Vasculitis: Types, Symptoms and Treatment

Vasculitis refers to inflammation of the blood vessels leading to their damage. This leads to poor blood supply to the tissues and organs supplied by these vessels resulting in problems all over the body.

Types of Vasculitis

There are different types of Vasculitis classified based on the size of the vessels predominantly affected.

Large Vessel Vasculitis

• Takayasu Arteritis: This involves inflammation of primarily the aorta and its branches. It often presents with pain and numbness of the arms. This generally affects individuals below the age of 30
• Giant Cell Arteritis or Temporal Arteritis: This also has a tendency to affect the aorta and its branches, but presents in those aged over 60 years. The disease preferentially affects the carotid arteries and the temporal arteries which present as pain in the temples and vision disturbance

Medium Vessel Vasculitis

• Polyarteritis Nodosa: This affects medium to small vessels all over the body, although it typically spares the lungs.
• Kawasaki Disease: This is a condition seen in children, usually less than 5 years of age. It affects the vessels supplying the heart and presents with fever, skin rash and lymph node swelling.

Small Vessel Vasculitis

• Antineutrophil cytoplasmic antibody (ANCA)- associated vasculitis: This is a group of vasculitides which is associated with the presence of these specific antibodies. There are primarily three types:
• Microscopic polyangiitis (MPA): This type affects the small vessels supplying the kidneys and the lungs. More than 90% of patients are positive for ANCA
• Granulomatosis with Polyangiitis (GPA): This was previously called Wegener’s Granulomatosis. It affects small and medium sized vessels primarily in the respiratory tracts from the nostrils to the lungs. It is characterised by the finding of a granuloma which is a pattern of inflammatory cells in the affected tissue.
• Eosinophilic Granulomatosis with polyangiitis (EGPA): This was previously called Chug-Strauss. This type is characterised by the presence of eosinophils which are white blood cells that have granules which stain red in their blood. They frequently have asthma or chronic inflammation of the sinuses

Immune complex small-vessel vasculitis

In this type the inflammation occurs due to the deposition of immune complexes in the vessel walls. These complexes are a combination of antibodies and the antigens they are bound to. This type frequently affects the kidneys. The common types are:

• Anti-glomerular basement membrane disease: This type is marked by antibodies against a lining of the vessels called the basement membrane. They specifically affect the vessels in the kidneys and lungs presenting with blood in the urine and in phlegm which has been coughed up.
• IgA vasculitis (Henoch Schonlein purpura): This type presents with skin lesions, joint pains, abdominal pains and kidney involvement. This occurs from the deposition of IgA type of antibodies in the vessels supplying the skin, gut, joints and kidney.

Symptoms of Vasculitic Diseases

Due to the involvement of vessels all over the body, these conditions can present in many ways. The most common symptoms are given below

• Fever
• Skin rash
• Headache which may be associated with blurred vision
• Joint pains and muscle pains
• Cough which may be associated with blood in the phlegm
• Blood in the urine
• Abdominal Pain
• Limb weakness or numbness

Diagnosis of Vasculitis

Based on the symptoms experienced, the doctor would perform a clinical examination and order the following investigations

• Complete Blood Counts
• Kidney and Liver Function Tests
• Inflammatory Markers such as Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP)
• Urine routine and microscopy testing
• Antinuclear Antibody (ANA)
• Serum complement levels
• Anti-neutrophil cytoplasmic antibody (ANCA), with special testing of the subtypes which will help classify the vasculitides
• Chest X-ray or Chest CT in patients with cough and blood in the phlegm
• Biopsy of involved tissue: the temporal artery in Giant Cell arteritis and skin lesions if present can help with diagnosis
• Vascular Imaging: These are tests done using MRI and CT after injecting a dye into the bloodstream which helps to visualize the affected blood vessels better

Treatment of Vasculitis

The treatment of vasculitis depends on the specific type and also varies based in the area of the body which is affected. Some general modalities of treatment are as follows:

• Steroids like prednisone are the first line. This may often be enough to control the condition, but are frequently needed in high does and are associated with many side effects.
• Steroid sparing Immune suppressive medications like methotrexate, azathioprine, cyclophosphamide. These drugs are used to avoid the side effects of steroids and can frequently be used as maintenance medications
• Biologicals: These are a new type of medicines which are drugs specifically targeted at inflammatory substances in the body. These are an upcoming treatment option, but are however very expensive.

Prognosis of Vasculitis

All the vasculitides are chronic conditions whose symptoms wax and wane over time. Medications are often required lifelong to control many of them. It is important to look out for possible complications of uncontrolled disease. Increased risk of infection is present due to requirement of immunosuppressive medications for therapy.

References:

• Vasculitis | National Heart, Lung, and Blood Institute (NHLBI) [Internet]. [cited 2018 Jul 13]
• Kasper DL, et al., eds. Vasculitis syndromes. In: Harrison’s Principles of Internal Medicine. 19th ed. New York, N.Y.: McGraw-Hill Education
• American College of Rheumatology