This report describes the case of a 29-day-old infant with Kawasaki's disease who presented in extremis with multiple coronary aneurysms and coronary thromboses, myocardial ischemia, and congestive heart failure. The infant successfully underwent emergent coronary thrombolysis with tissue plasminogen activator.
A 64-year-old Egyptian man who resides in the United States presented to the rheumatology clinic with 6 months history of episodic recurrent pauci--arthritis along with constitutional symptoms. His Mediterranean ancestry, anti-cyclic citrullinated peptide negativity, and cyclical palindromic rheumatism prompted an investigation for familial Mediterranean fever gene mutation. He was found to have heterozygous 694I gene mutation during MEFV analysis. He also met Liveneh 1 major and 1 minor criteria for the diagnosis of familial Mediterranean fever.
In the past several years, significant progress has occurred in the therapeutic management of antineutrophil cytoplasmic antibody-associated vasculitis. This has resulted in improved survival rates for all members of this group of inflammatory vascular disorders. However, the rate of treatment failure or resistance and relapse has remained relatively constant despite therapeutic advances. This article reviews risk factors associated with these failures, with hopes that better understanding may lead to improved therapeutic responses and less therapy-related toxicity.
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