Journal de vascularite

Systemic AA amyloidosis is a relatively rare complication of chronic inflammatory disorders, e.g. rheumatoid arthritis, ankylosing spondylitis, familial Mediterranean fever and other autoinflammatory syndromes, Crohn’s disease, malignancies and recurrent infections. Isolated case of AA amyloidosis have been reported in many other chronic diseases, including systemic vasculitides, diffuse connective tissue diseases, sarcoidosis, chronic hepatitis, etc. In the past ten years the incidence of AA amyloidosis decreased significantly due to earlier and more aggressive treatment of underlying conditions such as rheumatoid arthritis or infections. In the recent series of 474 patients with renal amyloidosis from Mayo clinic there were only 7% cases of AA amyloidosis while 86% patients presented with AL form of disease. Nevertheless, AA amyloidosis remains a life-threatening complication of immune-mediated and inflammatory conditions and deserves effective prevention and treatment. Takayasu’s arteritis (TAK) is a rare largevessel vasculitis of unknown etiology that more frequently affects women of childbearing age and is defined as "granulomatous inflammation of the aorta and its major branches" by the revised Chapel Hill Consensus Conference. The search in Pubmed database revealed only a dozen reports of AA amyloidosis in patients with TAK. The objective of our study was to evaluate the incidence of AA amyloidosis in a cohort of consecutive TAK patients.

There are several vascultic disorders still labeled as difficult-to-treat cases. Effective treatment for those patients is warranted to reduce the mortality and morbidity that resulting from these disorders. An extensive review for the literatures that addressed using of ant TNF-alpha in several vascultic disorders was conducted. Use of anti TNFalpha agents is a promising modality in several vascultic disorders. Despite lacking well-conducted randomized controlled trials, more open-label studies are required to examine in-depth the safety and efficacy of those agents.