Science Quiz / Immunodeficiencies & Autoimmunities

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Can you name the immunodeficiency or autoimmunity?

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

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