Correct!
There are multiple clinical findings that suggest vasculitis. Some are listed below:
In a patient suspected of rheumatologic vasculitis some of the laboratory tests that are useful in separating the disorders include:
The patient has ANCA-positive vasculitis (APV) which is usually separated from the other vasculitis by ANCA positivity. APV is subdivided into three diseases (2):
These are distinguished by clinical findings, ANCA test positivity, and biopsy. The different diseases have different prevalences of ANCA positivity listed below (Table 1).
Table 1. Prevalence of ANCA positivity in the ANCA-positive vasculitis-adapted from (2).
Necrotizing granulomatous vasculitis (NGV, formerly known as Wegener’s granulomatosis) is the most common ANCA-positive vasculitis that presents with respiratory tract disease. It can present in a generalized form involving lungs, kidneys and other organs or a localized form involving a single organ system. Most have glomerulonephiritis (90%). Abnormal chest imaging can consist of alveolar, interstitial, nodular, and/or cavitary patterns. Biopsy shows necrotizing vasculitis and granulomas.
In microscopic polyangitis the patients invariably have glomerulonephritis but pulmonary disease is present in 30%. Joints, skin, and nerves may also be involved. Biopsy shows necrotizing vasculitis without granulomas.
Churg-Strauss syndrome (CSS) is the rarest of the ANCA-positive disorders. Asthma and eosinophilia usually accompany CSS. In addition to the lungs the vasculitis often involves the skin, nerves, sinuses, or heart. Biopsy shows a necrotizing vasculitis and granulomas with eosinophils.
Which of the following are therapies for the ANCA-positive vasculities?