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.
Wissenswertes über "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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Related search terms: Vasculitis + Vaskulitis + Vascular inflammation