Immunology Flashcards
Questions from Blackboard and Meeran's book
1
Q
Choose the most appropriate answer from the list: X-Linked Agammaglobulinaemia: A. Chromosome 22q11 B. Bruton’s tyrosine kinase (Btk) gene C. CD40 Ligand gene D. MHC Class II E. IL12 gene F. IFN Receptor 1 gene G. WASP gene H. CD3 mutation I. IL-2 receptor
A
B. Bruton’s tyrosine kinase (Btk) gene
2
Q
Choose the most appropriate answer from the list: DiGeorge’s Syndrome A. Chromosome 22q11 B. Bruton’s tyrosine kinase (Btk) gene C. CD40 Ligand gene D. MHC Class II E. IL12 gene F. IFN Receptor 1 gene G. WASP gene H. CD3 mutation I. IL-2 receptor
A
A. Chromosome 22q11
3
Q
Choose the most appropriate answer from the list: Severe Combined Immunodeficiency A. Chromosome 22q11 B. Bruton’s tyrosine kinase (Btk) gene C. CD40 Ligand gene D. MHC Class II E. IL12 gene F. IFN Receptor 1 gene G. WASP gene H. CD3 mutation I. IL-2 receptor
A
I. IL-2 receptor
4
Q
Choose the most appropriate answer from the list: Wiskott-Aldrich Syndrome A. Chromosome 22q11 B. Bruton’s tyrosine kinase (Btk) gene C. CD40 Ligand gene D. MHC Class II E. IL12 gene F. IFN Receptor 1 gene G. WASP gene H. CD3 mutation I. IL-2 receptor
A
G. WASP gene
5
Q
Choose the most appropriate answer from the list: Bare lymphocyte syndrome A. Chromosome 22q11 B. Bruton’s tyrosine kinase (Btk) gene C. CD40 Ligand gene D. MHC Class II E. IL12 gene F. IFN Receptor 1 gene G. WASP gene H. CD3 mutation I. IL-2 receptor
A
D. MHC Class II
6
Q
This region encodes C2, C4 and factor B A. IFN Receptor 1 gene B. Adenosine Deaminase (ADA) gene C. WASP gene D. IL-2 receptor E. Bruton’s tyrosine kinase (Btk) gene F. Chromosome 22q11 G. CD40 Ligand gene H. MHC Class III I. IL12 gene J. CD3 mutation
A
H. MHC Class III
7
Q
A 25 year old woman comes to her GP about family planning. She is worried because she had an older brother who died before she was born and her grandmother lost two children which she things were both boys. Her GO thinks there may be a genetic disorder in her family affecting the IL-2 receptor. If correct she has a 50% of inheriting the trait from her mother and being a carrier herself. And there would be a 50% chance of passing it to her children. If inherited, her daughters would be carriers and her sons would require treatment which is usually a bone marrow transplant but gene therapy is sometimes used.
A. Bruton’s X linked hypogammaglobulinaemia
B. Common variable immune deficiency
C. Hyper IgM syndrome
D. TAP deficiency
E. Bare lymphocyte syndrome
F. Severe combined immunodeficiency (SCID)
G. Selective IgA deficency
H. DiGeorge syndrome
I. Reticular dysgenesis
A
F. Severe combined immunodeficiency (SCID)
8
Q
A jaundiced 8 month old child presents with failure to thrive, and a history of recurrent infections (viral, bacterial and fungal). On examination there is hepatomegally and blood tests show a raised alk phos and low CD4 count. A defect is found in the proteins that regulate MHC Class II transcription.
A. Bruton’s X linked hypogammaglobulinaemia
B. Common variable immune deficiency
C. Hyper IgM syndrome
D. TAP deficiency
E. Bare lymphocyte syndrome
F. Severe combined immunodeficiency (SCID)
G. Selective IgA deficency
H. DiGeorge syndrome
I. Reticular dysgenesis
A
E. Bare lymphocyte syndrome
9
Q
Patient X’s GP writes inquiring about whether to vaccinate. The patient suffers from recurrent respiratory tract infections and has been diagnosed with one of the B-cell maturation defects. For which one is immunisation still effective?
A. Bruton’s X linked hypogammaglobulinaemia
B. Common variable immune deficiency
C. Hyper IgM syndrome
D. TAP deficiency
E. Bare lymphocyte syndrome
F. Severe combined immunodeficiency (SCID)
G. Selective IgA deficency
H. DiGeorge syndrome
I. Reticular dysgenesis
A
G. Selective IgA deficency
10
Q
For which disorder would a bone marrow transplant be unhelpful but a thymic transplant may provide a cure?
A. Bruton’s X linked hypogammaglobulinaemia
B. Common variable immune deficiency
C. Hyper IgM syndrome
D. TAP deficiency
E. Bare lymphocyte syndrome
F. Severe combined immunodeficiency (SCID)
G. Selective IgA deficency
H. DiGeorge syndrome
I. Reticular dysgenesis
A
H. DiGeorge syndrome
11
Q
A 4-month-old girl is referred to a paediatrician with failure to thrive, after suffering from recurrent infections since birth, especially recurrent candida infections of her skin and mouth. Blood tests reveal a diminished T-cell count; further lymphocyte testing demonstrates non-functional B cells. A Kostmann syndrome B Severe combined immunodeficiency C Hyper IgM syndrome D Leukocyte adhesion deficiency E Protein-losing enteropathy F Cyclic neutropenia G Bruton’s agammaglobulinaemia H Di George’s syndrome I AIDS
A
B Severe combined immunodeficiency
12
Q
A 5-month-old boy is referred to a paediatrician after suffering with recurrent infections since his birth. His mother has noticed increased irritability. Blood tests reveal a neutrophil count of 350/μL. NBT test is normal. A Kostmann syndrome B Severe combined immunodefi- ciency C Hyper IgM syndrome D Leukocyte adhesion deficiency E Protein-losing enteropathy F Cyclic neutropenia G Bruton’s agammaglobulinaemia H Di George’s syndrome I AIDS
A
A Kostmann syndrome
13
Q
A 4-year-old girl is referred to a paediatrician after experiencing recurrent chest infections. Blood tests demonstrate a reduced B-cell count as well as low IgA, IgM and IgG levels. A Kostmann syndrome B Severe combined immunodefi- ciency C Hyper IgM syndrome D Leukocyte adhesion deficiency E Protein-losing enteropathy F Cyclic neutropenia G Bruton’s agammaglobulinaemia H Di George’s syndrome I AIDS
A
G Bruton’s agammaglobulinaemia
14
Q
A 48-year-old woman presents to her GP with a history of diarrhoea for 3 weeks, which occasionally contains blood. She has felt increasingly tired and feverish. The patient has had similar episodes in the past which were treated with mesalazine. She also reports recurrent chest infections since her first epi- sode of diarrhoea. A Kostmann syndrome B Severe combined immunodefi- ciency C Hyper IgM syndrome D Leukocyte adhesion deficiency E Protein-losing enteropathy F Cyclic neutropenia G Bruton’s agammaglobulinaemia H Di George’s syndrome I AIDS
A
E Protein-losing enteropathy
15
Q
A 3-year-old girl is seen by a GP due to recurrent mild chest infections. The doctor notices the girl has a cleft lip. Blood tests reveal a reduced T-cell count as well as hypocalcaemia. A Kostmann syndrome B Severe combined immunodefi- ciency C Hyper IgM syndrome D Leukocyte adhesion deficiency E Protein-losing enteropathy F Cyclic neutropenia G Bruton’s agammaglobulinaemia H Di George’s syndrome I AIDS
A
H. Di George’s syndrome
16
Q
A 4-year-old boy is referred to a paediatrician after suffering recurrent chest infections over the preceding few months. The boy has a history of eczema as well as recurrent nose bleeds. Blood tests reveal a reduced IgM level but raised IgA and IgE levels. A Selective IgA deficiency disease B Common variable immuno- deficiency C Nephrotic syndrome D Bare lymphocyte syndrome deficiency E Sickle cell anaemia F Chronic granulomatous G Reticular dysgenesis H Wiskott–Aldrich syndrome I Interferon-gamma receptor
A
H Wiskott–Aldrich syndrome
17
Q
A 20-year-old man presents to his GP with signs of a mild pneumonia. The patient states he has had several similar episodes in the past. Further investigations by an immunologist reveal the patient has a genetic condition caused by a mutation of MHC III. A Selective IgA deficiency disease B Common variable immuno- deficiency C Nephrotic syndrome D Bare lymphocyte syndrome deficiency E Sickle cell anaemia F Chronic granulomatous G Reticular dysgenesis H Wiskott–Aldrich syndrome I Interferon-gamma receptor
A
B Common variable immuno-
deficiency
18
Q
A 3-year-old girl is referred to a paediatrician after concerns about recurrent skin infections she has suffered from since birth. A nitro-blue-tetrazolium test is negative (remains colourless). A Selective IgA deficiency disease B Common variable immuno- deficiency C Nephrotic syndrome D Bare lymphocyte syndrome deficiency E Sickle cell anaemia F Chronic granulomatous G Reticular dysgenesis H Wiskott–Aldrich syndrome I Interferon-gamma receptor
A
F Chronic granulomatous
19
Q
A 4-year-old boy is referred to a paediatrician after a period of mild but chronic diarrhoea. On examination the child is found to have icteric sclera and hepatomegaly. Following blood tests, the doctor has a high suspicion that the child could have a defect in MHC I. A Selective IgA deficiency disease B Common variable immuno- deficiency C Nephrotic syndrome D Bare lymphocyte syndrome deficiency E Sickle cell anaemia F Chronic granulomatous G Reticular dysgenesis H Wiskott–Aldrich syndrome I Interferon-gamma receptor
A
D Bare lymphocyte syndrome
deficiency
20
Q
A 22-year-old woman visits her GP after several chest infections in the past few years. As well as the chest infections, the patient reports that she has had several bouts of diarrhoea over the same time period. A Selective IgA deficiency disease B Common variable immuno- deficiency C Nephrotic syndrome D Bare lymphocyte syndrome deficiency E Sickle cell anaemia F Chronic granulomatous G Reticular dysgenesis H Wiskott–Aldrich syndrome I Interferon-gamma receptor
A
A Selective IgA deficiency disease
21
Q
A 2-year-old girl is seen by an infectious disease paediatrician after suffering recurrent infections since she was born. Her neutrophil count is normal. A nitro- blue-tetrazolium (NBT) test is performed, which remains colourless. What is the diagnosis? A Kostmann syndrome B Cyclic neutropenia C Leukocyte adhesion deficiency D Chronic granulomatous disease E Von Gierke’s disease
A
D Chronic granulomatous disease
22
Q
A 29-year-old woman presents to her GP with recent onset joint pain and tiredness. On examination she has a malar rash. Further blood tests reveal she is anti- nuclear antibody and anti-double stranded DNA positive. Which component of the complement system is she most likely to be deficient in? A C3 B C4 C C6 D C9 E C1 inhibitor
A
B C4
23
Q
A 4-year-old girl is seen by a paediatrician to investigate possible developmental delay and learning difficulties. Initial blood tests reveal hypocalcaemia, reduced CD4+ and CD8+ T-cell counts as well as deficiency in IgG and IgA. FISH analysis reveals the child has a deletion of 22q11. What is the diagnosis? A Di George’s syndrome B Severe combined immunodeficiency C Bare lymphocyte syndrome D Wiskott–Aldrich syndrome E Interferon-gamma receptor deficiency
A
A Di George’s syndrome
24
Q
A 24-year-old man with a history of coeliac disease visits his GP after several bouts of chest and gastrointestinal infections in the past few years. Although the infections are mild, the patient is worried about the cause. What is the diagnosis? A Severe combined immunodeficiency B Bruton’s agammaglobulinaemia C Hyper IgM syndrome D Selective IgA deficiency E Common variable immunodeficiency
A
D Selective IgA deficiency
25
Q
A 40-year-old man is referred to an infectious disease specialist after he is admitted to hospital with Pneumocystis jerovicci pneumonia. On examination the patient also has multiple Kaposi’s sarcoma lesions on his chest and abdomen. What is the most likely diagnosis? A Inflammatory bowel disease B Hyposplenism C Nephrotic syndrome D AIDS E Prematurity
A
D AIDS
26
Q
A 56-year-old woman presents to the rheumatologist with pain in her hands. On examination there are obvious deformities of her proximal interphalyngeal joints and metacarpophalyngeal joints. Swan-neck deformities are seen but the patient has retained functionality of her fingers. A. Anti-smooth muscle B. p-ANCA C. Anti-Jo1 D. Anti-cyclic citrullinated protein E. Anti-centromere F. Anti-double stranded DNA G. Anti-parietal cell H. Anti-thyroid stimulating hormone I. Anti-topoisomerase
A
D Anti-cyclic citrullinated protein
27
Q
A 45-year-old woman is referred to a hepatologist after suffering an episode of jaundice, fatigue and fever. Liver function tests reveal an increased AST. Biopsy of the liver reveals cirrhosis and an autoimmune pathology is suspected. A. Anti-smooth muscle B. p-ANCA C. Anti-Jo1 D. Anti-cyclic citrullinated protein E. Anti-centromere F. Anti-double stranded DNA G. Anti-parietal cell H. Anti-thyroid stimulating hormone I. Anti-topoisomerase
A
A. Anti-smooth muscle
28
Q
A 42-year-old woman presents to the rheumatologist with weakness in her proximal muscles and describes how she is finding it difficult to climb stairs. On examination, a rash is observed surrounding both eyes. A high resolution CT scan reveals a pulmonary fibrosis picture. A. Anti-smooth muscle B. p-ANCA C. Anti-Jo1 D. Anti-cyclic citrullinated protein E. Anti-centromere F. Anti-double stranded DNA G. Anti-parietal cell H. Anti-thyroid stimulating hormone I. Anti-topoisomerase
A
C. Anti-Jo1
29
Q
A 43-year-old man is referred to the rheumatologist after experiencing paleness in his fingers, especially when exposed to cold weather. The patient also complains of recent onset difficulty in swallowing solid food. A. Anti-smooth muscle B. p-ANCA C. Anti-Jo1 D. Anti-cyclic citrullinated protein E. Anti-centromere F. Anti-double stranded DNA G. Anti-parietal cell H. Anti-thyroid stimulating hormone I. Anti-topoisomerase
A
E. Anti-centromere
30
Q
A 42-year-old woman presents to the rheumatologist with joint pain and stiff- ness. On examination, the patient appears to have a tight mouth and fine end inspiratory crackles on auscultation of the lungs. The woman also has a wide- spread itchy rash on her body. A. Anti-smooth muscle B. p-ANCA C. Anti-Jo1 D. Anti-cyclic citrullinated protein E. Anti-centromere F. Anti-double stranded DNA G. Anti-parietal cell H. Anti-thyroid stimulating hormone I. Anti-topoisomerase
A
I. Anti-topoisomerase
31
Q
A 25-year-old woman presents to her GP with a dry mouth and eyes for a period of 2 weeks. The patient also complains of joint pains over this time-course. A Anti-mitochondrial B c-ANCA C Anti-cardiolipin D Anti-ribonucleoprotein E Anti-glutamic acid decarboxylase F Anti-Ro G Anti-nuclear H Anti-intrinsic factor I Anti-endomysial
A
F Anti-Ro
32
Q
A 52-year-old man is referred to a gastroenterologist with itchy skin and malaise. On examination, the man has bruising on his arms and legs. A Anti-mitochondrial B c-ANCA C Anti-cardiolipin D Anti-ribonucleoprotein E Anti-glutamic acid decarboxylase F Anti-Ro G Anti-nuclear H Anti-intrinsic factor I Anti-endomysial
A
A. Anti-mitochondrial
33
Q
A 10-year-old girl is brought to see a GP. Her mother describes how she has recently been urinating with greater frequency than previously as well as feel- ing thirsty and has lost several kilograms in weight in the recent weeks. A Anti-mitochondrial B c-ANCA C Anti-cardiolipin D Anti-ribonucleoprotein E Anti-glutamic acid decarboxylase F Anti-Ro G Anti-nuclear H Anti-intrinsic factor I Anti-endomysial
A
E Anti-glutamic acid decarboxylase
34
Q
A 42-year-old man presents to accident and emergency with haemoptysis. The patient also describes how he has been experiencing nose bleeds with increasing frequency in recent weeks. The patient is noted to have a saddle-shaped nose. A Anti-mitochondrial B c-ANCA C Anti-cardiolipin D Anti-ribonucleoprotein E Anti-glutamic acid decarboxylase F Anti-Ro G Anti-nuclear H Anti-intrinsic factor I Anti-endomysial
A
B c-ANCA
35
Q
A 22-year-old woman presents to her GP with recent onset diarrhoea and abdominal cramping after she has eaten meals containing wheat. A Anti-mitochondrial B c-ANCA C Anti-cardiolipin D Anti-ribonucleoprotein E Anti-glutamic acid decarboxylase F Anti-Ro G Anti-nuclear H Anti-intrinsic factor I Anti-endomysial
A
I Anti-endomysial
36
Q
An 11-year-old girl presents to the GP with increased thirst and urinary fre- quency. Urine dipstick demonstrates the presence of glucose. A TSH receptor B Nuts C DNA D Nickel E Type IV collagen F Chlamydia trachomatis G Mouldy hay H Grass pollen I Pancreatic beta cell proteins
A
I Pancreatic beta cell proteins
37
Q
A 13-year-old girl eats a slice of cake at a birthday party and quickly develops swollen lips, itchy skin and difficulty breathing. A shot of intramuscular adrena- line is immediately administered. A TSH receptor B Nuts C DNA D Nickel E Type IV collagen F Chlamydia trachomatis G Mouldy hay H Grass pollen I Pancreatic beta cell proteins
A
B Nuts
38
Q
A 56-year-old farmer presents to his GP with a 2-month history of worsen- ing shortness of breath. He mentions that he has experienced periodic fevers, malaise and mild shortness of breath, which has recently become so bad that he has had to stop work. A TSH receptor B Nuts C DNA D Nickel E Type IV collagen F Chlamydia trachomatis G Mouldy hay H Grass pollen I Pancreatic beta cell proteins
A
G Mouldy hay
39
Q
A 45-year-old man presents to accident and emergency with a sudden onset of haemoptysis. His wife mentions that the patient had noticed some blood in his urine a few days previously but had thought nothing of it. A TSH receptor B Nuts C DNA D Nickel E Type IV collagen F Chlamydia trachomatis G Mouldy hay H Grass pollen I Pancreatic beta cell proteins
A
E Type IV collagen
40
Q
A 12-year-old boy experiences a runny nose, itchiness of his eyes and nasal congestion. His GP suggests he has a seasonal condition, and should begin tak- ing anti-histamines to help relieve him of his symptoms. A TSH receptor B Nuts C DNA D Nickel E Type IV collagen F Chlamydia trachomatis G Mouldy hay H Grass pollen I Pancreatic beta cell proteins
A
H Grass pollen
41
Q
A 26-year-old woman at a work dinner has ordered a curry. Soon after eating the meal, she feels short of breath and wheezy. Her husband who is present swiftly administers an intramuscular shot of adrenaline. A Stony fruit B HBsAg C Myelin basic protein D Rhesus antigens E Glycoprotein IIb–IIIa F Peanuts G Antiserum H Synovial membrane antigens I Poison ivy
A
F Peanuts
42
Q
A 35-year-old woman presents to the GP with blurry vision and weakness in her legs. Cerebrospinal fluid demonstrates oligoclonal bands of IgG on electrophoresis. A Stony fruit B HBsAg C Myelin basic protein D Rhesus antigens E Glycoprotein IIb–IIIa F Peanuts G Antiserum H Synovial membrane antigens I Poison ivy
A
C Myelin basic protein
43
Q
A 34-year-old man who has been taking amoxicillin for pneumonia has devel- oped tiredness and palpitations since taking the medication. Blood tests reveal a normocytic anaemia and direct antiglobulin test is positive. A Stony fruit B HBsAg C Myelin basic protein D Rhesus antigens E Glycoprotein IIb–IIIa F Peanuts G Antiserum H Synovial membrane antigens I Poison ivy
A
D Rhesus antigens
44
Q
A 34-year-old man, who is a known intravenous drug user, presents to accident and emergency with a 1-week history of fever, fatigue and abdominal pain. The patient also has associated joint pain. An angiogram reveals the presence of multiple aneurysms. A Stony fruit B HBsAg C Myelin basic protein D Rhesus antigens E Glycoprotein IIb–IIIa F Peanuts G Antiserum H Synovial membrane antigens I Poison ivy
A
B HBsAg
45
Q
A 45-year-old man with diagnosed systemic lupus erythematosus (SLE) presents to the GP with a recent onset of nose bleeds and bleeding of his gums when he brushes his teeth. Blood tests reveal a very low platelet count. A Stony fruit B HBsAg C Myelin basic protein D Rhesus antigens E Glycoprotein IIb–IIIa F Peanuts G Antiserum H Synovial membrane antigens I Poison ivy
A
E Glycoprotein IIb–IIIa
46
Q
A 34-year-old man presents to his GP with fever, joint pain and a rash on his trunk. On examination, a new murmur is auscultated. Blood investigations reveal a raised anti-streptolysin O titre. What is the most likely mechanism for this disease process? A Defective immunoregulation B Molecular mimicry C T-cell bypass D Release of hidden self antigens E Cytokines
A
B Molecular mimicry
47
Q
A 12-year-old girl has developed a runny nose, itchy eyes and nasal congestion during the summer months for the past 4 years. She is prescribed anti-histamines to help her symptoms. Which of the following cells is responsible for the initial encounter with the allergen? A Mast cell B B cell C Macrophage D TH1 cell E TH2 cell
A
C Macrophage
48
Q
A 14-year-old girl with a history of eczema presents to accident and emergency with itching and tingling of her lips and tongue. The girl’s lips are evidently swollen. All observations are normal. The doctor believes her condition is due to cross-reactivity of allergens. What is the most likely trigger for her allergy? A Penicillin B Eggs C Nickel D Dust mite E Fruit
A
E Fruit
49
Q
A 21-year-old woman is at a Thai restaurant, eating her main course when she sud- denly develops shortness of breath, wheeze and swelling of her lips. The patient
has a known peanut allergy. What is the most appropriate treatment in the first instance?
A Allergen avoidance
B Adrenaline
C Oral anti-histamines
D Doxepin
E Nasal steroids
A
B Adrenaline
50
Q
A demanding mother takes her 6-year-old son to see the GP. She is concerned by his numerous allergies, including pollen and various foods. She is keen for her son to have allergy testing to determine the substances he is allergic to. Which of the following would be the best test for investigating allergy in this child? A Radioallergosorbent test B Skin prick test C Double-blind challenge D Serum tryptase levels E Total serum IgE
A
B Skin prick test
51
Q
A 56-year-old diabetic man is undergoing a kidney transplant as a result of chronic renal failure. After the operation, the man immediately develops fever and has no urine production. Background checks reveal there was an error in ABO matching of the donor and recipient; the donor’s blood group was A, while the recipient’s is O. Which of the following immune components is the first to initiate a response in this case? A Natural-killer cells B C1 C Neutrophil D Mannose binding lectin E Macrophages
A
B C1
52
Q
A 54-year-old woman is referred to a dermatologist after developing blisters which she first noticed in her mouth but have now appeared on her right arm. On examination, Nikolsky’s sign is positive and immunofluorescent staining demonstrates the presence of acantholytic cells. What is the most likely target for antibodies in this case? A Gastric parietal cell B Rhesus antigen C Acetylcholine receptor D Demoglein 1 E M proteins on group A streptococci
A
D Demoglein 1
53
Q
A 35-year-old woman presents to her GP with intermittent fatigue and joint pain which began 1 month previously. On examination, the patient has a malar rash on her face. Blood tests reveal anaemia. What is the most likely target for autoantibodies in this disease process? A Mouldy hay B Chlamydia trachomatis C DNA D Antiserum proteins E Hepatitis B virus antigen
A
C DNA
54
Q
A 34-year-old woman notices an itchy and desquamating, erythematous rash on her wrist, which has emerged approximately 3 days after wearing a new brace- let. Which cytokine is the first to be released during the initial exposure to the allergen? A IL-10 B IFN- C IL-2 D TNF- E IL-12
A
E IL-12
55
Q
A 56-year-old woman presents to her GP with blurry vision. On examination the woman has some bilateral weakness in her legs. The patient mentions that her vision seems to become more blurry just after she has had a bath. What is the most likely target in this disease process? A Pancreatic -cell proteins B Nickel C Proteolipid protein D Synovial membrane proteins E Tuberculin
A
D Synovial membrane proteins
56
Q
A 52-year-old woman presents to her GP with dry eyes and mouth for the past few weeks. Despite using moisturizer the woman also complains of dry skin. The patient has a history of coeliac disease. Which of the following antibodies is most likely to be diagnostic for this patient’s condition? A Anti-Jo1 B Anti-cyclic citrullinated protein C Anti-centromere D Anti-topoisomerase E Anti-Ro
A
E Anti-Ro
57
Q
A 42-year-old man is referred to the rheumatology outpatient clinic. The patient has been experiencing muscle and joint pain for the past month. On examination a heliotrope rash is observed on the patient’s eyelids. Blood tests reveal the patient has circulating anti-nuclear antibodies. Which immunofluorescence staining pattern will be observed in this disease process? A Homogeneous B Nucleolar C Speckled D Peripheral E Kinetoplast
A
C Speckled
58
Q
A 34-year-old woman, diagnosed with Chlamydia trachomatis infection 2 weeks previously, sees her GP after experiencing a 1-week history of joint pain and blurry vision. She also complains of a burning sensation when she passes urine. Blood tests reveal a raised CRP and ESR. A joint aspirate of her knee is however sterile. What is the most likely diagnosis? A Ankylosing spondylitis B Reactive arthritis C Enteropathic arthritis D Psoriatic arthritis E Anterior uveitis
A
B Reactive arthritis
59
Q
A 54-year-old woman is referred to a rheumatologist. The patient states that she has noticed her fingers becoming very pale on cold days; when she heats herhands against the radiator, she notices her hands becoming red. She mentions that she has also had joint pains in her hands. On inspection, the patient has a small mouth. Which of the following factors is most responsible for fibrosis in this disease process? A von Willebrand factor B IL-2 C TGF- beta D TNF- alpha E Endothelin-1
A
C TGF- beta
60
Q
A 12-year-old boy is referred to the paediatric endocrinology outpatient clinic after experiencing recent onset weight loss, tiredness, frequency of urination and thirst. A fasting plasma glucose test reveal a level of 10.1mmol/L and a diagno- sis of type 1 diabetes mellitus is made. Which of the following autoantibodies has tyrosine phosphatase as the target antigen?
A Islet cell surface antibody
B Insulin autoantibody
C Anti-glutamic acid decarboxylase antibody
D Anti-IA-2 antibody
E Islet cell antibody
A
D Anti-IA-2 antibody
61
Q
A 40-year-old woman presents to an endocrinologist with weight loss which has occurred over the past month, associated with a tremor, excessive sweating and a sense of feeling warm even on a cool day. On examination, the patient has exophthalmos and an irregularly irregular pulse. Which of the following autoantibodies is most likely to be responsible for the patient’s disease process? A Anti-TSH receptor (stimulating) B Anti-TSH receptor (non-stimulating) C Anti-thyroid peroxidase D Anti-thyroglobulin E Thyroid growth stimulating antibody
A
A Anti-TSH receptor (stimulating)
62
Q
A 10-year-old boy is referred to a paediatrician after experiencing a seizure 1 week previously. Blood tests reveal that the seizure may have occurred secondary to low calcium levels; blood glucose levels are found to be high. The child was already being investigated for ptosis and difficulty with eye movements. What is the most likely diagnosis? A Hirata’s disease B IPEX C Kearns–Sayre syndrome D POEMS syndrome E APECED syndrome type 1
A
C Kearns–Sayre syndrome
63
Q
A 6-year-old girl presents to accident and emergency with severe haematemesis, endoscopy revealing the presence of oesophageal varices. Blood tests reveal liver function test derangement and a low level of circulating IgA. Subsequent liver biopsy demonstrates interface hepatitis. Treatment with steroids shows a poor response. Which autoantibody is most likely to be present in this child?
A Anti-nuclear antibody B Anti-smooth muscle antibody C Anti-liver kidney microsomal antibody D Anti-mitochondrial antibody E Anti-HBs antibody
A
C Anti-liver kidney microsomal antibody
64
Q
A 24-year-old man is referred to a gastroenterologist following episodes of diar- rhoea in the last month. The patient also feels more tired than usual. The man undergoes a colonoscopy and jejunal biopsy results show villous hypertrophy with crypt hyperplasia and an increase in intraepithelial lymphocytes. Which of the following is associated with the greatest predisposition to developing this disease? A Dermatitis herpetiformis B Vitiligo C IgA deficiency D HLA DQ8 E HLA DQ2
A
E HLA DQ2
65
Q
A 52-year-old Mediterranean woman is referred to the dermatology outpatient clinic as a result of blisters that have developed in her mouth and on her arms. The patient describes the blisters as being very fragile and rupturing easily. Immunological testing reveals the presence of anti-desmoglein 3 antibodies and punch biopsy of a lesion demonstrates the presence of acantholytic cells. What is the most likely diagnosis? A Pemphigus foliaceous B Pemphigus vulgaris C Bullous pemphigoid D Epidermolysis bullosa E Dermatitis herpetiformis
A
B Pemphigus vulgaris
66
Q
An Afro-Caribbean man with a history of type 1 diabetes mellitus presents to the dermatology outpatient clinic with depigmented areas of his face, arms and legs. On examination the affected areas are completely white. The patient admits that the lesions are leading to low mood. Which of the following is most associated with this disease process?
A. Beta Haemolytic streptococcal infection
B Vancomycin
C Pregnancy
D Anti-melanocyte antibodies
E Multiple myeloma
A
D Anti-melanocyte antibodies
67
Q
A 4-year-old boy is referred to a renal physician after his mother noticed swell- ing of his legs. A week previously the boy had been stung by a bee. Urine dip- stick reveals the presence of proteinuria, while blood tests show hypoalbumin- aemia and hyperlipidaemia. The child’s symptoms rapidly disappear with a course of steroids. What is the most likely diagnosis? A Alport syndrome B Reflux nephropathy C Shunt nephritis D Systemic lupus erythematosus E Minimal change disease
A
E Minimal change disease
68
Q
A 24-year-old woman is seen by the GP after noticing she is urinating less often as well as seeing some blood when she does pass water. Urine investigations reveal the presence of red cell casts and dysmorphic red blood cells. The patient admits to having had a sore throat 2 weeks previously. Anti-streptolysin O titres are raised. What is the most likely diagnosis?
A IgA nephropathy
B Henoch–Schonlein purpura
C Post-streptococcal glomerulonephritis
D Membranoproliferative glomerulonephritis
E Rapidly progressive glomerulonephritis
A
C Post-streptococcal glomerulonephritis
69
Q
A 44-year-old man with known systemic lupus erythematosus is seen by a renal physician. Initially the patient had proteinuria on a routine urine dipstick. A subsequent renal biopsy demonstrated granular patterned deposition of IgG, IgM, IgA and C3 confined to the mesangium on both light and electron microscopy. Which stage of lupus nephritis is suggested by these findings? A Stage I B Stage II C Stage III D Stage IV E Stage V
A
B Stage II
70
Q
A 53-year-old man presents to accident and emergency with haemoptysis. Blood tests demonstrate deranged renal function and further tests reveal the presence of circulating c-ANCA antibodies. The patient is noted to have a saddle-shaped nose. What is the most likely diagnosis? A Cryoglobulinaemia B Wegener’s granulomatosis C Microscopic polyarteritis D Polyarteritis nodosa E Churg–Strauss syndrome
A
B Wegener’s granulomatosis
71
Q
A 35-year-old builder is referred to a neurologist after experiencing increas- ing axial rigidity over the previous few weeks; his symptoms are interfering with his work. The patient has a history of type 1 diabetes mellitus and vitiligo. Immunological investigations reveal the presence of circulating anti-glutamic acid decarboxylase antibodies. What is the most likely diagnosis? A Myasthenia gravis B Multiple sclerosis C Acute disseminated encephalomyelitis D Lambert–Eaton myasthenic syndrome E Stiff man syndrome
A
E Stiff man syndrome
72
Q
A 35-year-old man is transferred to the intensive care unit for ventilator support after suffering an episode of respiratory distress. The patient was admitted 5 days previously after experiencing weakness of his legs. Approximately 2 weeks prior to his admission the man had suffered a bout of gastroenteritis caused by the bacterium Campylobacter jejuni. Which of the following is the most likely antigenic target for autoantibodies in this disease process? A Ganglioside LM1 B Ganglioside GM1 C Hu D Myelin-associated glycoprotein E Purkinje cells
A
A Ganglioside LM1
73
Q
A 35-year-old woman is referred to an ophthalmologist after seeing floaters in her right eye. On examination, there is loss of accommodation in the same eye. The patient’s notes reveal there had been trauma to the left eye following a car accident 3 weeks previously. It is explained to the patient that she could suffer potential loss of vision if steroid treatment is not commenced urgently. What is the most likely diagnosis? A Keratoconjunctivitis sicca B Sympathetic ophthalmia C Uveitis D Keratitis E Scleritis
A
B Sympathetic ophthalmia
74
Q
A 52-year-old woman diagnosed with systemic lupus erythematosus develops jaundice and on examination is found to have conjunctival pallor. Blood tests reveal an elevated unconjugated bilirubin. Which of the following is the most useful investigation to determine the diagnosis? A Skin prick test B Direct antiglobulin test C Western blot D Immunofluorescence test E Patch testing
A
B Direct antiglobulin test
75
Q
A 48-year-old man has undergone a kidney transplant operation as a result of renal failure caused by long-standing diabetes mellitus. However, despite immunosuppression, signs of organ rejection become evident just 1 hour after the procedure. A HLA-matching B Corticosteroids C Cyclosporine A D Azathioprine E Sirolimus F OKT3 G IL-2 receptor antibody H Tacrolimus I Anti-lymphocyte antibody
A
A HLA-matching
76
Q
A 45-year-old man undergoes a heart transplant due to end-stage heart failure. Seventy-two hours after the operation, the patient shows signs of organ rejection which is resistant to corticosteroid therapy. A mouse monoclonal antibody is administered to save the transplant. A HLA-matching B Corticosteroids C Cyclosporine A D Azathioprine E Sirolimus F OKT3 G IL-2 receptor antibody H Tacrolimus I Anti-lymphocyte antibody
A
F OKT3
77
Q
A 32-year-old woman undergoes a bone marrow transplant for chronic lympho- blastic leukaemia. She is prescribed a medication that inhibits calcineurin. On examination, the patient has gum hyperplasia. A HLA-matching B Corticosteroids C Cyclosporine A D Azathioprine E Sirolimus F OKT3 G IL-2 receptor antibody H Tacrolimus I Anti-lymphocyte antibody
A
C Cyclosporine A
78
Q
A 62-year-old man who has undergone a kidney transplant was started on an immunosuppressive agent prior to the operation. The patient is warned that he will only be on the medication for a short period due to long-term side effects such as osteoporosis. A HLA-matching B Corticosteroids C Cyclosporine A D Azathioprine E Sirolimus F OKT3 G IL-2 receptor antibody H Tacrolimus I Anti-lymphocyte antibody
A
B Corticosteroids
79
Q
A 62-year-old man who is undergoing a liver transplant as a result of cirrhosis is prescribed a medication that inhibits DNA synthesis in an attempt to prevent proliferation of T cells. A HLA-matching B Corticosteroids C Cyclosporine A D Azathioprine E Sirolimus F OKT3 G IL-2 receptor antibody H Tacrolimus I Anti-lymphocyte antibody
A
D Azathioprine
80
Q
A 46-year-old man with long-standing SLE is seen by his rheumatologist. He had previously been treated with corticosteroids, but has now developed end- organ involvement of his kidneys, lungs and heart. A Cyclophosphamide B Mycophenolate mofetil C Basiliximab D Abatacept E Rituximab F Efalizumab G Infliximab H Ustekinumab I Denosumab
A
A Cyclophosphamide
81
Q
A 56-year-old woman is seen in the rheumatology outpatient clinic. She has long-standing rheumatoid arthritis, which despite treatment with methotrexate has become more severe. The rheumatologist decides that a CTL4- immunoglobulin fusion protein may help. A Cyclophosphamide B Mycophenolate mofetil C Basiliximab D Abatacept E Rituximab F Efalizumab G Infliximab H Ustekinumab I Denosumab
A
D Abatacept
82
Q
A 56-year-old man who is undergoing kidney transplant surgery is given medi- cation to prevent allograft rejection. The drug prevents guanine synthesis to induce immunosuppression. A Cyclophosphamide B Mycophenolate mofetil C Basiliximab D Abatacept E Rituximab F Efalizumab G Infliximab H Ustekinumab I Denosumab
A
B Mycophenolate mofetil
83
Q
A 58-year-old woman who suffers from rheumatoid arthritis is seen by her rheumatologist. She has been taking long-term disease modifying anti- rheumatic drugs, but her condition has recently worsened. As a result the doctor prescribes a TNF- inhibitor. A Cyclophosphamide B Mycophenolate mofetil C Basiliximab D Abatacept E Rituximab F Efalizumab G Infliximab H Ustekinumab I Denosumab
A
G Infliximab
84
Q
A 56 year old with known systemic lupus erythematosus has been treated with long-term steroids. The patient presents to a rheumatologist with back pain and a DEXA scan confirms osteoporosis. A Cyclophosphamide B Mycophenolate mofetil C Basiliximab D Abatacept E Rituximab F Efalizumab G Infliximab H Ustekinumab I Denosumab
A
I Denosumab
85
Q
A 50-year-old woman presents to accident and emergency with haematuria. Blood tests demonstrate deranged renal function and further tests reveal the presence of circulating cANCA antibodies. The patient is noted to have a saddle- shaped nose. A Minimal change disease B Wegener’s granulomatosis C Microscopic polyangitis D Lupus nephritis E IgA nephropathy F Membranoproliferative glomerulonephritis G Rapidly progressive glomerulonephritis H Post-streptococcal glomerulonephritis I Goodpasture’s syndrome
A
B Wegener’s granulomatosis
86
Q
A 24-year-old man presents to his GP with a few days’ history of blood in his urine. Urinary investigations reveal the presence of proteinuria, red and white cell casts and dysmorphic red cells. The patient’s notes state that he was diag- nosed with pharyngitis in the previous week. Blood tests reveal a raised IgA level. A. Minimal change disease B Wegener’s granulomatosis C Microscopic polyangitis D Lupus nephritis E IgA nephropathy F Membranoproliferative glomerulonephritis G Rapidly progressive glomerulonephritis H Post-streptococcal glomerulonephritis I Goodpasture’s syndrome
A
E IgA nephropathy
87
Q
A 25-year-old man presents to his GP with symptoms and signs of nephritic syndrome. The patient had a sore throat 2 weeks previously. Blood tests reveal anti-streptolysin titre is high, while IgA levels are normal. A. Minimal change disease B Wegener’s granulomatosis C Microscopic polyangitis D Lupus nephritis E IgA nephropathy F Membranoproliferative glomerulonephritis G Rapidly progressive glomerulonephritis H Post-streptococcal glomerulonephritis I Goodpasture’s syndrome
A
H Post-streptococcal glomerulonephritis
88
Q
A 65-year-old man with known renal failure is transferred to the renal team by the accident and emergency department with worsening renal function. A renal biopsy is taken which demonstrates the presence of crescents on histology; immunofluorescence staining of IgG/C3 reveals a granular pattern. The man is very ill with suggestions that he may require a renal transplant. A. Minimal change disease B Wegener’s granulomatosis C Microscopic polyangitis D Lupus nephritis E IgA nephropathy F Membranoproliferative glomerulonephritis G Rapidly progressive glomerulonephritis H Post-streptococcal glomerulonephritis I Goodpasture’s syndrome
A
G Rapidly progressive glomerulonephritis
89
Q
A 3-year-old boy is seen by the GP after his mother noticed swelling of his legs. A week previously the boy had been stung by a bee. Urine dipstick reveals the presence of proteinuria, while blood tests show hypoalbuminaemia and hyper- lipidaemia. A. Minimal change disease B Wegener’s granulomatosis C Microscopic polyangitis D Lupus nephritis E IgA nephropathy F Membranoproliferative glomerulonephritis G Rapidly progressive glomerulonephritis H Post-streptococcal glomerulonephritis I Goodpasture’s syndrome
A
A. Minimal change disease
90
Q
A 39-year-old homosexual man presents to accident and emergency with short- ness of breath and a dry cough. A chest X-ray shows widespread pulmonary opacification. PCR confirms the diagnosis of Pneumocystis pneumoniae infec- tion. A test is ordered to confirm the underlying diagnosis. A Histocompatibility testing B Immunofluorescence C Latex fixation test D Radioallergosorbent test E Patch testing F Kveim test G Skin prick test H Western blot I Direct antiglobulin test
A
H Western blot
91
Q
A 45-year-old man presents to accident and emergency with worsening short- ness of breath. Examination findings are consistent with pulmonary fibrosis. Chest X-ray demonstrates the presence of bihilar lymphadenopathy. Erythema nodosum is observed on the patient’s shins. A Histocompatibility testing B Immunofluorescence C Latex fixation test D Radioallergosorbent test E Patch testing F Kveim test G Skin prick test H Western blot I Direct antiglobulin test
A
F Kveim test
Tests for sarcoidosis
92
Q
A 50-year-old man with known SLE develops jaundice. On examination he is found to have conjunctival pallor and is short of breath. Blood tests reveal an elevated unconjugated bilirubin level. A Histocompatibility testing B Immunofluorescence C Latex fixation test D Radioallergosorbent test E Patch testing F Kveim test G Skin prick test H Western blot I Direct antiglobulin test
A
I Direct antiglobulin test
93
Q
A 12-year-old girl is referred to a paediatrician after suffering with allergies to a number of foods including peanuts and eggs. Her mother wants to check if she is allergic to any other foods, inhalants or specific materials, so that she can be prevented from coming into contact with potential allergens. A Histocompatibility testing B Immunofluorescence C Latex fixation test D Radioallergosorbent test E Patch testing F Kveim test G Skin prick test H Western blot I Direct antiglobulin test
A
G Skin prick test
94
Q
A 5-year-old boy presents to accident and emergency with purpura on his legs and buttocks, joint pain and abdominal pain. The boy’s mother states that the child had suffered from a sore throat approximately 1 week previously. The doctor would like to perform an investigation to make sure of the diagnosis. A Histocompatibility testing B Immunofluorescence C Latex fixation test D Radioallergosorbent test E Patch testing F Kveim test G Skin prick test H Western blot I Direct antiglobulin test
A
B Immunofluorescence
95
Q
A 10-year-old boy is seen by a paediatrician after suffering recurrent chest infections. His mother reports purulent sputum production and cough for the previous 2 years. Genetic testing reveals the child has a F508 mutation on chromosome 7. Which physical barrier to infection is most likely to be affected by the child’s condition? A Skin B Gastric acid C Mucociliary clearance D Tears E Gut flora
A
C Mucociliary clearance
96
Q
62-year-old woman sees her GP for a regular check-up. On examination, she has notable deformities of her hands, including swan-neck and Boutonniere deformities of her fingers. Blood tests reveal a raised CRP. Which of the follow- ing investigation results will most likely feature? A Reduced AH50 and normal CH50 B Reduced C1 inhibitor C Reduced C3 and C4 D Reduced C3 and normal C4 E High CH50
A
E High CH50
97
Q
A 25-year-old woman, who has a history of allergy to nuts, is taken to accident and emergency after eating a dessert containing peanuts. She has an evident wheeze with an increased respiratory rate, swelling of her lips and itchy skin. Which cell of the innate immune system is most likely to be responsible for her symptoms? A Natural-killer cells B Dendritic cells C Eosinophils D Mast cells E Neutrophils
A
D Mast cells
98
Q
A 35-year-old man develops diarrhoea with fever and malaise 24 hours after eating a take-away meal. Stool cultures reveal the source of the infection is Salmonella spp. Which antibody is responsible for protecting against gastrointestinal infections? A IgA B IgD C IgG D IgM E IgE
A
A. IgA
99
Q
A 23-year-old man presents to his GP with recent onset diarrhoea, fatigue and weight loss. The patient suggests that his symptoms are worsened after eating bread or rice. Which human leukocyte antigen is most likely to be associated with his disease process? A HLA B27 B HLA DR2 C HLA DR3 D HLA DR4 E HLA DQ2
A
E. HLA DQ2
100
Q
A 3-year-old Afro-Caribbean boy is referred to a paediatrician after concerns about his recurrent chest infections. The child’s hair slowly fell out and there is evidence of depigmentation of his skin. Blood tests reveal hypocalcaemia and high TSH levels. Which component of the immune tolerance system is likely to be dysfunctional? A Regulatory T cell B TGF- beta C Autoimmune regulator D Dendritic cells E IL-10
A
C. Autoimmune regulator
He has APSI = a monogenic auto-immune condition
101
Q
A 34-year-old man presents to his GP with fever, joint pain and a rash on his trunk. On examination, a new murmur is auscultated. Blood investigations reveal a raised anti-streptolysin O titre. What is the most likely mechanism for this disease process? A Defective immunoregulation B Molecular mimicry C T-cell bypass D Release of hidden self antigens E Cytokines
A
B Molecular mimicry
102
Q
A 40-year-old diabetic man is to undergo a kidney transplant as a consequence of stage 5 chronic kidney disease. The patient has an identical twin who is will- ing to donate a kidney, and has been HLA matched at all loci. Which term best describes the type of organ transplant proposed? A Autograft B Split transplant C Allograft D Isograft E Xenograft
A
D Isograft
103
Q
A 45-year-old man, who has blood group O, has undergone a liver transplant secondary to chronic alcoholic liver disease which has led to cirrhosis. One hour after the operation the patient develops a fever and pain in his right upper quad- rant. It is soon realized that the donor had blood group B. Which of the following best describes the type of allograft rejection? A Hyperacute rejection B Acute cellular rejection C Chronic rejection D Acute vascular rejection E Graft-versus-host disease
A
A Hyperacute rejection
Hyperacute rejection = takes place within hours of the transplant and is caused by pre-formed antibodies binding to ABO or HLA.
Acute rejection = Type IV delayed hypersensitivity reaction and takes place within days, sometimes weeks of the transplantation
Chronic rejection = takes place months or years after the transplant
104
Q
A 54-year-old man is to undergo a heart transplant as a result of severe heart failure. Prior to the operation the transplant team initiate an immunosuppressive regimen using a drug that inhibits calcinurin. Which of the following drugs is this most likely to be? A Cyclosporine A B OKT3 C Azathioprine D Corticosteroids E Daclizumab
A
A Cyclosporine A
