Vasculitis (Latin: vasculum – vessel, itis – inflammation) refers to a group of conditions that causes inflammation of the blood vessels in the body. This leads to narrowing, weakening, or blockages in the blood vessels and thus reduce blood flow to vital organs and tissues. Any part of the body can be affected by vasculitis, and multiple different organs are often affected at the same time (systemic vasculitis), leading to a wide range of symptoms.
There are many different types of vasculitis, including primary vasculitis (where the exact cause of which is unknown) and secondary vasculitis (triggered by other conditions such as infections, cancers and other autoimmune conditions). Different types of blood vessels may be affected depending on the specific condition, ranging from the small capillaries to the larger blood vessels including the veins and/or arteries. The arteries, which are the blood vessels that supply oxygenated blood from the heart to all parts to the body, are more commonly affected.
Primary vasculitis can be classified as:
Small vessel vasculitis (affecting the small arteries):
ANCA-associated vasculitis (vasculitis associated with the anti-neutrophil cytoplasmic antibody, ANCA)
Eosinophilic granulomatosis polyangiitis
Granulomatosis polyangiitis
Microscopic polyangiitis
Other small vessel vasculitis not associated with the ANCA antibody but due to autoimmune cause:
IgA vasculitis (also known as Henoch–Schönlein purpura)
Cryoglobulinemic vasculitis
Hypocomplementemic urticarial vasculitis
Medium vessel vasculitis (affecting the medium sized arteries)
Variable vessel vasculitis (inflammation that can affect all types of vessels including the veins and arteries)
Behcet’s disease
Cogan syndrome
What causes vasculitis?
In most cases of primary vasculitis, the exact cause is not well understood, but is thought to result from an autoimmune response, where the body’s immune system mistakenly attacks its own blood vessels. This can be contributed by a complex relationship between genetic and environmental factors.
For secondary vasculitis, the blood vessel inflammation is triggered due to an underlying autoimmune condition, infections, certain medications, or cancers.
Symptoms vary depending on the type of disease, size of vessel and organs involved. Fever, loss of weight or loss of appetite, and fatigue are symptoms that are common to all of the systemic vasculitis.
Brain and Nerves:
Numbness or weakness
Slurred speech
Difficulty swallowing
Double vision
Headache, worse when chewing on food (jaw claudication)
Unsteadiness
Eyes:
Redness of the eyes
Blurring of vision or loss of vision
Painful eyes
Severe sensitivity when looking at the light (photophobia)
Ears and nose
Nose bleed
Recurrent sinus problems
Blocked nose or blocked ears
Pain inside the ears
Ear discharge
Hearing loss
Heart and Lungs
Chest pain
Shortness of breath
Leg swelling
Cough, including coughing up blood
Skin
Mottled purplish skin (also known as livedo reticularis)
Various rashes, most classically purplish rashes which may be painful
Painful ulcers and nodules
Kidneys
High blood pressure
Generalised swelling
Bubbles in the urine
Blood in the urine which may not be visible to the naked eye (microscopic haematuria)
Reduced urine volume
Gut
Abdominal pain worse after meals
Diarrhea including bloody diarrhea
Musculoskeletal
Joint pains
Muscle aches
Pain in the arms/legs after activity (limb claudication)
Not everyone will experience all of these symptoms. Clinical findings are often different in different patients, and they can also evolve over time.
For more details on the symptoms of specific types of vasculitis, please refer to the individual disease webpages.
How is vasculitis diagnosed?
Diagnosis involves a thorough clinical assessment with history taking including a comprehensive review of medication list, and physical examination.
Blood tests are performed to assess the extent of disease, including blood counts, kidney function, inflammatory markers such as the Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Other tests such as antibody tests and infection screens may be performed depending on clinical suspicion.
Imaging tests such as CT, ultrasound, MRI or PET-CT scans may be performed to look for inflammation of the blood vessels, complications and other secondary causes such as cancers. The choice of imaging modality depends on various factors such as the suspected blood vessels involved.
Tissue biopsy may be required in certain types of vasculitis to confirm presence of blood vessel inflammation in one or more of the affected organs (e.g. skin, kidney, nerve, lung biopsy).
What is the treatment for vasculitis?
Vasculitis can be effectively treated with medications. Treatment depends on the type of vasculitis, severity and extent of organ involvement, the underlying cause of vasculitis (for secondary vasculitis), and other patient factors. Prompt treatment is required in life or organ-threatening situations.
Treatment usually includes medications to suppress the inflammation and calm down the immune system. These include: •Corticosteroids are often the initial treatment given to rapidly bring down the vessel inflammation and ameliorate the organ/tissue damage. In severe cases, high doses may be required initially (intravenous injections through the veins or oral), then reduced over time. •Immunosuppressive agents – these are usually used in addition to steroids to reduce the need for long-term steroids and for control of disease activity. These may be given intravenously or orally. •Biologic agents are newer targeted medications given intravenously or subcutaneously into the abdomen or thighs.
Any underlying condition that is triggering the vasculitis needs to be addressed. This includes treatment of underlying cancers and infections (e.g. antibiotics and antivirals).
Additionally, other medical conditions are addressed as part of holistic care, such as high blood pressure, high cholesterol, diabetes, and osteoporosis. To prevent infections when on high doses of immune suppression medications, prophylactic antibiotics and certain vaccinations are encouraged (e.g. influenza, pneumococcal, COVID-19, shingles and respiratory syncytial virus).
Given that vasculitis usually affects multiple organ symptoms, patients are often cared for by a multidisciplinary team of medical professionals, comprising a primary rheumatologist with other relevant subspecialty teams and allied health professionals. Your treatment plan will be carefully tailored based on your symptoms, preferences, and goals of care.
What should I do if I have vasculitis?
Patients with vasculitis are able to live active lives with the right treatment and support. Some recommended lifestyle changes include: •Eat a balanced diet. Avoid raw and partially cooked food to reduce risks of infections, especially if you are on medications that suppress the immune system (e.g. steroids). •Avoid smoking. •Stay active and exercise in moderation. Ensure sufficient rest and sleep well. •Attend regular medical check-ups to monitor your health. Participate actively in your care and ask questions during your clinic consultations. •Take your medications as prescribed, even when you feel well, to avoid disease flares. •Seek medical attention if you are unwell or suspect a flare of vasculitis
What should I do if I suspect I have vasculitis?
Seek a doctor's advice. You will be referred to a specialist (rheumatologist) who can confirm the diagnosis and start you on the appropriate treatment. Once diagnosed, it is important to start treatment early and follow your rheumatologist’s advice. This condition is managed by the Division of Rheumatology and Allergy.
