Immune System, TBP Flashcards
Hypersensitivity reactions, type: IgE mediated
Type I
Hypersensitivity reactions: TH2 cells release this substance that stimulates eosinophils
IL-5
Hypersensitivity reactions: TH2 cells release this substance that activates IgE-producing b-cells
IL-4
Hypersensitivity reactions: IgE binds to what cells
Mast cells
Hypersensitivity reactions: Binding of antigen to IgE on mast cells result in
Mast cell degranulation and release of mediators
Type I hypersensitivity: Phases
Early and late
Type I hypersensitivity: Early phase occurs within
5-30 minutes
Type I hypersensitivity: Early phase is due to
Binding of antigen to IgE bound to mast cells and release of mediators
Type I hypersensitivity: Late phase occurs within
2-24 hours
Type I hypersensitivity: Late phase is characterized by
Infiltration of inflammatory cells and release of mediators by these cells
Type I hypersensitivity: Marker of anaphylaxis
Serum tryptase
Hypersensitivity reactions, type: Antibody-mediated
Type II
Type II hypersensitivity: Mechanisms (3)
1) Complement-dependent
2) Antibody-dependent cell-mediated cytotoxicity
3) Antibody-mediated cellular dysfunction
Type II hypersensitivity mechanism: Cell types that bear receptors for Fc of IgG mediate removal of antigen
Antibody-dependent cell-mediated cytotoxicity
Type II hypersensitivity mechanism: Antibodies themselves affect function of antigen
Antibody-mediated cellular dysfunction
Hypersensitivity reactions, type: Immune complex-mediated
Type III
Hypersensitivity reactions, type: T cell-mediated
Type IV
Type IV hypersensitivity: Mechanisms
1) Delayed form in which CD4+ Th1 cells sensitised from previous exposure to antigen secretes IF-γ that activates macrophages
2) Cell-mediated form in which CD8+ cytotoxic T cells kill antigen-bearing cells
Type IV hypersensitivity: Mechanisms of cell-mediated cytotoxicity
1) Perforin-granzyme system
2) FAS-FAS ligand system
Type IV hypersensitivity, mechanism: Holes are produced in the plasma membrane of cells, allowing granzyme to enter cells and activate apoptosis
Perforin-granzyme system
Type IV hypersensitivity, mechanism: FAS ligamnd on T lymphocytes bind to FAS on target cells leading to apoptosis
FAS-FAS ligand system
Type of hypersensitivity: Bee sting
Type I
Type of hypersensitivity: Drug allergy
Type I
Type of hypersensitivity: Glomerulonephritis
Type II or III
Type of hypersensitivity: Transfusion reactions
Type II
Type of hypersensitivity: Tuberculin reaction
Type IV, CD4+-mediated
Type of hypersensitivity: Erythroblastosis fetalis
Type II
Type of hypersensitivity: Contrast media allergy
Type I
Type of hypersensitivity: Serum sickness
Type III
Type of hypersensitivity: Urticaria
Type I
Type of hypersensitivity: AIHA
Type II
Type of hypersensitivity: Graves disease
Type II
Type of hypersensitivity: Insect venoms
Type I
Type of hypersensitivity: Food allergy
Type I
Type of hypersensitivity: Myasthenia gravis
Type II
Type of hypersensitivity: Contact dermatitis
Type IV
Type of hypersensitivity: Transplant rejection
Type II and IV
Mechanisms of transplant rejection (2)
Cell-mediated or humoral mediated
Classifications of transplant rejection
1) Hyperacute
2) Acute
3) Chronic
Mechanisms by which cytotoxic T cells kill graft cells
1) Perforin-granzyme pathway
2) FAS-FAS ligand pathway
Forms of cellular rejection: Body recognises MHC molecules on surface of APCs in the graft
Direct
Forms of cellular rejection: Antigens of the graft are presented by recipient’s cells
Indirect
Classification of transplant rejection: Humoral reaction due to preformed antibodies to graft endothelium
Hyperacute rejection
Classification of transplant rejection: Cellular or humoral reaction
Acute rejection
Classification of transplant rejection: 4-6 months to years following graft
Chronic rejection
Classification of transplant rejection: Possibly due to indirect form of cellular rejection
Chronic rejection
Classification of transplant rejection: Minutes following transplantation
Hyperacute
Classification of transplant rejection: Days to months to years following transplantation
Acute
Classification of transplant rejection: Cyanosis of organ and mottled parenchyma
Hyperacute
Classification of transplant rejection: Endothelial injury, neutrophils in arterioles, infarcts of parenchyma
Hyperacute
Classification of transplant rejection: Vascular changes and interstitial fibrosis
Chronic
Classification of transplant rejection: Interstitial mononuclear infiltrate and edema
Acute
Classification of transplant rejection: Interstitial hemorrhage and endothelia’s (swollen endothelial cells)
Acute
Classification of transplant rejection: Interstitial mononuclear infiltrate and schema with loss of tissue
Chronic
Classification of transplant rejection: Necrotizing vasculitis, neutrophilic infiltrates, and infarcts of parenchyma
Acute
Classification of transplant rejection: Will respond to cyclosporine
Acute
Immune competent cells in the graft recognize antigens in the host
GVHD
GVHD: Occurs in transplant of what
1) Bone marrow transplant
2) Solid organ transplant when organ is rich in lymphocytes
3) Non-irradiated blood
GVHD: Forms
1) Acute
2) Chronic
GVHD, acute vs chronic: Days to weeks
Acute
GVHD, acute vs chronic: Skin rash
Acute
GVHD, acute vs chronic: Fibrosis of dermis and skin appendages
Chronic
GVHD, acute vs chronic: Cholestatic jaundice
Chronic
GVHD, acute vs chronic: Jaundice
Acute
GVHD, acute vs chronic: Esophageal strictures
Chronic
GVHD, acute vs chronic: Bloody diarrhea
Acute
GVHD, acute vs chronic: Immunodeficiency
Acute and chronic
GVHD: Infection commonly associated with immunodeficiency in GVHD
CMV pneumonia
General mechanism of autoimmune diseases
Loss of self-tolerance
Autoimmune diseases: Contributing factors to loss of self-tolerance (2)
1) Susceptibility genes such as HLA B27 in ankylosing spondyltitis
2) Infections that upregulate costimulatory proteins on APCs or molecular mimicry
Associated antibodies: SLE
1) Anti-dsDNA
2) Anti-Smith
Associated antibodies: Drug-induced lupus
Antihistone
Associated antibodies: RA
IgM vs Fc of Ig
Associated antibodies: Sjogren
Anti-SSA and anti-SSB
Associated antibodies: CREST syndrome
Anti-centromere
Associated antibodies: Diffuse scleroderma
Anti-scl70
CREST in CREST syndrome
1) Calcinosis
2) Raynaud phenomenon
3) Esophageal dysfunction
4) Sclerodactyly
5) Telangiectasia
SLE: Age of predilection
Child-bearing age
SLE: Male vs female
Female
SLE: Male to female ratio in children and older adults
More or less equal
SLE: Criteria
SOAP BRAIN MD
1) Serositis
2) Oral ulcers
3) Arthritis
4) Photosensitivity
5) Blood disorders
6) Renal involvement
7) ANA
8) Immunologic phenomena
9) Neurologic didorder
10) Malar rash
11) Discoid rash
SLE: # of criteria to be fulfilled for diagnosis
4/11
SLE: Proteinuria
> 0.5g/dL
SLE: Presence of antiphospholipid Ab is detected by what tests
1) Positive lupus anticoagulant test
2) Abnormal level of IgG or IgM anticardiolipin
3) False-positive syphilis test
SLE: Best screening test
ANA
SLE: SLE-specific
Anti-dsDNA, anti-Sm
SLE: Associated with decreased risk for nephritis
Anti-Ro (SSA) and anti-La
SLE: Associated with neonatal lupus with congenital heart block
Anti-Ro
SLE: Type of hypersensitivity
Type III
SLE: Risk factors
Genetics and environment
SLE: Most common cause of death
Renal disease and intercurrent infections
SLE: Second most common cause of death
CNS diseases
SLE: Endocarditis
Libman-Sacks
SLE: T/F Vegetations in SLE endocarditis is sterile
T
SLE: LE cell
Neutrophil with phagocytized nuclei
SLE: Classes of nephritis
I - No disease II - Increase in mesangial matrix with deposition of immune complexes III- FPGN IV - DPGN V - MGN
SLE: Characteristic pattern seen in Class V SLE nephritis
Wire-loop pattern
Due to antibody that delays clotting in vitro and induces a hyper coagulable state in vivo
APAS
APAS: Classic clinical triad
1) Thrombosis
2) Thromobocytopenia
3) Recurrent abortions
Forms of APAS: Occurs as sole entity and not associated with SLE
Primary
Forms of APAS: Occurs in patients with SLE
Secondary
SLE: Drugs associated with drug-induced lupus
PIMCH
1) Procainamide, Penicillamine
2) Isoniazid
3) Methyldopa
4) Chlorpromazine
5) Hydralazine
6) Quinidine
Characteristics of drug-induced lupus that differentiates it from SLE (5)
1) Gender ratios nearly equal
2) Predominancy of anti-histone abs
3) Renal and CNS manifestations are uncommon
4) No anti-dsDNA or hypocomplementemia
5) Discontinuation of drugs lead to resolute of symptoms and lab parameters
RA: Men vs women
Men
RA: Type of arthritis
Non-suppurative, proliferative
RA: Joints affected
Bilateral, most commonly in hands
RA: Classic presentation
Morning stiffness >1 hour in duration
RA: Classic signs
1) Boutonniere deformity
2) Swan neck deformity
3) Baker’s cyst
4) Rheumatoid nodules
RA: Arthritis of PIP
Swan neck deformity
RA: Arthritis of DIP
Boutonniere deformity
RA: Antibody
Rheumatoid factor
RA: Type of hypersensitivity reaction
Type III
RA: Proliferating synovial cells mixed with inflammatory cells and granulation tissue
Pannus
RA: Pannus formation can lead to
Ankylosis (fibrosis and calcification of joint space)
RA: Central fibrinoid necrosis surrounded by palisading macrophages, with an outer rim of lymphocytes and plasma cells
Rheumatoid nodules
Sjogren syndrome: Age of predilection
50-60 years
Sjogren syndrome: Men vs women
Women
Sjogren syndrome: Triad
1) Xerostomia
2) Xerophthalmia
3) Autoimmune disorder (usually RA)
Sjogren syndrome: Antibody associated with higher likelihood of having systemic manifestations
Anti-SSA
Sjogren syndrome: Neoplastic complication
MALToma (neoplasm of mucosa-associated lymphoid tissue
Autoimmune disorder associated with fibrosis of organs
Scleroderma
Scleroderma: Age of predilection
50-60
Scleroderma: Male vs female
Female
Scleroderma: Clinical presentations (2)
1) Limited scleroderma or CREST syndrome
2) Diffuse scleroderms
Scleroderma: Anti-Scl70 is an antibody against
DNA topoisomerase I
Autoimmune disorder with findings suggestive of SLE, polymyositis, RA, and systemic sclerosis
Mixed CT disorder
Mixed CT disorder: Associated antibodies
Anti-RNP particle containing U1
Mixed CT disorder: Renal disease
Little or none
Mixed CT disorder: Response to corticosteroids
Good
Autoimmune disorders, microscopic: Lymphocytic and plasmacytic infiltrate of salivary and lacrimal glands, associated with ductal damage
Sjogren syndrome
Autoimmune disorders, microscopic: Fibrosis involving dermis, muscularis of GIT, and alveolar septae in the lung and interlobular arteries in kidney
Scleroderma
Amorphous, hylaine deposition that has apple-green birefringence upon polarisation after Congo red staining
Amyloidosis
Amyloidosis: Due to
Abnormal production and deposition of protein
CKD with large kidneys (4)
1) Amyloidosis
2) HIV
3) DM
4) Polycystic kidney disease
Hereditary immunodeficiency states: Failure of maturation of B cells
X-linked agammaglobulinemia of Bruton
X-linked agammaglobulinemia of Bruton: Mutated gene
Gene for B cell tyrosine kinase
X-linked agammaglobulinemia of Bruton: Inheritance pattern
X-linked recessive
X-linked agammaglobulinemia of Bruton: Manifests by age of
6 months
X-linked agammaglobulinemia of Bruton: Delay in manifestation due to
Presence of maternal IgG
Forms of amyloidosis, associated disease: Ig light chain
Multiple myeloma
Forms of amyloidosis, associated disease: Serum amyloid-associated protein
Hereditary amyloidosis
Forms of amyloidosis, associated disease: Transthyretin
Systemic senile amyloidosis
Forms of amyloidosis, associated disease: Amyloid precursor protein
Alzheimer disease
Forms of amyloidosis, associated disease: Calcitonin
Medullary thyroid carcinoma
Hereditary immunodeficiency states: No one mode of inheritance
Common variable immunodeficiency
Hereditary immunodeficiency states: X-linked inheritance with mutation of CD40L
Hyper-IgM syndrome
Hereditary immunodeficiency states: Wiskott-Aldrich syndrome inheritance
X-linked recessive
Hereditary immunodeficiency states: B cells proliferate in response to antigen but cannot produce Ig
Common variable immunodeficiency
Hereditary immunodeficiency states: Defect in differentiation of B lymphocytes to IgA-producing cells
Isolate IgA deficiency
Hereditary immunodeficiency states: T cells fail to stimulate B cells to produce antibody other than IgM
Hyper-IgM syndrome
Hereditary immunodeficiency states: Defect in cytokine receptor for IL-7 which is required for lymphocyte proliferation
SCID, X-linked recessive
Hereditary immunodeficiency states: Accumulation of deoxy-ATP, which is toxic to lymphocytes
SCID, autosomal recessive
Hereditary immunodeficiency states: Recurrent infections before the age of 6 months with wide range of pathogens
SCID
Hereditary immunodeficiency states: Thrombocytopenia with resultant bleeding at circumcision site, eczema, and recurrent infections
Wiskott-Aldrich syndrome
Hereditary immunodeficiency states: Mutation in gene for adenosine deaminase
SCID, autosomal recessive
Hereditary immunodeficiency states: Autosomal vs X-linked SCID, male predominance
X-linked
Hereditary immunodeficiency states: Mutation in gene for common γ chain subunit of cytokine receptors
SCID, X-linked recessive
Hereditary immunodeficiency states: Recurrent pyogenic infections and pneumocystis pneumonia
Hyper-IgM syndrome
Hereditary immunodeficiency states: Hyper-IgM syndrome, mutated in autosomal inheritance
CD40
Hereditary immunodeficiency states: Recurrent sinopulmonary infections, diarrhea, and increased incidence of autoimmune diseases
Isolated IgA deficiency
Hereditary immunodeficiency states: Isolated IgA deficiency may be acquired due to what infections
1) Toxoplasmosis
2) Measles
Hereditary immunodeficiency states: Lymphoid follicular hyperplasia
Common variable immunodeficiency
Hereditary immunodeficiency states: Common variable deficiency, age of presentation
Later childhood and adolescence
Hereditary immunodeficiency states: Underdeveloped germinal centres in lymphoid organs
X-linked agammaglobulinemia of Bruton
Hereditary immunodeficiency states: Hypoplasia of tonsils and adenoids
X-linked agammaglobulinemia of Bruton
AIDS: HIV, type of virus
RNA retrovirus
AIDS: Modes of transmission
1) Sexual
2) Parenteral
3) In-utero, transplacental, or intrapartum
AIDS: Major capsid protein
p24
AIDS: Encodes p24
gag gene
AIDS: Encodes reverse transcriptase
pol gene
AIDS: Binds to CD4
gp120
AIDS: Binding of gp120 to CD4 results in exposure of binding site of ___ on T cells
CXCR4
AIDS: Binding of gp120 to CD4 results in exposure of binding site of ___ on macrophages
CCR5
AIDS: Binding of gp120 to CD4 results in a change in gp41 resulting in
Viral-cell fusion
Organs colonised by HIV
Lymphoid organs
Pneumonia that produces a fluffy-pink exudate in the alveolar spaces
Pneumocystis pneumonia
AIDS: Risk factor for pneumocystis pneumonia
CD4+ count
AIDS: Opportunistic infection that causes enteritis
Cryptosporidiosis
AIDS: Chest radiograph of pneumocystis
Diffuse interstitial ground-glass pattern
AIDS: Common associated malignancies (2)
1) Kaposi sarcoma
2) Non-Hodgkin lymphoma (B cell lymphoma of brain)
AIDS: Kaposi sarcoma is derived from
Blood vessels
AIDS: Opportunistic infection that causes pneumonia or CNS infection
Toxoplasmosis
AIDS: opprotunistic infection that causes meningitis
Cryptococcosis
AIDS: Mycobacteria that are opportunistic in patients with AIDS
M. tuberculosis and M. avium intracellulare
AIDS: Kaposi sarcoma is related to what virus
HHV-8