: e.g. rituximab (anti-CD20) for ANCA-associated vasculitis; tocilizumab for giant cell arteritis

Treating the Underlying Cause

In secondary vasculitis, treating the underlying condition (e.g. antiviral therapy for hepatitis) is essential to controlling the vascular inflammation.

Long-term Management

As vasculitis is often chronic and prone to relapse, regular medical follow-up and, in many cases, long-term maintenance therapy are necessary. Patients benefit from close collaboration with specialists such as rheumatologists, nephrologists, and internists.

Prognosis

The prognosis of vasculitis depends strongly on the specific type, the extent of organ involvement, and how early the diagnosis is made. Early and consistent treatment significantly improves outcomes. Some patients achieve full remission, while others require lifelong medical management.

References

Jennette JC et al. - 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis & Rheumatism, 2013; 65(1):1-11.
European League Against Rheumatism (EULAR): Recommendations for the management of ANCA-associated vasculitis. Annals of the Rheumatic Diseases, 2022.
Watts RA, Robson J - Introduction, epidemiology and classification of vasculitis. Best Practice & Research Clinical Rheumatology, 2018; 32(1):3-20.

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