Which of the following is (are) Heredofamilial amyloidosis –
Correct Answer: Familial mediterranean fever
Description: A variety of familial forms of amyloidosis have been described; most are rare and occur in limited geographic areas. The best-characterized is an autosomal recessive condition called familial Mediterranean fever. This is a febrile disorder characterized by attacks of fever accompanied by inflammation of serosal surfaces, including peritoneum, pleura, and synol membrane. This disorder is encountered largely in persons of Armenian, Sephardic Jewish, and Arabic origins. It is associated with widespread tissue involvement indistinguishable from reactive systemic amyloidosis. The amyloid fibril proteins are made up of AA proteins, suggesting that this form of amyloidosis is related to the recurrent bouts of inflammation that characterize this disease. The gene for familial Mediterranean fever is called pyrin and encodes a protein that is a component of the inflammasome (Chapter 2). Patients have gain-of-function mutations in pyrin that result in constitutive overproduction of the proinflammatory cytokine IL-1 and persistent inflammation Robbins 9 th edition page no. 155
Category:
Pathology
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