Large-vessel vasculitis

The vasculitides affecting large and medium-sized vessels are heterogeneous. This group includes such disorders as giant cell arteritis, Takayasu's disease, and sarcoidosis. There are several challenges that may arise in the care of patients with these disorders. Diagnosis may be elusive when initially evaluating patients with large-vessel vasculitis. Most serologic markers are not specific, and tissue biopsy is often impractical. We will present data emphasizing the most common disease manifestations, to aid in recognition of a clinical picture suggestive of large-vessel vasculitis. We will also focus on the more common pulmonary manifestations and minimize case reports, which may not reflect the usual course of disease. Large cohort studies and randomized controlled trials to provide an evidence-based approach to therapy in these disorders are uncommon. We have included in this review discussion of those studies that we judged to be most rigorously conducted and clinically relevant. We provide guidelines for initial therapy, with caveats to assist in minimizing potential treatment-related complications and toxicities. Although monitoring of disease activity is often difficult, this is a most crucial element in minimizing disease and treatment-associated morbidity and mortality.