Dr Himanshu Aggarwal

What is Systemic vasculitis? 

Systemic vasculitis also known as systemic vasculitides, refers to a group of rare autoimmune disorders characterized by inflammation of the blood vessels (vasculitis) throughout the body. In these conditions, the immune system mistakenly attacks the blood vessels, causing inflammation, damage, and a wide range of symptoms, which can affect various organ systems. Systemic vasculitis can be a serious and potentially life-threatening condition if not properly managed.

There are multiple types of systemic vasculitis, each named based on the size and type of blood vessels primarily affected. Some common types of systemic vasculitis include:

1. Giant Cell Arteritis (GCA): GCA primarily affects large and medium-sized arteries, often in the head, neck, and upper body. It is most commonly seen in older adults and can lead to complications such as blindness if not treated promptly.

2. Takayasu Arteritis: This type primarily affects the aorta and its major branches, which can lead to narrowing and reduced blood flow to various organs.

3. Granulomatosis with Polyangiitis (GPA, formerly Wegener’s granulomatosis): GPA typically involves small to medium-sized blood vessels in the respiratory tract and kidneys. It can cause respiratory problems, kidney damage, and other systemic symptoms.

4. Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss syndrome): EGPA often affects small to medium-sized blood vessels and is associated with elevated levels of eosinophils in the blood. It can affect the respiratory system, skin, nerves, and other organs.

5. Microscopic Polyangiitis (MPA): MPA primarily affects small blood vessels in the kidneys, lungs, and skin. It can lead to glomerulonephritis (kidney inflammation) and pulmonary hemorrhage.

6. Polyarteritis Nodosa (PAN): PAN typically affects medium to large-sized arteries and can lead to damage in various organs, including the skin, nerves, and gastrointestinal tract.

The exact cause of systemic vasculitis is not well understood, but it is generally believed to result from an abnormal immune response. Genetic and environmental factors may also play a role in the development of these conditions.

Diagnosis of systemic vasculitis often involves a combination of clinical evaluation, blood tests to detect inflammation markers and specific antibodies, imaging studies, and sometimes a biopsy of affected tissue. Early diagnosis and treatment are crucial to manage symptoms and prevent complications.

Treatment typically involves medications to suppress the immune system’s overactive response and reduce inflammation. The choice of treatment depends on the specific type of vasculitis and its severity. In some cases, long-term or even lifelong treatment may be necessary to control the disease and prevent flare-ups. Healthcare providers with expertise in rheumatology or immunology usually manage the care of individuals with systemic vasculitis.

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