Vasculitis

Vasculitis encompasses a set of disorders characterized by the destructive inflammation of blood vessels. This inflammation affects both the arteries and veins of the circulatory system. In some contexts, Lymphangitis, which involves inflammation of the lymph vessels, is also considered a form of vasculitis. The primary cause of vasculitis is the migration of leukocytes or white blood cells, which leads to subsequent damage to the vessel walls.

While inflammation of veins (phlebitis) or arteries (arteritis) can occur in vasculitis, these conditions are distinct entities when they occur independently of vasculitis.

Signs and symptoms

The symptoms of vasculitis can vary widely, depending on the specific blood vessels involved. Some possible symptoms include:

General symptoms: These could include fever and weight loss. Skin: Visible purpura (small blood vessels that burst to form red or purple spots on the skin) or livedo reticularis (a condition that causes a purplish discoloration of the skin) may be present. Muscles and joints: Myalgia (muscle pain) or myositis (muscle inflammation), arthralgia (joint pain) or arthritis (joint inflammation) could be experienced. Nervous system: Symptoms can include Mononeuritis multiplex (damage to multiple individual nerve fibers), headache, stroke, tinnitus (ringing in the ears), reduced visual acuity (sharpness of vision), or acute visual loss. Heart and arteries: Myocardial infarction (heart attack), hypertension (high blood pressure), and gangrene (tissue death due to lack of blood supply) could occur. Respiratory tract: Nose bleeds, bloody cough, and lung infiltrates may be present. Gastrointestinal (GI) tract: Abdominal pain, bloody stool, and perforations can occur. Kidneys: Glomerulonephritis (a type of kidney disease) can occur.

Cause

Classification

Vasculitis can be classified based on its cause, the location of the inflammation, the type of blood vessel involved,

granulomatosis with polyangiitis (Churg-Strauss)	Perinuclear antineutrophil cytoplasmic antibody	Myeloperoxidase	High Eosinophil count, elevated CRP
Henoch-Schönlein purpura	None	None	Increased IgA
Medium and large vessel vasculitis
Polyarteritis nodosa	Hepatitis B surface antigen and Hepatitis B surface antibody	Hepatitis B virus	Elevated ESR, anemia, leukocytosis, increased serum IgA, C-ANCA
Kawasaki disease	None	None	Elevated ESR, leukocytosis, thrombocytosis, increased serum IgA and IgG, sterile pyuria, CSF pleocytosis
Takayasu arteritis	None	None	Elevated ESR, anemia
Giant cell (temporal) arteritis	None	None	Elevated ESR, anemia

Treatment

The primary treatment is with corticosteroids and, in some cases, immunosuppressant drugs. For some forms of vasculitis, treatment may include plasmapheresis (removal and reinfusion of the patient's own blood after treatment to remove antibodies), or intravenous immunoglobulin (IVIG).

In some cases, the underlying cause of the vasculitis may be treatable, as in the case of hepatitis C-induced vasculitis. In these cases, treatment of the underlying cause may lead to a resolution of the vasculitis.

Corticosteroids are the most common treatment for vasculitis, and these are often used in combination with other immunosuppressive drugs. In general, the goal is to reduce inflammation in the affected blood vessels, and to suppress the immune response causing the disease.

While the treatment of vasculitis has significantly improved over the last 50 years, the disease can cause serious complications, and it is therefore considered a serious condition.

The specific treatment plan depends on the type and severity of vasculitis, and the organs involved. For instance, in large vessel vasculitis such as Takayasu's arteritis, treatment is usually a combination of corticosteroids and immunosuppressive drugs. In contrast, small vessel vasculitis such as Henoch-Schonlein purpura may resolve on its own without treatment, though corticosteroids can be used to alleviate symptoms.

Prognosis

The prognosis for vasculitis varies widely depending on the type of vasculitis, the severity of the condition, and the overall health of the patient. In general, early detection and treatment of vasculitis can significantly improve the prognosis.

For some forms of vasculitis, such as Henoch-Schönlein purpura in children, the prognosis is generally good, with most children recovering without any serious long-term complications.

However, for other forms of vasculitis, such as Wegener's granulomatosis (now known as Granulomatosis with polyangiitis) and Microscopic polyangiitis, the prognosis can be more serious. Without treatment, these conditions can be life-threatening. Even with treatment, there can be serious complications, including kidney failure and other organ damage.

In some cases, vasculitis can become a chronic condition, requiring long-term management with medications to control inflammation and prevent flare-ups of the disease.

The prognosis can also be influenced by the presence of other underlying health conditions, such as hepatitis B in the case of Polyarteritis nodosa, which can make the vasculitis more difficult to treat.

In general, it's important for individuals with vasculitis to work closely with their healthcare provider to manage their condition and monitor for potential complications.

Epidemiology

The incidence and prevalence of vasculitis varies widely depending on the specific type of vasculitis. Some forms of vasculitis, like Giant Cell Arteritis, are relatively common, especially in older adults. Other forms, like Kawasaki disease, are more common in children. Some forms of vasculitis, like Takayasu arteritis, are much rarer.

The incidence of vasculitis also varies by geographic region and ethnicity. For example, Giant Cell Arteritis primarily affects people of Northern European descent, and is rare in people of Asian, African, and Hispanic descent. Kawasaki disease, on the other hand, is much more common in people of Asian descent, particularly in Japan.

Overall, vasculitis is not a common condition, but it can be serious and potentially life-threatening, so early detection and treatment is crucial.

External links

• Vasculitis Foundation
• National Heart, Lung, and Blood Institute: Vasculitis

External links

Classification	ICD-10: I77.6, I80, L95, M30-M31 ICD-9-CM: 446, 447.6 MeSH: D014657 DiseasesDB: 13750

Vasculitis Resources
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