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CHaPTEr 60  Systemic Autoinflammatory Syndromes                834.e1


              MULTIPLE-CHOICE QUESTIONS

           1.  Autoinflammatory diseases are characterized by:    3.  What is the treatment of first choice for FMF?
              A. Mutations in the NLRP3 inflammasome                 A. Acetaminophen
              B. Spontaneous recurrent or persistent inflammation    B. Colchicine
              C. The presence of autoantibodies                      C. Interleukin (IL)-1 inhibition
                                                                     D. Tonsillectomy
           2.  What is the MOST common monogenic autoinflammatory
             disease worldwide?                                   4.  What is the MOST common long-term complication of the
              A. Cryopyrin-associated periodic syndrome (CAPS)      monogenic autoinflammatory diseases?
              B. Familial Mediterranean fever (FMF)                  A. Amyloid A (AA) amyloidosis
              C. Mevalonate kinase deficiency (MKD)                  B. Chronic meningitis
              D. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis    C. Joint contractures
               syndrome (PFAPA)                                      D. Malignancy
              E.  Schnitzler syndrome
              F.  Tumor necrosis factor receptor–associated periodic syn-
               drome (TRAPS)
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