Immuno hard to recall Flashcards
(262 cards)
1
Q
What type of anaphylaxis does aspirin cause? What is another name for this?
A
Non-IgE mediated anaphylaxis
Mast cell degranulation
2
Q
Difference between acute vs chronic urticaria?
A
Acute = <6 weeks intermittent rash which appears in same site for <24 hours each time Chronic = >6 weeks
3
Q
When would an urticarial rash make you suspicious of an underlying vasculitis?
A
If it lasts more than 24 hours in a single site
If it resolves with bruising or skin depigmentation
4
Q
What does C1 inhibitor deficiency cause?
A
acquired angioedema
5
Q
What dose of adrenaline should be given in anaphylaxis?
A
IM 1ml of 1:1000 (i.e. 1mg/ml)
6
Q
Kostmanns syndrome is a congenital deficiency of which component of the immune system?
A
Neutrophil
7
Q
Which infection is most common as a consequence of B cell deficiency?
A
Bacterial
8
Q
Meningococcal infections are quite common as a result of which deficiency of the component of the immune system?
A
Complement
9
Q
A complete deficiency in this molecule is associated with recurrent respiratory and gastrointestinal infections
A
IgA
10
Q
Leukocyte Adhesion Deficiency is characterised by a very high count in which cell line?
A
neutrophils
11
Q
Which crucial enzyme is vital for the oxidative killing of intracellular micro-organisms?
A
NADPH oxidase
12
Q
Which complement factor is an important chemotaxic agent?
A
C3a
13
Q
What type of hypersensitivity reaction is RA?
A
Type IV – T-cell mediated
14
Q
What type of hypersensitivity reaction is T1DM?
A
Type IV – T-cell mediated
15
Q
What type of hypersensitivity reaction is ITP?
A
Type II – Antibody mediated
16
Q
What type of hypersensitivity reaction is Hepatitis C associated membranoproliferative glomerulonephritis type I?
A
Type III – Immune complex mediated
17
Q
What type of hypersensitivity reaction is Goodpasture’s?
A
Type II – Antibody mediated
18
Q
What type of hypersensitivity reaction is MG?
A
Type II – Antibody mediated
19
Q
Antibody in SLE?
A
Anti-dsDNA
20
Q
Antibody in Wegener’s?
A
c-ANCA
21
Q
Antibody in RA?
A
anti-CCP
22
Q
Antibody in PBC?
A
AMA
23
Q
What is the specific auto-antigen that is the target of the immune system in goodpasture’s?
A
Type IV collagen
24
Q
Tx for goodpasture’s?
A
prednisolone
25
Which cytokines exert an antiviral effect?
Interferons
26
Which Ig is a dimer?
IgA
27
Which cytokine acts on hepatocytes to induce synthesis of acute phase proteins in response to bacterial infection
IL6
28
What are the first immunoglobulins expressed on B cells before they undergo antibody class switching?
IgD and IgM
29
What is the most abundant (in terms of g/L) immunoglobulin in normal plasma?
IgG
30
Deficiency in which pathway predisposes to SLE?
Classical complement pathway
31
X-Linked Agammaglobulinaemia is due to a problem in which gene?
Bruton’s tyrosine kinase (Btk) gene
32
X-linked Severe Combined Immunodeficiency is due to a problem in which receptor?
IL2 receptor
33
Wiskott-Aldrich Syndrome is due to a problem in which gene?
WASP
34
Bare lymphocyte syndrome type 2 is due to a problem in which part of the immune system?
MHC class II
35
For which disorder would a bone marrow transplant be unhelpful but a thymic transplant may provide a cure?
DiGeorge syndrome
36
In acute rejection what is released by CD8 lymphocytes to kill target cells?
Granzyme B
37
What is a risk factor for chronic allograft rejection?
HTN
38
What are the 3 most important HLA types to screen for in renal transplantation when matching donor and recipient, in order of importance
HLA DR > B > A
39
Which lymphocytes respond to foreign HLA DR types
CD4+ T cells
40
Which lymphocytes respond to foreign HLA DR types
CD8+ T cells
41
Which immunosuppressant drug prevents DNA replication especially of T cells by impairing guanine synthesis
Mycophenolate mofetil
42
Which immunosuppressant drug causes a transient rise in neutrophils?
Prednisolone
43
SE of prednisolone?
HTN
44
SE of ciclosporin?
Hypertension and reduced GFR
45
SE of azathioprine?
Bone marrow depression
Hepatotoxicity
Hypersensitivity reactions
46
SE of methotrexate?
Pneumonitis, pulmonary fibrosis and cirrhosis
47
SE of immunoglobulin?
Anaphylaxis
48
Give an example of A sub-unit / conjugate vaccine
pneumococcal vaccine
49
What type of vaccine is oral poliomyelitis vaccine?
A live attenuated viral vaccine
50
Give an example of Inactivated preparations of the bacteria for vaccine
Whole cell typhoid vaccine
51
Give an example of a vaccine that is made using recombinant DNA technology
Hep B
52
Name a A feature of immunological central memory
IL2
53
Which live attenuated organism do BCG vaccines contain?
Mycobacterium bovis
54
What type of vaccine is HiB?
Conjugated
55
What Agent is used in humans that promotes a predominantly antibody response through the release of Il-4 that primes naïve B-cells?
Alum
56
What Diploid cell vaccine containing inactivated virus is given before or after exposure to those considered at risk?
Rabies
57
What type of vaccine is Hep A?
Inactivated
58
What is a A water-in-oil emulsion containing mycobacterial cell wall components that could be used to increase the immune response of a vaccine?
Freund's adjuvant
59
What monoclonal antibody targets RANKL?
Denosumab
60
What drug class does dolutegravir belong to?
Integrase Inhibitor
61
The paediatric SpR is called to review a 21 day old infant who is pyrexic and appears unwell. He is noted to be hypocalcaemic and has a cleft palate. He has a fever of 39C. Blood tests reveal a neutrophil count of 8 (normal range: 1.5-8.0), absent T cells and normal B cells. What primary immunodeficiency is this patient likely to have?
DiGeorge Syndrome
62
What is the primary target of the antibody C-ANCA in Wegener's?
Proteinase 3
63
What is the primary target of the antibody p-ANCA?
Myeloperoxidase
64
A patient's autoimmune panel of bloods reveals positive anti-glycoprotein IIb antibodies. On the patient's lower legs, there are pinprick petechiae. The patient's platelet level is very low. What autoimmune condition is associated with this autoantibody and clinical presentation?
Autoimmune Thrombocytopenic Purpura
65
Encapsulated bacteria NHS?
Neisseria
Haemophilus
Streptococcus/Pneumococcus
66
What monoclonal antibody targets IL-17 and is involved in the treatment of psoriasis, psoriatic arthritis and ankylosing spondylitis?
Secukinumab
67
What immunoglobulin mediates type I hypersensitivity reactions?
IgE
68
What is the causative agent of Progressive Multifocal Leukoencephalopathy?
JC virus
69
A 5 year old girl is seen by her GP. Her mother says she is unable to sleep at night as her knees, elbows and shoulders are very red and painful, especially to touch. Her mother has also noticed small bumps under her skin. She is currently pyrexic, but the mother says she is still recovering from a bad sore throat two weeks ago.
What is the likely diagnosis?
Acute Rheumatic Fever
70
What is the short-term treatment for severe, life threatening antibody mediated damage or dysfunction, for example myasthenic crisis, cyroglobulinaemia or antibody mediated rejection?
Plasmapheresis
71
What lymphocyte lineage does azathioprine predominantly inhibit?
T lymphocytes
72
What assay is used as the gold-standard for diagnosis of HIV or as a confirmatory test of infection?
western blot
73
A 74 year old woman presents to the GP with of loss of vision and a severe headache around the sides of their head. In addition, she reports feeling generally tired and has trouble "getting going" in the morning.
An urgent referral to A&E is made. She treated urgently by the team and is admitted to hospital after blood results showed an elevated CRP. 24 hours after admission, a new set of bloods showed a moderate leukocytosis, which was not present on the previous bloods. In the absence of other symptoms, what is the most likely cause of the leukocytosis?
Corticosteroids
74
Which cell type secretes immunoglobulins?
Plasma cells
75
What is measured during an anaphylactic reaction to monitor response to treatment?
Mast Cell Tryptase
76
What viral enzyme is predominantly responsible for the high mutation rate of HIV and other RNA viruses?
Reverse Transcriptase
77
What specific cell subtype expresses both CD3 and CD4?
T Helper cells
78
What blood product is most commonly contaminated by bacteria?
Platelets
79
A 31 year old accountant reports an itchy rash over the trunk and back that has been present for the last 7 weeks. On examination, there are smooth erythematous papules with evidence of excoriation. She reports no association with food, time and has changed her washing powder to a "Non-Bio" formulation.
What condition is she suffering from?
Chronic Urticaria
80
What cell type is the hallmark of acute inflammation?
Neutrophils
81
What is the name given to a substance that increases the effectiveness of an immune reponse to a vaccination without altering the specificity of the response?
Adjuvant
82
A 70 year old gentleman is referred to the nephrologists for worsening kidney failure and haematuria. Over the last 3 months, he has lost 20% of his body weight, reports intermittent fevers and joint aches. He is also seeing his GP as he recently started coughing blood.
What underlying immunological condition may this patient have?
Goodpasture's Disease
83
What is the inheritance pattern of reticular dysgenesis?
Autosomal recessive
84
An autoimmune panel of bloods reveals positive anti-dsDNA antibody at a titre of 1:1600.
What autoimmune condition is associated with this autoantibody?
SLE
85
Name 4 drugs which target TNF alpha
Infliximab
Adalimumab
Certolizumab
Golimumab
86
What is the only lymphocyte subset considered to be part of the innate immune system?
Natural killer cells
87
A 38 year old lady with known polycystic kidney disease received 3 units of blood over the last day due to a post-partum haemorrhage. She is complaining of shortness of breath. On examination she has mild pitting oedema of the sacrum and lower limbs. Serum BNP is elevated. She is apyrexic. What complication of blood transfusion is she suffering from?
Transfusion Associated Circulatory Overload
88
IgE antibodies bind semi-permanently to the surface of what cell of the innate immune system?
Mast cells
89
The paediatric SpR is called to review a 21 day old infant who is pyrexic and appears unwell. The parents think he has a problem with his hearing as he has been tugging at his ear. There is pain, tenderness and swelling over the mastoid process of his left ear. Blood tests reveal a neutrophil count of 0.1 (normal range: 1.5-8.0) What primary immunodeficiency is this patient likely to have?
Kostmann Syndrome
90
A 21 year old woman reports weight loss, tiredness, diarrhoea and non-bloody offensive smelling stools. She has had three chest infections requiring antibiotics in the last three years. Blood tests are ordered and reveals a hypochromic, microcytic anaemia with low ferritin. Anti-TTG and anti-endomysial antibodies are negative. There are normal levels of IgG, IgM and IgE. No IgA is found.
What is the most likely cause of her gastrointestinal symptoms?
Coeliac Disease
| Anti-endomysial and anti-TTG are IgA autoantibodies so may be falsely negative in IgA deficiency
91
What does a correctable/reversible deformity of something like swan neck deformity in the hands suggest?
Suggests Jaccoud's arthropathy as part of SLE rather than a diagnosis of rheumatoid arthritis
92
An arterial biopsy of a patient with a suspected arteritis reveals a "rosary bead" appearance of small aneurysms.
What vasculitis is associated with this clinical sign?
Polyarteritis Nodosa
93
An autoimmune panel of bloods reveals positive anti-liver kidney microsomal-1 antibody and anti-smooth muscle antibody. There is also derangement of LFTs. What autoimmune condition is associated with these autoantibodies and deranged LFTs?
Autoimmune Hepatitis
94
Which cytokine is predominantly responsible for T cell proliferation and survival?
IL2
95
Triad in Granulomatosis with Polyangitis?
Rapidly progressive glomerulonephritis
nosebleeds
haemoptysis
96
What is the most severe form of scid?
Reticular dysgenesis
97
What intracellular pro-proliferative molecule does cyclosporine and tacrolimus inhibit? What cytokine does this prevent release of?
Calcineurin
| IL2
98
What is the specific antigen recognised by the immune system in Goodpasture's syndrome?
Type IV collagen
99
What type of bacteria are conjugated vaccines effective against?
Encapsulated
100
A neutrophil releases preformed myeloperoxidase, defensins and neutrophil elastase. What is this process known as?
Degranulation
101
Prior to starting terbinafine, a systemic antifungal, what blood test should be ordered?
LFTs
102
Tx for Q fever?
Doxycycline or ciprofloxacin
103
Preggers and UTI?
nitrofurantoin
104
A 50 year old woman is seen by her GP with 3 days of mild headache, sneezing and coughing. She also has a sore throat. She takes ramipril for hypertension but has no other past medical history. On examination her chest is clear, there is no cervical lymphadenopathy and no visible exudate on the tonsils. What is the likely responsible organism?
Rhinovirus
105
What family of virus does dengue virus belong to?
Flavivirus
106
Other than T lymphocytes, what cell type is primarily infected by HIV?
Macrophages
107
What other 2 diseases should patients newly diagnosed with T1DM be screened for?
Coeliac
| Hypo/hyperthyroidism
108
What type of vaccine uses antigenic proteins to induce immunity?
Subunit (recombinant)
109
What is the maximum number of viral capsids that a molecule of IgG may bind to?
2
110
An autoimmune panel of bloods reveals a positive anti-centromere antibody. What autoimmune condition is associated with this autoantibody?
Limited Systemic Sclerosis
111
What do you measure in a mantoux test?
Diameter of induration
112
What monoclonal antibody targets IL-23 and IL-12 and is used in the treatment of psoriasis or psoriatic arthritis?
Ustekinumab
113
Which cytokine is important in the pathogenesis of rheumatoid arthritis, anyklosing spondylitis and inflammatory bowel disease?
Tumour Necrosis Factor Alpha
114
What HLA allele is associated with Systemic Lupus Erythematosus?
HLA-DR3
115
Where are IgA antibodies typically found in the body?
Mucosal surfaces
116
Autoantibodies directed against Fibrillarin and Scl70 are classically associated with what autoimmune condition?
Diffuse Cutaneous Systemic Sclerosis
117
In haemolytic disease of the newborn, what maternal class of immunoglobulin is responsible for destruction of neonatal erythrocytes?
IgG
118
What is the maximum number of viral capsids that a molecule of secretory IgA may bind to?
4
119
What class of medication acts by inhibiting phospholipase A2 and reduces the expression of pro-inflammatory cytokines?
Corticosteroids
120
What serine protease released by neutrophils causes hepatitis and emphysema in patients deficient in alpha-1 antitrypsin?
Neutrophil elastase
121
List 6 type 2 hypersensitivity diseases
Pernicious anaemia (anti parietal cell antibodies)
Graves' disease (anti-TSH receptor)
ITP (anti-platelet antibodies such as anti-glyoprotein IIb/IIIa)
Goodpasture's syndrome (Anti-GBM antibodies [specifically anti-type IV collagen antibodies])
Haemolytic disease of the newborn (maternal IgG against fetal erythrocyte antigens)
Pemphigus Vulgaris (anti-cadherin)
122
Which interleukins are classically responsible for inducing fever?
IL-1 and IL-6
123
Which type of immunoglobulin is highly specific and is produced more than 5 days after an inital infection?
IgG
124
2 examples of NRTIs?
Zidovudine
| Abacavir
125
Example of NNRTI?
Efavirenz
126
Example of protease inhibitor?
Ritonavir
127
Example of integrase inhibitor?
Dolutegravir
128
What primary immunodeficiency is suggested in a patient with recurrent infections with E Coli, Staphylococcus Aureus, Listeria spp, Klebiella spp, Serratia marcescens and Candida species?
Chronic Granulomatous Disease
129
What immune cell releases granzyme and perforin when activated?
CD8+ T cell
130
What is the target of the autoantibodies responsible for pemphigus vulgaris?
Cadherin
131
What test can be used to measure the levels of serum IgE directed against brazil nut antigens?
Radioallergosorbent test
132
Which arm of the complement pathway is implicated in the pathogenesis of systemic lupus erythematosus?
Classical
133
What autoantibody is associated with Granulomatosis with Polyangiitis (Wegner's Granulomatosis)?
c-ANCA
134
What gene is defective in Bruton's agammagobulinaemia?
BTK gene
135
Immunoglobulins may bind to multiple pathogens at once in order to enhance phagocytosis.
What is this process known as?
Agglutination
136
What autoimmune condition is associated with AMA?
PBC
137
Which subtype of T helper cells are implicated in the development of allergic diseases, such as asthma or eczema?
Th2
138
What monoclonal antibody targets the IL-6 receptor and is involved in the treatment of rheumatoid arthritis?
Tocilizumab
139
What monoclonal antibody targets alpha4 integrin and is used in the treatment of relapsing remitting multiple sclerosis?
Natalizumab
140
What monoclonal antibody targets IL2a-receptor (CD25) and is used in the prophylaxis and treatment of allograft rejection? How does it do this?
Basiliximab
| Reduces T cell proliferation
141
What antigen does the immune system erroneously target in autoimmune thrombocytopenic purpura?
Glycoprotein IIb/IIIa
142
What drug is given prophylactically to infants at high risk of developing life threatening RSV infection?
Palivizumab
143
What is the maximum number of viral capsids that a molecule of IgM may bind to?
10
144
What is the minimum number of Fc receptors on the surface of mast cells that must bind to an antigen to cause mast cell degranulation?
2
145
What lymphocyte lineage does cyclophosphamide predominantly inhibit?
B cells
146
A woman is undergoing investigation for a suspected primary immunodeficiency. An initial blood test revealed a markedly low neutrophil count. However, a repeat blood test two weeks later was normal. What primary immunodeficiency is this patient likely to have?
Cyclic neutropenia
147
What hematological cells create a "respiratory burst" in order to kill phagocytosed pathogens?
Neutrophils
148
What primary immunodeficiency is a patient with a negative dihydrorhodamine (DHR) test likely to have?
Chronic granulomatous disease
149
What autoantibody is associated with Microscopic Polyangitis?
P-ANCA
150
CD19 is typically a marker of what immune cell?
B cell
151
An alcoholic patient presents to their GP for the first time following years of excessive drinking and poor self care.
The GP undertakes a number of investigations, including a blood film. What neutrophil abnormality would you expect to see?
Hypersegmented
152
Describe febrile non-haemolytic reaction to whole blood products and why it occurs.
Symptoms include a fever >38 in mild cases or a high fever >39 with chills and rigors in severe reactions
Caused by white blood cells releasing cytokines such as IL-1 during storage
153
How do you treat febrile non-haemolytic reaction to whole blood products?
Paracetamol
154
In skin prick testing, what is used as a positive control?
Histamine
155
An ANA test using indirect immunofluorescence suggests a homogenous pattern of ANA staining. What autoantibody is this pattern of ANA staining associated with?
anti-dsDNA
156
An ANA test using indirect immunofluorescence suggests a speckled pattern of ANA staining. What autoantibody is this pattern of ANA staining associated with?
anti-centromere antibodies
157
Diagnostic criteria of Common Variable Immunodeficiency? (3)
Decrease in serum IgG and a decrease in one of IgM or IgA
There is a lack of antibody response to antigens or immunisation
More than 4 years old
158
Treatment of common variable immunodeficiency?
IVIG for life
159
What is the gold-standard test for diagnosis of food allergy?
Double Blind Oral Food Challenge
160
What specific malignancy are people with Sjogren's syndrome at greater risk of?
Mucosa Associated Lymphoid Tissue Lymphoma
161
CD14 is typically a marker of what type of immune cell?
Monocytes
162
What immunoglobulin subtype binds to Fc receptors on the surface of mast cells?
IgE
163
What type of hypersensitivity reaction is Type 1 Diabetes?
Type IV
164
What auto-antibody is associated with Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)?
p-ANCA
165
A 15 day old infant is brought to A&E by their concerned parents. He has been feeding poorly (<80% of normal) and is producing fewer wet nappies. He is quite sleepy.
On examination there are multiple discharging abscesses approximately half a centimeter in diameter visible around his umbilical cord stump. They are swollen and very red. The child's father says his dad has a problem with white blood cells and takes injections for it. He is unsure what it is. The child is pyrexial at 38c and has a heart rate of 150bpm. A full blood count reveals a low neutrophil count. He was born at full term. What primary immunodeficiency is this child likely to have?
Kostmann Syndrome
166
A severe asthmatic presents to their GP for the third time in three years complaining of blocked sinuses. However, over the last 6 months, she has also had unintentional weight loss and reports intermittent fevers. She also has an indistinct erythematous macular rash on both her ankles. A Full Blood Count reveals an eosinophilia (>10% of white blood cells). What rare immunological condition may be responsible for her symptoms?
Eosinophilic Granulomatosis with Polyangiitis
167
A tumour cell has mutated and no longer expresses endogenous peptides and MHC Class I on the surface. What immune cell would recognise and kill this mutated cell?
Natural Killer cells
168
What is the most common inherited primary immunodeficiency?
Selective IgA Deficiency
169
A 34 year old man has severe Crohn's disease. His gastroenterologist wishes to start an anti-TNF drug to see if it controls his symptoms better. What first-line test should the patient undergo before starting adalimumab?
Mantoux Test
170
A 31 year old woman presents to their GP with tiredness and offensive smelling diarrhoea but no blood. Since the diarrhoea has started, she has complained of recurrent bouts of skin infections and food poisoning like symptoms. She is anti-TTG and anti-endomyseasal antibody positive. A full STI screen is negative. FBC is unremarkable. Albumin and Immunoglobulins are low. What is the reason for her recurrent infections?
Protein losing enteropathy
171
How do you diagnose a protein losing enteropathy?
faecal alpha-1 antitrypsin levels
172
An autoimmune panel of bloods reveals positive anti-Jo-1 antibody in a woman with breast cancer. What autoimmune condition is associated with this autoantibody?
Dermatomyositis
173
Which type of immunoglobulin is primarily secreted into breast milk and is responsible for passive immunity in newborns?
IgA
174
Immunoglobulins may bind to bacteria to allow phagocytosis to occur. What is this process known as?
Opsonisation
175
What enzyme, deficient or defective in chronic granulomatous disease, is responsible for the "respiratory burst" that may kill phagocytosed pathogens?
NADPH oxidase
176
Immunoglobulins may bind to cell surface receptors on viruses, to prevent viral binding and entry of human cells.
What is this function of immunoglobulins known as?
Neutralisation
177
What CD number do T reg cells have on their surface and what transcription factor do they express?
FOXP3
| CD25
178
What protein is defective in X linked Severe Combined Immunodeficiency?
Common Gamma Chain
179
An prenatal autoimmune panel of bloods reveals positive anti-Ro antibody in the blood of a pregnant woman with SLE. What cardiological condition is her unborn baby at risk of?
Congenital Heart Block
180
A 33 year old man is seen by the rheumatologists for evaluation of his raynaud's syndrome. He says his hands are becoming more sensitive to changes in temperature. In addition, the skin is cracked and dry. During the appointment, he is constantly sipping water from a water bottle. An autoimmune antibody screen reveals positive ANA (Anti-Ro, Anti-La positive). What autoimmune condition explains his symptoms?
Sjogren's Syndrome
181
What immunomodulatory agent may be given to treat chronic granulomatous disease?
Interferon Gamma
182
A 10 year old has severe hayfever. Twice in the last month she has complained to her parents of tingly lips after eating celery. She also says the same thing happens when she eats apples. What allergic condition is the cause of her symptoms?
Oral Allergy Syndrome
183
A mutation in what protein is the most common cause of Hyper IgM syndrome?
CD40 ligand
184
What monoclonal antibody targets CD52 and causes depletion of all lymphocyte subsets?
Alemtuzumab
185
Congenital absence of C4 (Complement) predisposes to what autoimmune disease?
SLE
| Also absence of C2 or C3
186
How may mast cell degranulation be measured?
Tryptase
187
What are the pathogenic antibodies in bullous pemphigoid?
Anti-Hemidesmosome
188
Mutation in what cell surface receptor may confer immunity from HIV?
CCR5
189
Clinical symptoms of Eosinophilic Granulomatosis with Polyangiitis?
Patients affected typically have severe allergies or sinus problems, which progress to severe asthma prior to the systemic vasculitis.
190
Clinical features of SCID?
Recurrent infections
Diarrhoea
Failure to thrive
Skin disease
191
Autosomal recessive SCID is caused by a mutation in which gene?
Adenosine deaminase
192
Causes of protein losing enteropathy?
Crohn's
Coeliac
Menetrier's disease
193
Where is the mutation in cyclic neutropenia and what is the inheritance?
Neutrophil elastase gene (ELA2)
| AD
194
Features of Wiskott-Aldrich syndrome?
Lymphoma
Thrombocytopenia - easy bruising, nose bleeds, GI bleeds
Eczema
Recurrent bacterial infections
195
Blood test results for Wiskott-Aldrich syndrome (in terms of Ig)
Decreased IgM
Raised IgA and IgE
Variable IgG
196
Where is the mutation in CVID and what does it cause?
```
MHC III
Causes aberrant class switching - leads to infection with H. influenzae and Strep pneumoniae
```
197
When does CVID present?
In adulthood
198
What happens in bare lymphocyte syndrome type 1 and 2
Type 1: MHC Class I deficiency - all T cells become CD4+
| Type 2: MHC Class II deficiency - all T cells become CD8+
199
Clinical features of bare lymphocyte syndrome?
Sclerosing cholangitis
Hepatomegaly
Jaundice
200
What is the role of IgA?
Provides mucosal immunity mainly to respiratory and GI system
201
Clinical features of selective IgA deficiency?
Recurrent Mild respiratory and GI infections
202
What does deficiency in common pathway of complement predispose you to?
Infection with encapsulated bacteria
203
What does deficiency in interferon gamma receptor predispose you to?
Intracellular infection e.g. TB and salmonella
204
What monoclonal mouse ab used to be used to prevent allograft rejection and what did it bind to?
Muromonab (OKT3)
| CD3
205
What is Felty's syndrome?
```
Extra-articular manifestations of RA:
Pulmonary fibrosis
Pericardial effusion
Rheumatoid nodules
Splenomegaly
```
206
What Ab is seen in dermatomyositis?
Anti-Jo1
207
Clinical features of dermatomyositis?
Heliotrope rash
Gottron's papules
Proximal myopathy
Pulmonary fibrosis
208
What type of hypersensitivity reaction is extrinsic allergic alveolitis?
Type III
209
What type of hypersensitivity reaction is AIHA?
Type II
210
Triggers of AIHA?
```
Idiopathic
SLE
CLL
Methyldopa
Penicillin
```
211
What type of hypersensitivity reaction is polyarteritis nodosa?
Type III
212
What investigation results would you expect to see in polyarteritis nodosa?
Raised CRP, ESR and Ig
p-ANCA
Multiple aneurysms
213
What hypersensitivity reaction is occuring in ITP and what is it directed at?
Type 2
| IgG targets GlpIIb-IIIa
214
What foods are associated with oral allergy syndrome?
Stony fruits
215
SE of cyclophosphamide?
Bone marrow suppression
Hair loss
Bladder cancer
216
Indications for cyclophosphamide therapy?
SLE
connective tissue disease
vasculitis (Wegener's)
217
What is abatacept and what is it used to treat?
CTLA-4-immunoglobulin fusion protein
| RA
218
SE of abatacept?
Infection from TB, HBV, HCV
219
MOA of abatacept?
Binds to CD80 and CD86 on surface of APCs and prevents APCs from delivering co-stimulatory molecule to T cells to activate them
220
SE of Mycophenolate mofetil?
Bone marrow suppression
| HSV reactivation
221
What does efalizumab bind to and what does this cause? What is it used to treat?
CD11a on T cells
Inhibits T cell migration
Psoriasis
222
What does ustekinumab bind to and what does this cause? What is it used to treat?
IL12 and IL23
Prevents T cell and NK cell activation
Psoriatic arthritis
223
Triad in wegener's?
Epistaxis
Haemoptysis
Haematuria
224
When does IgA nephropathy occur?
After GI or URTI
225
What are the 3 types of rapidly progressive glomerulonephritis and what is seen on IgG/C3 immunofluorescence?
Immune complex disease - granular staining
Pauci-immune disease - absent/scant staining
Anti-GBM disease - linear staining
226
What is there activation of in membranoproliferative glomerulonephritis?
Classical and alternative complement pathway
227
What test is used to detect sarcoidosis?
Kveim test
| Injection of spleen from known sarcoid patient followed by non-caseating granuloma formation 4-6 weeks later
228
What does a high CH50 indicate?
Acute or chronic inflammation
229
Features of APECED?
```
Mild immune deficiency
Dysfunctional parathyroid + adrenal
Hypothyroid
Gonadal failure
Alopecia
Vitiligo
```
230
Pathological mechanism of post-step rheumatic fever?
Molecular mimicry
231
Mechanism of mycoplasma causing AIHA?
T cell bypass and modification of RBC surface antigens
232
In SLE which falls first C3 or C4?
C4
233
Which patients are blood transfusions contraindicated in?
SCID
234
Clinical features of Bare lymphocyte syndrome?
Sclerosing cholangitis with hepatomegaly and jaundice
235
What is WAS associated with?
Lymphoma (causes lymphopenia)
Thrombocytopenia
Eczema
236
Release of which cytokine from T cells activates macrophages?
Interferon gamma
237
What does IFN-g deficiency increase risk of?
Intracellular organism infection e.g. TB and salmonella
| Since granulomas can't form
238
What infection are people with hyper IgM syndrome at risk of?
PCP infection
239
Clinical features of CVID?
Bronchiectasis
| Sinusitis
240
In a type 2 hypersensitivity reaction what do IgG and IgM stimulate?
Classical complement pathway
241
What do acantholytic cells on biopsy suggest?
| Which antibody is involved?
Pemphigus vulgaris
| Anti-demoglein
242
Explain the steps of type 4 hypersensitivity
1. APCs (macrophages) engulf antigen and present to CD4 which binds
2. Macrophage then releases IL12 - making memory Th1 cells
3. On second exposure to allergen macrophage presents it to CD4 cells
4. Memory Th1 cells release IFNg, IL2 and IL3
5. These cytokines activate TNF-a production by macrophages
243
Proteins involved in MS?
Myelin basic protein
| Proteolipid protein
244
Define isograft
From identical twin
245
When does acute cellular rejection occur after transplant?
1 week after
246
When does acute vascular rejection occur after transplant? With which type of graft?
4-6 days after
| Xenograft
247
Daclizumab? Used for what?
```
IL2 antibody (CD25)
Kidney transplant to prevent rejection
```
248
What is interferon alpha used to treat?
Hep B
Hep C
Kaposi's sarcoma
CML
249
What is interferon beta used to treat?
MS
250
What is interferon gamma used to treat?
chronic granulomatous disease
251
What does homogenous staining on immunofluorescence suggest?
Anti-histone: drug induced SLE
252
What does nucleolar staining on immunofluorescence suggest?
Anti-RNA polymerase: systemic sclerosis
253
What does peripheral staining on immunofluorescence suggest?
Anti-dsDNA: SLE
254
What does speckled staining on immunofluorescence suggest?
Anti-Jo: dermatomyositis
Anti-Smith: SLE
Anti-RNP: mixed connective tissue disease
Anti-Ro: Sjogren's
255
What causes the fibrosis in limited systemic sclerosis?
TNF-beta
256
Features of Kearns-Sayre syndrome?
```
Ptosis and difficulty with eye movement
Pigmentary retinopathy
Proximal muscle weakness
Cardiac conduction defects
Hearing loss
Cerebellar ataxia
Endocrine: hypoparathyroid, hypogonad, DM, hypopituitary
```
257
Features of IPEX?
DM
Eczema
Enteropathy
258
Biopsy findings of autoimmune hep?
Interface hepatitis
259
Autoimmune hep type 1 vs 2?
Type 1: Adults, anti-SMA, ANA
| Type 2: kids, anti-LKM, steroid resistant, associated with IgA deficiency
260
What is seen on immunofluorescence of focal segmental glomerulonephritis?
IgM and C3 deposition
261
What is seen on immunofluorescence of membranous glomerulonephritis? Also post-strep glomerulonephritis
Sub-epithelial granular deposits of IgG and C3
262
Most abundant immunoglobulin in breast milk?
IgA
