(1) Background: Familial Mediterranean Fever (FMF) is the prototypal autoinflammatory disease, characterized by recurrent bursts of neutrophilic inflammation. (2) Methods: In this study we look at the most recent literature on this condition and integrate it with novel information on treatment resistance and compliance. (3) Results: The canonical clinical presentation of FMF is in children with self-limited episodes of fever and polyserositis, associated with severe long-term complications, such as renal amyloidosis. It has been described anecdotally since ancient times, however only recently it has been characterized more accurately. We propose an updated overview on the main aspects of pathophysiology, genetics, diagnosis and treatment of this intriguing disease. (4) Conclusions: Overall, this review presents the all the main aspects, including real life outcome of the latest recommendation on treatment resistance of FMF, a disease, that not only helped understanding the pathophysiology of the auto inflammatory process but also the functioning of the innate immune system itself.
A stringent procedure for the diagnosis of human T-lymphotropic virus (HTLV) infection was applied to 1,732 volunteer blood donors, 401 patients with various hematological disorders and 78 individuals at high risk for HIV infection. It consisted of a viral lysate-based screening assay (Abbott Laboratories, North Chicago, Ill., USA), and two confirmatory assays (Western blot and radioimmunoprecipitation assay). A confirmed positive sample had to react with at least two different HTLV gene products. Evidence of HTLV infection was not found in either blood donors or patients with hematological disorders. In fact, HTLV infection was only observed in 10 intravenous drug abusers or their sexual partners. Contrary to previous reports that claimed HTLV seroprevalences of between 0.3 and 8% in blood donors from Apulia (Italy), our data suggest that infection with this virus is principally restricted to intravenous drug abusers.
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