Science Quiz / Immunodeficiencies & Autoimmunities

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Can you name the immunodeficiency or autoimmunity?

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Cytokine that activates Ig class switching to non-cytolytic IgG2 and IgG4
Receptor for iC3b on macrophages
Deficiency in this can result in hereditary angioneurotic edema
Characteristic clinical presentation of SCID
Most common gene mutation in CVID
Deficiency in this can result in paroxysmal nocturnal hemoglobinuria
IPEX (immune dysregulation polyendocrinopathy enteropathy x-linked disease) is caused by loss of what?
Immunodeficiency in which phagocytosis is impaired
What antibody is useful for measuring disease activity of lupus?
Deficiency of this enzyme leads to accumulation of toxic purine metabolites in cells that are actively synthesizing DNA
Main steroidal treatment of lupus
Signaling subunit that undergoes a mutation in X-linked SCID
Gene that permits receptor editing in peripheral tissues
G-protein coupled receptor on T cells, B cells, and hematopoietic stem cells for SDF-1/CXCL-12; important co-receptor for HIV in late infection
Important distinguishing feature of malar rash in lupus (spares what?)
Cytokine that can induce promiscuous killing by CTLs, activate killing by NK cells, and overcome T cell anergy
Chief cytokine for proliferation of lymphocytes, deficiency in this cytokine strongly resembles SCID
Antibody associated with spontaneous abortions in lupus
Rapid onset of wrist drop or foot drop is a nervous system manifestation characteristic of what?
C1 INH deficiency results in
Ligand on T cells for its costimulatory receptor on B cells and dendritic cells
Most important gene in current research for lupus
What antibody is very SPECIFIC for lupus?
HintAnswerExtra
Cytokine secreted by Th1 cells and NK cells, growth factor for T cells and B cells
HIV envelope glycoprotein that binds to CD4 co-receptor on class II MHC-restricted T cells
Patients that are homozygous deficient in this receptor are virtually immune to HIV
What bacterial infection can recur in patients with deficiencies in late-acting complement components?
Cytokine produced by basophils and Th2 cells
Cytokine in psoriasis that drives keratinocyte hyperplasia and secretion of IL-17
Possible infectious cause of lupus in children
Mutated enzyme in X-linked agammaglobulinemia
Innate receptor that may be responsible for mechanism of emphysema
Patients with active hepatitis B might develop what type of vasculitis?
Cytokine in psoriasis that causes T cells to differentiate into Th17 and Th22 cells
Irreversible blindness can occur in what type of vasculitis?
Anti-phospholipid syndrome, or an autoimmune hypercoagulative state, is characterized by what?
Immunodeficiency that often does not present until the patient is in his/her 20s or 30s
Gene that mediates VDJ recombination of TCR and BCR
Defective enzyme in chronic granulomatous disease
What therapy clears bullous pemphigus?
Patients with drug-induced lupus usually test positive for what?
DAF deficiency can result in
Hormone or hormone derivative that stimulates thymocytes, lymphocytes, cytokines, and VCAM/ICAM
Rapid onset dypsnea is characteristic of what type of vasculitis?
Enzyme that undergoes mutation in autosomal SCID
Mutations in this ligand cause X-linked hyper-IgM syndrome
HintAnswerExtra
Most common skin manifestation of lupus
Most common treatment for XLA
Patients lacking this enzyme cannot make either T cells or B cells
Component of receptors for IL-2, IL-4, IL-7, IL-9, and IL-15
Major co-receptor for HIV found on T cells and macrophages, initial entry point
T cell deficiency due to congenital malformation resulting in defective development of the thymus, PT glands, and the heart
CD4+ T cell count in which a patient infected with HIV is considered to be suffering from AIDS
Neutrophils release what in response to carbon black?
Chimeric anti-CD20 monoclonal antibody indicated for lupus
Immunodeficiency characterized by recurrent intracellular bacterial and fungal infections
Deficiency in this receptor results in macrophages being unable to control mycobacterial infections
Substance that activates neutrophils and macrophages in emphysema
Absent molecule in leukocyte adhesion deficiency
Most common clinical presentation of XLA
Hormone or hormone derivative that reduces apoptosis in self-reactive B cells
Biologic therapy for lupus Belimumab stops B cell activity by inhibiting what?
Cleavage product of iC3b that binds CD21 on B cells to help stimulate them
What cytokine might prime endothelial antigens to be exposed, bringing in anti-neutrophil cytoplasmic antibodies in vasculitis?
20 yo Asian female presents with weight loss, fever, one arm 10mmHg in BP less than the other, diastolic murmur in aortic area. What is the tx?
Activation of this receptor co-stimulates and upregulates AID, responsible for Ig class switching and affinity maturation
What antibody is very SENSITIVE for lupus?
Watery diarrhea & failure to thrive, dermatitis and infant-onset IDDM are typical features of what immunodeficiency?

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