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CHaPTEr 40  Autoantibody-Mediated Phenocopies of Primary Immunodeficiency Diseases                567.e1


              M u LTIPLE-CHOICE Q u ESTIONS

           1.  High-titer, neutralizing autoantibodies against which cytokine   3.  A previously healthy HIV-negative 34-year-old woman
             is commonly seen in autoimmune polyendocrinopathy      presented with few days of fever, headache, neck pain, and
             candidiasis ectodermal dystrophy (APECED) syndrome?    vomiting. She also reported progressively increasing exertional
              A. Interferon (IFN)-α                                 dyspnea since the past 6 months. Lumbar puncture showed
              B. IFN-γ                                              increased opening pressure. Cerebrospinal fluid (CSF) analysis
              C.  Granulocyte  macrophage–colony-stimulating  factor  revealed increased white blood cell (WBC) count with lym-
               (GM-CSF)                                             phocytic predominance, low glucose, and elevated protein. The
              D. Interleukin (IL)-6                                 CSF cryptococcal antigen was positive, and the culture grew
                                                                    Cryptococcus neoformans. Brain magnetic resonance imaging
           2.  A 45-year-old female who moved from Taiwan to the United
             States at the age of 20 years presents with disseminated   (MRI) was normal. Chest computed tomography (CT) showed
             Mycobacterium abscessus with pulmonary and bone lesions   ground-glass opacities in both lungs. Bronchoalveolar lavage
             and cervical lymphadenopathy.  Autoantibodies to which   (BAL) revealed periodic acid-Schiff (PAS)–positive material
             cytokine may be associated with her disease?           on cytology, consistent with a diagnosis of pulmonary alveolar
              A. IL-17                                              proteinosis (PAP). Autoantibodies against which cytokine may
              B. IL-22                                              be associated with her manifestations?
              C. IFN-α                                               A. GM-CSF
              D. IFN-γ                                               B. IL-6
                                                                     C. G-CSF
                                                                     D. Erythropoietin
                                                                     E.  IFN-γ
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