Vasculitis is a term that refers to a group of conditions that all lead to inflammation and necrosis of a blood vessel with subsequent impairment of blood flow. The damage to the structure of the blood vessel leads to an inability to deliver oxygen and nutrients to various tissues resulting in eventual cell injury and death via thrombosis and/or ischemia. In general, the affected vessels vary in size, type and location. Clinically, the consequences of untreated vasculitis depend on the size of blood vessels involved, leading to the damage of various vital organs like the skin, kidneys, lungs and brain.
Classification
There are multiple distinct forms of systemic vasculitis, all of which are quite uncommon. Overall, they typically present with multi-system symptoms or nonspecific clinical findings. The different forms can be grouped based on the size of the affected blood vessels:
Type of Vasculitis Based on Size of Blood Vessel (link)
Large-vessel vasculitis
- Giant cell (temporal) arteritis (GCA)
- Takayasu’s arteritis
Medium-vessel vasculitis
- Polyarteritis nodosa (PAN)
- Kawasaki’s disease
Small-vessel vasculitis
ANCA Vasculitis
- Granulomatosis with Polyangiitis (GPA)
- Microscopic Polyangiitis (MPA)
- Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Other Small Vessel Vasculitis
- IgA Vasculitis (also known as Henoch-Schonlein Purpura, or HSP)
- Cryoglobulinemic Vasculitis
- Hypocomplementemic Urticarial Vasculitis
- Anti-GBM Disease
Variable-vessel vasculitis (can involve any size blood vessel)
- Behçet’s disease
Clinical Presentation & Diagnosis
There is no single typical presentation. Vasculitis may present as a rash, headache, joint pain, hand or foot weakness, or nonspecific 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 these symptoms occur in a young person, it is especially concerning and warrants evaluation.
The diagnostic workup should encompass tests to evaluate for suspected vasculitis. These may include blood 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 be of benefit can also include imaging of the chest and/or sinuses, nerve conduction studies, echocardiography, angiography, and ultimately a biopsy of the affected tissue. Not all these tests may be needed on every patient, and each workup is individually tailored to the symptoms and the type of suspected vasculitis.
Treatment
The general approach to the treatment of vasculitis includes identifying and removing any inciting agents, like medications. Additionally, it is essential to address the underlying disease associated with the vasculitis, such as infection or cancer, if there is one present. Treatment also often involves anti-inflammatory and potent immunosuppressive medications, such as steroids or rituximab. Vasculitis often requires prolonged treatment courses, so it is important to be aware of complications related to therapy and address these as well. These complications can include infection, osteoporosis, and an increased risk of heart disease.
Overall, these are serious conditions that need prompt evaluation and treatment by a rheumatologist.
