Vasculitis

What is Vasculitis?

Vasculitis is a group of rare diseases in Singapore, characterised by inflammation of blood vessels (arteries and veins). This inflammation can damage your blood vessel walls, causing them to thicken, which in turn causes the diameter of the vessel to narrow, weaken, or even close off entirely. As a result, your blood flow to various organs and tissues may be restricted or interrupted. Vasculitis may be acute or chronic.

There are many types of vasculitis depending on the size of the blood vessels affected:

• Behcet’s disease: characterised by recurrent oral and genital ulcers, skin lesions, and inflammation of blood vessels.
• Buerger’s disease: affects blood circulation to the arms and legs.
• Central nervous system (CNS) vasculitis: also known as primary CNS angiitis, affects blood vessels in the central nervous system.
• Cogan’s syndrome: an autoimmune condition that causes inflammation of the eyes and ears.
• Cryoglobulinemic vasculitis: associated with abnormal proteins in the blood, forming deposits in blood vessels. This causes poor circulation and is linked to hepatitis C.
• Eosinophilic granulomatosis with polyangiitis (EGPA): formerly known as Churg- Strauss Syndrome. It affects small and medium-sized blood vessels and can affect various organs, including lungs, skin, nerves, gastrointestinal, and heart.
• Giant cell arteritis: also known as temporal arteritis, affects the medium and large arteries, and commonly involves the arteries around the temples
• Granulomatosis with polyangiitis (GPA): formerly known as Wegener’s granulomatosis. It is a form of vasculitis that affects small and medium-sized blood vessels. It often affects the lungs, kidneys, nose, and throat.
• Immunoglobulin A (IgA) vasculitis: formerly known as Henoch-Schonlein purpura, is a type of vasculitis affecting small blood vessels. It is more common in children but can also occur in adults. It causes joint pain, skin rash, abdominal pain and kidney inflammation.
• Kawasaki disease: it involves inflammation of small and medium-sized arteries, including the coronary artery of the heart. It is an acute febrile illness that primarily affects children under the age of 5.
• Microscopic polyangiitis: It primarily affects small blood vessels, leading to inflammation in the kidneys, lungs, joints, and skin.
• Polyarteritis nodosa (PAN): primarily affects medium-sized arteries, leading to aneurysms (ballooning in blood vessel caused by a weakening vessel wall). This causes inflammation in various organs, such as the skin, joints, nerves, and gastrointestinal tract, resulting in rash, joint pain and abdominal pain. 
• Retinal vasculitis: involves inflammation of the blood vessels in the retina (eye), leading to various visual symptoms. This is often associated with systemic diseases like autoimmune disorders or infections.
• Takayasu vasculitis: primarily affects the aorta and its branches. It typically occurs in young to middle-aged Asian women.

What are the common causes of Vasculitis in Singapore?

Vasculitis is an autoimmune disease where the body’s immune cells attack healthy cells and tissues. However, the exact cause of vasculitis is still not fully understood. It is believed to result from a complex interplay of factors involving the body's immune system, genetic predisposition, and potential environmental triggers such as certain infections (Hepatitis B or C).

Due to this complexity and the various potential causes, the exact trigger for vasculitis can be challenging to identify in many cases. Management and treatment often involve suppressing the immune response and addressing the underlying inflammation, which can help alleviate symptoms and prevent further damage to blood vessels.

What are the symptoms of Vasculitis?

The symptoms of vasculitis vary depending on the type and affected body part. Some symptoms of vasculitis include: 

• Fever: vasculitis can cause an unexplained and prolonged fever. 
• Fatigue: persistent fatigue and weakness are prevalent among individuals with vasculitis.
• Muscle and joint pain: pain and stiffness in your muscles and joints can affect mobility and reduce physical activity.
• Skin changes: rashes, skin sores, or discolouration of the skin, which can be distressing and sometimes painful, are common symptoms of some vasculitis.
• Nerve problems: nerve involvement can result in symptoms like numbness, tingling, or limb weakness. This can affect your sensation and strength.
• Headaches: persistent and severe headaches are possible with certain types of vasculitis
• Eyesight changes: vasculitis may impact your eyesight, resulting in vision problems and redness in the eyes.
• Organ-specific symptoms: you may experience symptoms specific to those affected organs. For example, you may have shortness of breath or even cough up blood if it affects your lungs and abdominal pain if the gastrointestinal tract is affected or there are changes in kidney function.

If you suspect you have vasculitis or are experiencing any of the symptoms mentioned above, make an appointment with Asia Arthritis Rheumatology Clinic today.

Is Vasculitis painful?

Yes, vasculitis can be painful. The level and type of pain vary depending on several factors, including the specific blood vessels affected and the extent of inflammation.

Individuals with vasculitis often experience muscle and joint pain, which can be uncomfortable and affect mobility. Skin symptoms, such as rashes or ulcers, can also be painful. In some cases, headaches, especially in certain types of vasculitis, can be severe and distressing.

Additionally, nerve problems like numbness and tingling can cause discomfort. The pain associated with vasculitis is typically one of the reasons individuals seek medical attention from a rheumatologist for diagnosis and treatment.

Who is at risk of Vasculitis in Singapore?

Vasculitis can affect individuals of any age, gender, or ethnicity. In Singapore, the risk of vasculitis is not limited to a particular group. However, several factors may increase the risk of developing vasculitis; these are:

• Age: some types of vasculitis, such as giant cell arteritis are more common in older adults and rarely occurs before the age of 50
• Gender: certain types of vasculitis are more common in certain genders. For example, giant cell arteritis is more common in women, while Buerger’s vasculitis is more common in men.
• Genetics: Behcet’s disease and granulomatosis with polyangiitis sometimes run in families.
• Infection: infections such as hepatitis C increase your risk of developing cryoglobulinemic vasculitis.
• Medications: vasculitis can sometimes be triggered by certain medications such as propylthiouracil, hydralazine and minocycline.
• Lifestyle choices: certain vasculitis, such as Buerger’s vasculitis, are caused by tobacco use and smoking. 
• Immune problems: individuals with other autoimmune conditions such as lupus, rheumatoid arthritis, and scleroderma have a higher risk of vasculitis. 

How is vasculitis diagnosed?

Vasculitis can be diagnosed by a rheumatologist and is usually conducted in the following ways:

• Medical history: your rheumatologist will begin by discussing your medical history and any symptoms you are experiencing, including fevers, muscle and joint pain, changes in your skin, headaches, nerve issues, and how long these symptoms persist.
• Physical examination: a physical exam is conducted to check for symptoms such as skin rashes, joint swelling, or other physical manifestations of vasculitis.
• Blood tests: various blood tests are performed to look for specific markers of inflammation, such as elevated levels of C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR). Certain autoantibodies, such as antineutrophil cytoplasmic antibodies (ANCA) antinuclear antibodies (ANA), may be positive. 
• Imaging tests: imaging tests, including X-rays, ultrasound, computed tomography scans (CT scans), or magnetic resonance imaging (MRI) scans, may identify inflammation in the blood vessels and any resulting organ damage.
• Biopsy: in many cases, a tissue biopsy is the most definitive way to diagnose vasculitis. A small sample of the affected tissue, such as skin, kidney, or lung, is extracted and examined under a microscope. This can confirm the presence of vasculitis and help determine the type.

Diagnosing vasculitis can be complex due to its diverse symptoms and the need to rule out other conditions. It is a collaborative effort between you and your rheumatologist. Early diagnosis is crucial for effective management and treatment.

If you suspect you have vasculitis or are experiencing symptoms, consult a rheumatologist for a proper evaluation and diagnosis.

What are the treatment options available for Vasculitis in Singapore?

The treatment of vasculitis typically involves a collaborative effort between rheumatologists and other specialists to develop a tailored approach based on the specific type and severity.

Common treatment options for vasculitis include the following:

• Medications: immunosuppresive drugs such as methotrexate, azathioprine or cyclophosphamide, corticosteroids, or biologics such as rituximab may be prescribed to control inflammation of blood vessels.
• Plasma exchange: in severe cases, plasma exchange, also known as plasmapheresis, can be used to remove and replace harmful antibodies from the blood.
• Lifestyle adjustments: maintaining a healthy lifestyle, including regular exercise, a balanced diet, and stress management, can help overall well-being.
• Regular monitoring: close follow-up is crucial to monitor disease activity and adjust treatment if necessary.

Treatment plans are highly individualised, and your rheumatologist will work closely with you to ensure the most effective management. Communication and regular check-ups are key to managing vasculitis successfully.

Frequently Asked Questions