Treatment starts with an induction phase to prevent organ damage and control inflammation using cyclophosphamide, rituximab or methotrexate. Maintenance therapy’s goals are to prevent relapse and improve quality of life with methotrexate, azathioprine, rituximab and, as a second-line therapy, mycophenolate. Patients will taper glucocorticoids in the background in addition to their disease-modifying therapies. Remission is no active disease, although it can be hard to spot if there is tissue damage, he said.
Small-vessel vasculitis may not produce noticeable symptoms for years, said Alexandra Villa-Forte, MD, MPH, director of the Center for Vasculitis Care and Research at the Cleveland Clinic. They may present with a palpable, purpuric rash, usually on the legs. Biopsy on a new rash or the periphery of the rash is preferable.
Secondary Disease
When vasculitis is secondary, the underlying cause may be difficult to identify and treat, she said. Secondary vasculitis may be caused by viral infections, such as polyarteritis nodosa from hepatitis B and CV from hepatitis C (HCV), as well as vasculitis caused by cancer, systemic diseases or as a medication side effect.8 Rashes may be mistaken for allergic reactions until biopsy shows vasculitis signs, she said.
“Diagnosis is difficult in the early stages,” said Dr. Villa-Forte. Multisystem disease is one clue to suspect secondary vasculitis, especially kidney, polyneuropathic, skin or musculoskeletal involvement. “The onset of these diseases is usually subacute,” she added. “Usually, your patient doesn’t pay attention to the preceding symptoms, so you have to ask questions.”
Symptoms of secondary vasculitis may be frequent, but nonspecific and constitutional, such as fevers, weight loss or malaise, as well as arthralgia and myalgia, she said. Patients may have these symptoms for months before they seek attention.
‘There’s a lot of overlap in terms of the sizes of blood vessels involved in these diseases. Some forms of vasculitis don’t follow the rules.’ —Dr. Springer
Although not a gold standard for secondary vasculitis diagnosis, biopsy may help diagnosis, ideally on the edges of a skin lesion within 48 hours of its appearance, said Dr. Villa-Forte. It may be hard to find an accessible place to biopsy if patients have skin lesions.
“Biopsy may show us inflammation of blood vessels, but not give us any clues as to the type of vasculitis we are dealing with,” she said. Laboratory testing should be based on clinical suspicions, but do not delay diagnosis because a patient’s creatinine results are normal, she said.
Where Rheumatologists Come In
When rheumatologists see patients with CV, successful treatment of the HCV infection may get rid of the vasculitis, said Dr. Villa-Forte. Patients with severe vasculitis may need prednisone with or without rituximab, or plasmapheresis if organs or life are threatened, she said.
