Vasculitis in a Nutshell
What is it and how is it diagnosed?
Vasculitis literally means inflammation of the blood vessels. It is defined by the presence of inflammatory white blood cells in the vessel walls resulting in reactive damage and loss of vessel integrity which can lead to rashes, bleeding and tissue or organ damage. Vasculitis is a rare condition and can be a primary process, or secondary to another underlying disease or autoimmune condition. Vasculitis can affect people of any age, but some types occur more in certain age groups and ethnicities. Classically, they are categorized by the predominant sizes of the blood vessels that are affected, and also by the presence or absence of antineutrophil cytoplasmic antibodies (ANCA), which can be detected with specific blood testing.
- Large vessel vasculitidies include Takayasu Arteritis and Giant Cell Arteritis (GCA), or Temporal Arteritis (which occurs in about 15% of patients with Polymyalgia Rheumatica).
- Medium sized vessel vasculitidies include Polyarteritis Nodosa (PAN), Kawasaki disease, and primary CNS Vasculitis.
- Small vessel vasculitidies include Wegener’s (now named Granulomatosis with Polyangiitis or GPA), Microscopic Polyangiitis, Churg-Strauss.
- Vasculitis can be secondary to conditions like Lupus and Rheumatoid Arthritis or occur after e exposure to medications, or infections like Hepatitis B and C, HIV, CMV, EBV and Parvovirus.
Symptoms are extremely varied, depending on the cause and which organ is involved; they can range from very mild to quite severe.
Diagnostic evaluation should consider age and gender, include a detailed history, including drug use, and infectious disease exposures. General labs should be obtained to identify which type of disorder is present and the degree of organ involvement if any.
Additional testing can be directed by a rheumatologist, and depending on pretest probability can include ANA (Anti-nuclear antibodies), complement levels, ESR, CRP, and ANCA. Other useful tests sometimes include EMG/ NCS (electromyography/nerve conduction studies), imaging including MRI/MRA or ultrasound, arteriography, and most definitively-tissue biopsy.
Treatment depends on the nature, severity and type of vasculitis, as well as what organs are affected and to what degree they are affected. For example, in mild hypersensitivity vasculitis due to a drug, discontinuing the offending drug may be adequate, along with antihistamines and observation. However, for other vasculitidies that cause pulmonary, neurologic, or renal manifestations, aggressive treatment may be required, including corticosteroids (Prednisone), and immunosuppressive therapies including Methotrexate, Azathioprine, Cyclophosphamide and Rituximab.
The goal of treatment is to induce remission, to minimize drug toxicity and to monitor for signs of recurrence.
You can find more information about vasculitis Here.
Infusion treatment medications include: