Vasculitis is inflammation and necrosis of a blood vessel with subsequent impairment of flow. It is a multi-system inflammatory disease. Vessel wall destruction can lead to perforation and hemorrhage into adjacent tissues with subsequent endothelial injury leading to thrombosis and ischemia/infarction of dependent tissues.

 

The damage to the structure of the blood vessel leads to an inability to deliver oxygen and nutrients to various tissues resulting in the tissues eventual death. In general, affected vessels vary in size, type and location. The consequences are damage to various vital organs in the body like the skin, kidneys, lungs and brain.

 

There are multiple distinct forms of systemic vasculitis. Each is uncommon. As a whole they usually present with multi-system symptoms or findings that are nonspecific. The diseases can be grouped by the size of the vessel they affect:

 

Large-vessel vasculitis

• Giant cell (temporal) arteritis

• Takayasu’s arteritis

 

Medium-vessel vasculitis

• Polyarteritis nodosa

• Kawasaki’s disease

 

Small-vessel vasculitis

• Wegener’s granulomatosis

• Churg-Strauss syndrome

• Microscopic polyangiitis

• Henoch-Schonlein purpura

• Essential cryoglobulinemic vasculitis

• Cutaneous leukocytoclastic angiitis

 

There is no single presentation typical of vasculitis. Vasculitis may present as a rash, headache, foot-drop, or vague constitutional symptoms like abdominal pain, nausea, fever, and weight loss. It can also present as a major event such as a stroke, bowel infarction or pulmonary hemorrhage. If any or all of these symptoms occur in a young person it is especially likely.

 

The diagnostic workup should encompass tests to evaluate for suspected vasculitis. These may include tests to assess for inflammation, organ involvement, immune complex formation and deposition, and ANCA-related vasculitis. Also tests to assess for concomitant infections might also be undertaken. Diagnostic studies that may aid in diagnosis include chest x-ray, sinus x-ray, CT scan, nerve conduction studies, echocardiography, angiography and finally a tissue biopsy. Not all these tests may be needed on every patient suspected of vasculitis. Each workup is individually tailored.

 

The general approach to treatment of vasculitis includes identifying and removing any inciting agents, like medications. Treat the primary underlying disease associated with the vasculitis. Initiate anti-inflammatory and immunosuppressive therapy. Prevent complications, like infections, osteoporosis and atherosclerosis by controlling blood pressure and lipids.

Overall, these are serious conditions that need prompt evaluation and treatment by a rheumatologist.