Immunology Flashcards
Kupffer Cell
Liver macrophage
Mesangial Cell
Kidney macrophage
Microglia
Neural macrophage
Langerhans Cell
Skin macrophage
Isotype Switching and Somatic Hypermutation happen where?
Germinal Centre
Memory response caused by which type of immunoglobulin?
IgG
Congenital neutropaenia
Kostmann Syndrome
HAX1 mutation
Neutropaenia every 6 weeks
Cyclic neutropaenia
Neutrophil Elastase mutation
Very high neutrophils in blood, none in tissue
Leukocyte Adhesion Deficiency: lack CD18 –> neutrophils can’t exit blood
Recurrent chickenpox
NK Deficiency
SLE caused by deficiency of which complement pathway?
Classical Pathway Deficiency
Which renal disease causes complement deficiency?
Membranous Glomerulonephritis = nephritic factors attack C3
High forehead, low-set ears, hypocalcaemia
DiGeorge Syndrome
DiGeorge Syndrome is due to deletion of what?
Chromosome 22q11.2
Do not respond to immunisations
Common Variable Immune Deficiency (very low IgG)
Gene locus in Coeliac
HLA-DQ2
Confirmation of Coeliac diagnosis
Upper GI endoscopy + distal duodenal biopsy
Periodic fevers, amyloid A amyloidosis and red rash
Familial Mediterranean Fever
Hypoparathyroidism and Addison’s
APS1 and APECED = defect in autoimmune regulator (AIRE) = transcription factor for T cell tolerance in thymus
Crohn’s caused by this mutations
NOD2 mutation in chromosome 16
NOD2 stimulates NFkB
Inflammation of entheses (insertion of ligament/tendon) and sacro-iliitis
Ankylosing Spondylitis
Anti-GAD65 and anti-IA2 antibodies
Type I Diabetes
Drooping eyelids and weakness after repetitive activity
Myasthenia Gravis
Autoimmune condition featuring crescentic nephritis on biopsy
Goodpasture’s Disease
Tensilon Test
Myasthenia Gravis
= edrophonium (anti-cholinesterase) prolongs life of ACh…
Rheumatoid Arthritis associated with these alleles
HLA DR1 and DR4
Porphyromonas gingivalis gum infection causes which autoimmune disease?
Rheumatoid Arthritis
Rheumatoid Arthritis associated with these polymorphisms
PAD2 and PAD4
PTPN22
Anti-Ro and anti-La
Sjogren’s
Heliotrope rash
Dermatomyositis
Severe side effect of cyclophosphamide
Haemorrhagic cystitis = toxic metabolite acrolein excreted in urine
People susceptible to bone marrow suppression with azathioprine
Thiopurine Methyltransferase (TPMT) polymorphism = can’t metabolise azathioprine…
Severe side effects of mycophenolate
Progressive Multifocal Leukoencephalopathy (JC virus which destroys oligodendrocytes)
Herpes reactivation
Side effect of ciclosporin
Dysmorphia
Side effect of rituximab
Progressive Multifocal Leukoencephalopathy
Side effects of anti-TNFa
Lupus-like condition
Demyelination
Melanoma
Side effect of denosumab
Avascular necrosis of jaw
Which malignancies are increased in transplant patients?
Virus-associated
- Kaposi Sarcoma (HHV8)
- Lymphoproliferative Disease (EBV)
Definition of AIDS
CD4 < 200
Definition of positive skin-prick test
> 3mm greater than negative control
Biomarker of anaphylaxis
Serial mast cell tryptase
Gold-standard test for allergy
Challenge Testing
Antibodies deposited along GBM in “smooth linear pattern”
Goodpasture’s
Low T cells with cleft palate
DiGeorge Syndrome = CATCH: Cardiac Atresia (oesophagus) Thymus absent Cleft Palate Hypocalcaemia
Mutation of MHC III causes this disease
Common Variable Immunodeficiency (recurrent chest infections)
Negative NBT Test (i.e. stays colourless)
Chronic Granulomatous Disease = deficiency of NADPH oxidase
(NADPH oxidase turns NBT blue)
–>have NORMAL neutrophil count as their numbers are fine, just their mechanism of killing
Deficiency in either MHC I or MHC II
Bare Lymphocyte Syndrome
-causes jaundice
Recurrent resp and GI infections
Anaphylaxis after 2nd blood transfusion from same donor
Selective IgA Deficiency Disease
- IgA = mucosal immunity, so have recurrent resp and GI infections
- Anaphylaxis after blood transfusions due to donor IgA (2nd transfusion only as need to make antibodies against donor IgA in 1st transfusion)
Low IgM
Raised IgA and IgG
Wiskott-Aldrich Syndrome
Deficiency in this enzyme causes SCID
Adenosine deaminase
BCG vaccine contains this live attenuated organism
Mycobacterium bovis
Vaccine made of detoxified exotoxin product of the microbe
Tetanus
Vaccine made using recombinant DNA
Hep B
Vaccine given to sero-negative women only
Rubella
Vaccine available in live attenuated (oral) and inactivated –> excreted in stools
Poliomyelitis
Conjugate vaccine given to neonates
HiB
Adjuvant that promotes antibody response via IL-4 (which primes naive B cells)
Alum
Which immunity is induced by vaccines: active or passive?
Active
Immunosuppressant causing gum hyperplasia
Cyclosporine A
- Steroid used to prevent rejection
2. Steroid used to treat rejection
- Prednisolone
2. Methylprednisolone
Anti-Jo1
Dermatomyositis
Anti-topoisomerase
Diffuse systemic scleroderma
Dry mouth and eyes
Joint pain
Sjogren’s Syndrome (exocrine glands destroyed)
–> diagnose dry eyes using Schirmer’s Test
Antibodies in Sjogren’s
Anti-Ro and Anti-La … also Rheumatoid Factor
Saddle-shaped nose, with haemoptysis and haematuria
Wegener’s Granulamatosis
Target of IgG in Goodpasture’s
Type IV collagen (found in GBM and lung basement membrane!)
Polyarteritis nodosa with these infections
Hepatitis B and C – look out for “IVDU” in the question!
22 year old female who has recently undergone surgery presents with difficulties breathing and swelling of the face, hands and feet. She also complains of severe abdominal pains. She has experienced similar problems in the past.
C1 inhibitor deficiency = Hereditary Angioedema
HLA associated with psoriatic arthritis
HLA-B27
HLA associated with dermatitis herpetiformis
HLA-DR3
Which cells mediate:
- Hyperacute organ rejection
- Acute and chronic organ rejection
- B cells
2. T cells
Complements that can activate mast cells to cause allergic symptoms
C3a and C5a
What type of hypersensitivity in:
- SLE
- Rheumatoid arthritis
- Serum sickness
- Post-strep glomerulonephritis
Type III = antibody-antigen complexes depsoit
- SLE = dsDNA
- Rheumatoid arthritis = Fc region of IgG
- Post-strep = strep wall antigens
What type of hypersensitivity in:
- T1 DM
- MS
- Contact dermatitis
- Hashimoto’s
Type IV = T cells
- T1 DM = beta cells
- MS = myelin protein
- Contact dermatitis = environmental chemicals
- Hashimoto’s = thyroglobulin
What type of hypersensitivity in:
- Autoimmune Haemolytic Anaemia
- ITP
- Goodpasture’s
- Pernicious anaemia
Type II = antibodies bind to cell-based antigen
- Autoimmune Haemolytic Anaemia = RBCs
- ITP = platelets
- Goodpasture’s = GBM
- Pernicious anaemia = parietal cells
What type of hypersensitivity in:
- Graves
- Myasthenia Gravis
Type V = antibodies bind to cell surface receptor
- Graves = TSH receptor
- Myasthenia Gravis = ACh receptor
Immunoglobulin which passes through:
- Placenta
- Breast milk
- IgG
2. IgA
First immunoglobulin secreted in response to infection
IgM
Cell surface marker associated with Reed-Sternberg cells
CD15
Complement deficiency predispose to which type of infection?
Encapsulated bacterial (meningococcus, pneumococcus…)
Which kidney disease causes secondary complement deficiency?
Membranous Glomerulonephritis = nephritic factors attack C3
Enzyme deficiency causing SCID
Adenosine Deaminase (ADA) Deficiency
Condition where you lack MHC II
Bare Lymphocyte Syndrome
- -> lack CD4 (have normal CD8 and B cells)
- ->low IgG and IgA as lack help from CD4!
Condition where you lack mature B cells
Bruton’s X-Linked Agammaglobulinaemia
= abnormal B cell tyrosine kinase
-only in boys as X-linked!
Condition where you have lots of IgM, but no other immunoglobulin
Hyper IgM Syndrome
=mutation in CD40 ligand (expressed on T cells)
–>IgM B cells can’t enter germinal centre
–>no isotype switching
Hyper IgM predisposes you to this infection
Pneumocystis jiroveci
B cell deficiency associated with Coeliac
Selective IgA Deficiency
Monogenic auto-inflammatory disorder
Familial Mediterranean Fever
Immunoglobulin that is a dimer
IgG = most abundant immunoglobulin
Immunoglobulin that is a pentamer
IgM
HLA DR3
Graves
SLE
Myasthenia Gravis
HLA DR4
T1DM
Rheumatoid Arthritis
SLE deficient in which complement pathway?
Classical (C1, 2, 4) = clears immune complexes and apoptotic cells
Autograft
Isograft
Allograft
Xenograft
Autograft = own Isograft = twin Allograft = same species Xenograft = different species
Azathioprine converted to this metabolite, which prevents DNA synthesis
6-mercaptopurine
Corticosteroids inhibit….
Phospholipase A2
How does HIV enter CD4?
gp120 binds to CXCR4 on surface of CD4
CCR5 is on surface of macrophages!
Important side effects of anti-TNF
Lupus-like condition
Demyelination
Cancer
Denosumab inhibits…
RANK ligand (inhibiting osteoclast)
Specific test for dry eyes in Sjogren’s
Schirmer’s Test
Anti-CCP target what in rheumatoid?
Filaggrin
Anti-centromere
Limited systemic scleroderma (CREST)
Anti-thyroid peroxidase and anti-thyroglobulin
Hashimoto’s
Abnormal FoxP3 –> dysfunctional Tregs
IPEX (X-linked)
- Diabetes
- Eczema
- Enteropathy
Mutations in AIRE (important for central tolerance)
APECED Syndrome Type 1
–>hyothyroid, alopecia and vitiligo
Anti-CD25 (part of IL-2R), prevents allograft rejection
Basiliximab
CTLA4 fusion protein, for rheumatoid arthritis
Abatacept
Monoclonal used in highly-active MS and last-resort for Crohn’s
Natalizumab = anti-α4 integrin
Denosumab inhibits
RANK ligand
Anti-IL-12 and anti-IL-23 used in psoriasis and Crohn’s
Ustekinumab
Side effect = TB
Most severe form of influenza
Influenza A
Bird flu is this strain if influenza
H5N1
What is anti-SCL-70?
Anti-topoisomerase in systemic sclerosis!
Biologic used in psoriasis
Ustekinumab = anti-IL-12 and anti-IL-23
Mechanism of cyclophosphamide
Alkylating agent –> alkylates guanine base of DNA –> damages DNA
Mechanism of mycophenolate
Anti-metabolite = Stops synthesis of new guanine –> prevents DNA replication
Immunosuppressant used in multi-system vasculitis (like Wegener’s)
Cyclophosphamide
Which type of antihistamine used in allergy: H1, 2, 3 or 4?
H1 antagonist (H2 = gastric mucosa)
Neutralising antibodies are formed against this HIV molecule
gp120
Non-neutralising antibodies are formed against this HIV molecule
p24
Co-receptors required for HIV entry into cells
CCR5 and CXCR4
What is CD40L deficiency?
Hyper IgM Syndrome!
Who can have falsely negative anti-TTG?
IgA Deficient (as anti-TTG is IgA)
Single gene mutation involving FOXP3 –> abnormal T regulatory cells
IPEX
Mutated pyrin-marenostrin
Familial Mediterranean Fever
Tests for Chronic Granulomatous Disease
NBT Test (does not change yellow to blue) and DHR Test (no fluorescence)
Recurrent atypical Mycobacterium infections
IFN-γ receptor deficiency
Initiates Classical Complement
Deficiency associated with SLE
C1q
Test used to diagnose Di George
FISH = detects microdeletions (22q11)
Recurrent meningococcal infections
Complement Deficiency
Recurrent resp and CNS infections (in a boy)
Bruton’s X-Linked Agammaglobulinaemia
Defect in Bruton’s X-Linked Agammaglobulinaemia
Abnormal B cell tyrosine kinase –> pre-B cells do not mature
Most specific test in Sjogren’s
Anti-La
Components of Classical Complement Pathway
C1, 2, 4 – C1 binds to antibody-antigen complexes, activating cascade
Components of Alternative Complement Pathway
C3 and Factors B, I, P – C3 binds to bacterial cell wall parts (like LPS)
Components of MBL Pathway
C2 and C4 – MBL binds to microbial cell surface carbs, stimulating Classical Pathway
Complement Pathway dependent of adaptive immune system
Classical (C1 binds to antibody-antigen complexes)
What complement gets activated in the major amplification step of the cascade?
C3
Membrane Attack Complex via which complements?
C5-9
Membrane Attack Complex formation defect (C5-9 deficiency) results in…
Meningococcal infections
Deficiency in Classical Complement Pathway associated with…
Immune-complex diseases (SLE)
Most common mode of inheritance in Di George
90% sporadic!
Low IgM, eczema and low platelets
Wiskott-Aldrich Syndrome
Recurrent PCP infections in a child
SCID (low T cells)
Recurrent Aspergillus infection
Chronic Granulomatous Disease (neutrophils can’t kill)
Hereditary Angioedema = deficiency in what?
C1 inhibitor protein –> have low C4 during attacks
Dose used in anaphylaxis
0.5mg of 1:1000
Antibody which correlates with disease severity in SLE
Anti-dsDNA
Positive hydrogen breath test, increased stool osmolality
Lactose Intolerance
Cause of commonest SCID
X-linked recessive mutation of common gamma chain on chr X (cytokine receptor)
Cause of SCID in girls
Adenosine Deaminase (ADA) Deficiency
DiGeorge is due to developmental defects in what during pregnancy?
3rd and 4th pharyngeal pouches = source of thymus and parathyroids
Deficiency in this complement increases risk of encapsulated organisms
C3
HLA in Reactive Arthritis
HLA-B27
HLA in Dermatitis Herpetiformis
HLA-DR3
HLA in Rheumatoid Arthritis
HLA-DR4
First immunoglobulin in response to infection
IgM = IgNow!
Immunoglobulin deficiency predisposing towards encapsulated organisms
IgG Deficiency
Biologic against alpa-4-integrin
Natalizumub
Calcineurin Inhibitors inhibit secretion of this cytokine
IL-2 (promotes T cell proliferation)
Diagnosis of coeliac in IgA deficient
Go straight to duodenal biopsy
Coeliac antibody that remains for 12 months after gluten-free diet
Anti-gliadin
Cytokine in central memory
IL-2
Vaccine made using recombinant DNA technology
Hep B
Antigen assembled in a multimeric form and saponin that provokes a strong serum antibody response.
Immunostimulatory complexes (ISCOMS)
Type of collagen targeted in Goodpasture’s
Type IV collagen