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INTRODUCTION

Key Clinical Questions

  • image When should a diagnosis of vasculitis be considered?

  • image How is a diagnosis of vasculitis made or excluded?

  • image What are the appropriate strategies for triage, consultation, and follow-up for vasculitis?

  • image What treatment and staging for vasculitis should be initiated in the hospital?

  • image In a patient with established vasculitis, how should new symptoms of illness be evaluated?

The diagnosis of vasculitis is considered far more often than these diseases are actually seen—appropriately, since these rare diseases have diverse presentations, are dangerous and often rapidly progressive, yet are treatable. All vasculitides feature inflammation of the walls of blood vessels, with symptoms and findings attributable to the size(s) and locations of the involved vessels. Vessel size, epidemiology, and/or a constellation of typical clinical features have proved useful for classifying the vasculitides, with key points summarized in (Table 257-1).

TABLE 257-1Classification of the Major Primary Vasculitides, with Some Key Features (Nonexhaustive List)

The classification of vasculitis remains imprecise with more than one system in use. “Primary” systemic vasculitides are further sorted by the sizes of the predominant arteries involved, leading to sets of small-, medium-, and large-vessel vasculitides. There are also several “secondary” forms of vasculitis associated with infections, drug exposure, or another form of systemic disease such as systemic lupus erythematosis or rheumatoid arthritis.

All of the primary, idiopathic vasculitides are rare diseases, each categorized as an “orphan” disease in the United States (prevalence of <200,000 ...

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