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HDM Exam 1 and 2 > Diseases to Remember > Flashcards

Diseases to Remember Flashcards

1
Q

Leprosy

A

Clinical Features: Cutaneous lesions, neuropathic changes and deformities

M. Leprae colonizes macrophages and other host cells and multiplies within
Macrophages need to be activated in order to kill it
Grows best at 86 degrees, so likes to hang out at joints

IFN-y can help

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2
Q

Mycobacteria

A

Evades the immune system by preventing the fusion go phagosome to lysosome, prevents degradation

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3
Q

Herpes Simplex Virus (HSV)

A

Evades immune system by reducing the function of TAP

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4
Q

Cytomegalovirus (CMV)

A

Evades immune system by preventing proteasomal activity and preventing Class 1 MHC from pinching off ER

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5
Q

Epstein Barr Virus (EBV)

A

Evades immune system by prevent proteasomal activity and inhibiting macrophage activity by releasing IL-10

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6
Q

Pox virus

A

Evades immune system by releasing soluble CXCRs

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7
Q

Toxic Shock Syndrome

A

Associated with localized S. aureus (gram +) infection, food poisoning, local colonization
Tampons provide a good food source
Toxic Shock Protein = super antigen that binds to B chain of TCR and does not require antigen or co-stimulatory receptors to process response

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8
Q

What are the consequences of super antigen?

A

Fever, rash, edema, hypotension, shock

TNF-a and IL-1 contribute to increase in vascular permeability that lead to leakage of fluid

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9
Q

Hyper IgM Syndrome

A 
5 types --> inability to class switch 
X-linked = defect in CD40L, autosomal = defect in CD40
X-linked --> probs with T-cell activation, suffers from opportunistic infections 
Syndrome with defect in AID --> probs with B-cells
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10
Q

C3 Deficiencies (Alternative pathway)

A

Life threatening, severe, recurrent infections

Deficiencies in Factor H and I mimic C3 deficiencies, because exhausts C3 from system
Pyogenic infections, SLE

Alternative pathway - Factor D, Properdin Def = Nisserial

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11
Q

MAC deficiencies

A

Generally healthy

Increase in infection by Nisseria gonorrhea and N. meningitis

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12
Q

Deficiencies in early complement activation (Classical)

A

C1, C2, C4 –> C2 most common
With C2 def. pts have high degrees of SLE
May arise from failure to clean immune complexes which deposit on blood vessel walls and tissues
Produces local inflammation –> may lead to breakdown of tolerance to self –> autoimmunity

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13
Q

C1inh Deficiency and Hereditary Angioneurotic Edema (HAE)

A

C1 not regulated properly –> C4/2 chronically low

Hereditary Angioneurotic Edema (HAE)
Pt have attacks with swelling involving extremities, GI tract, and Face
may affect upper respiratory tract leading to suffocation
Tx: Berinet, purifies human C1inh

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14
Q

Paroxysomal Nocturnal Hemoglobinuria (PHN)

A

Hemolytic disorder with spontaneous episodes of complement-mediated RBC lysis (also leks and platelets)
Tx: erythropoietin, corticosteroids, eculizumab

Defect in phosphatidylinositol glycan class A gene (PIG-A)
It is required for synthesis of glycosyl phosphatidylinositol (GPI) anchor
Lack of CD59 and CD55 complement reg proteins
Leads to complement mediated intravascular hemolysis

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15
Q

Class 1 HLA gene (HLA-B27) increases risk of…

A

Ankylosing Spondylitis (by 90)

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16
Q

Class 2 HLA genes (HLA-DR4) increase risk of…

A

Type 1 DM (25), Pemphigus vulgaris (14), RA (4)

17
Q

Describe PTPN22 role in autoimmunity.

A

Abnormal TYROSINE PHOSPHATASE reg of T-cell selection and activation

Disease association: RA, T1D, autoimmune thyroid diseas

18
Q

Describe CTLA4 role in autoimmunity.

A

Impaired inhibitory checkpoint and Treg function - loss of B-cell, T-cell function

Diseases: T1D, RA, many autoimmune diseases

19
Q

Describe AIRE role in autoimmunity.

A

Reduced expression of peripheral tissue antigens in thymus, leading to defective elimination of self-reactive T - necessary for thymic expression of self-proteins

Diseases: Autoimmune Polyendocrine syndrome (APS-1), DM, adrenal, parathyroid

20
Q

Describe FAS role in autoimmunity.

A

Defective apoptosis of self-reactive T and B cells in periphery - splenomegaly, lymphadenopathy

Disease: Autoimmune lymphoproliferative syndrome (ALPS), adrenals

21
Q

Describe FOXP3 role in autoimmunity.

A

Deficiency of T-regs

Disease: Immune dysregulation X-linked polyendocrinopathy and enteropathy - IPEX - widespread autoimmunity

22
Q

Infection - Disease association to remember

A

Streptococcus - Rheumatic Fever
Chlamydia - Reiter’s Syndrome (HLA-B27)
Shigella, Salmonella - Reactive Arthritis (HLA-B27)
Coxsackie, echovirus, rubella - IDDM (HLA-DQ..)

23
Q

SLE

A

Polytrophic Immune Complex Disease
Defective apoptotic process leading to increased nucleic acid Ags available to stimulate immune response
Susceptible gene + UV radiation

24
Q

RA

A

Inflammation in synovium
Th1/17 cytokine release sustains inflammation
Ab production against rheumatoid factor (Fc portion of Ig)
Defective clearance of immune complexes; failure of B cell tolerance

25
Q

Immunodeficiencies associated with lymphocyte MATURATION of BOTH Ts and Bs

A

X-linked SCID
ADA and PNP Def
JAK3 Def
RAG1/2 Def

26
Q

Immunodeficiencies associated with lymphocyte MATURATION of B-CELLS

A

X-linked Agammaglobulemia

Ig Heavy chain def

27
Q

Immunodeficiencies associated with lymphocyte MATURATION of T-CELLS

A

Di-George Syndrome

28
Q

Immunodeficiencies associated with lymphocyte ACTIVATION and FUNCTION of BOTH Ts and Bs

A

X-linked lymphoproliferative syndrome

29
Q

Immunodeficiencies associated with lymphocyte ACTIVATION and FUNCTION of B-CELLS

A

X-linked Hyper IgM syndrome
Common Variable Immunodeficiency
IgA Def
Isolated IgG subclass def

30
Q

Immunodeficiencies associated with lymphocyte ACTIVATION and FUNCTION of T-CELLS

A

Bare Lymphocyte Syndrome
MHC Class 1 Def
Wiskott-Aldrich syndrome
IPEX

31
Q

Immunodeficiencies in Innate Immunity

A 
Chediak-Higashi disease 
Leukocyte Adhesion disease 
Chronic Granulomatous disease
G6PD Def
Myeloperoxidase def
Severe Chronic Neutropenia
32
Q

Characteristics of Complement Deficiencies

A

Defects in Classical more common - Pt have high number of autoimmune disorder, including LUPUS-like syndrome

Defects in Alternative - present with Nisserial infections

33
Q

Type 1 Hypersensitivity Diseases

A

Anaphylaxis - Tx with Epi
Bronchial Asthma - Tx with corticosteroids, leukotriene antagonists, phosphodiesterase inhibitors
Allergies - Tx with desensitization, Anti-IgE, Antihistamine

34
Q

Type 2 Hypersensitivity Diseases

A 
Autoimmune Hemolytic anemia
Autoimmune thrombocytopenia purpura
Good pasture syndrome - C'/Fc receptor-mediated inflam
Graves disease
Myasthenia graves
Pemphigus Vulgaris
Pernicious anemia
Rheumatic Fever
35
Q

Type 3 Hypersensitivity Diseases

A 
SLE
Polyartertitis nodosa
Poststreptococcal glomerulonephritis
Serum sickness
Arthus reaction
36
Q

Type 4 Hypersensitivity Diseases

A 
RA 
Multiple Sclerosis
Type 1 DM
Crohn's
Contact sensitivity
Chronic infection
Viral Hepatitis
Superag mediated diseases

HDM Exam 1 and 2 (7 decks)

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