Update in the Diagnosis and Management of Pulmonary Vasculitis: Summary
Gayraud et al found similar results with infectious complications representing 12.9% of deaths among a sample of treated vasculitis patients. Likewise, drug toxicity is a relatively frequent and clinically important problem. In a study by Reinhold-Keller et al, in which WG patients were treated with cyclophosphamide, there was a 12% incidence of cystitis, an 8% incidence of myelodysplastic syndrome, and a 5% incidence of solid malignancy.
Approximately 50% of patients with ANCA-asso-ciated vasculitis will experience one or more disease flares despite receiving therapy, with relapse being more common among patients with WG (40 to 65%) and less common among patients with CSS (15 to 25%.) Click Here
Such flares may present with clinical manifestations similar to those seen at the time of the original diagnosis or with new signs and symptoms, or the involvement of previously unaffected organs. In general, disease relapse requires reinduction therapy with an escalation of a patient’s immunosuppressive regimen. While disease relapse remains a clinical diagnosis, the WGET study group recently published a study applying a serum proteomic approach to gauging the state of remission in patients with WG, raising the hope that sensitive serum markers may one day permit accurate discrimination between quiescent and active disease.
Considerable advances have been made in the management of and therapy for the small-vessel vasculitides. The recent completion of a number of critical, randomized, controlled trials has led to evidence-based recommendations regarding the management of these devastating diseases. The introduction of novel therapeutic approaches, combined with increased clinical awareness of the complex and competing considerations in the management of these patients, allows us to anticipate further improvements in the outcomes of patients with these severe diseases.