Internal medicine - Clinical immunology

Question 1

INT - 14.1
Which is NOT a side-effect of chronic steroid therapy?
A) Hypertension
B) Myopathy
C) Hyperpotassemia
D) Cataract
E) Glaucoma

Answer 1

ANSWER
C) Hyperpotassemia
EXPLANATION
During chronic steroid therapy hypopotassemia develops (with hypernatremia), which needs potassium supplement.

Question 2

INT - 14.2
Which autoantibody is NOT specific for poly-dermatomyositis?
A) Anti-Ku
B) Anti-Mi2
C) Anti-SRP
D) Anti-Sm
E) Anti-Jo1

Answer 2

ANSWER
D) Anti-Sm
EXPLANATION
Anti Sm (anti Smith) antibody is mostly associated with SLE, not with inflammatory muscle diseases. The other autoantibodies can be observed in myositis.

Question 3

INT - 14.3
Which immunosuppressive agent MAY NOT be used for SLE therapy?
A) Cyclophosphamide
B) Methotrexate
C) Belimumab
D) Adalimumab
E) Azathioprine

Answer 3

ANSWER
D) Adalimumab
EXPLANATION
Adalimumab is a TNF-alpha inhibitor. Anti TNF agents can cause SLE, therefore they are contraindicated in SLE. The other agents can be used in the therapy of SLE.

Question 4

INT - 14.4
Which is NOT a complication of systemic sclerosis?
A) Diffuse pulmonary fibrosis
B) Honeydew melon stomach
C) Esophageal stricture
D) Pulmonary hypertension
E) Fingertip-ulcer

Answer 4

ANSWER
B) Honeydew melon stomach
EXPLANATION
It is a funny answer: it is obviously watermelon-stomach…

Question 5

INT - 14.5
Which is NOT an ANCA-associated vasculitis?
A) Polyarteritis nodosa
B) Churg-Strauss syndrome
C) Granulomatous vasculitis
D) Microscopic polyangiitis
E) Wegener granulomatosis

Answer 5

ANSWER
A) Polyarteritis nodosa
EXPLANATION
Polyarteritis nodosa is usually ANCA-negative, the others are ANCA-associated. Granulomatous vasculitis and Wegener-granulomatosis are two names of the same disease.

Question 6

INT - 14.6
Which antibody is specific for Antiphospholipid syndrome?
A) Anti-apexin V.
B) Anti-beta2-microglobulin I.
C) Anti-beta2-glycoprotein I
D) Anti-cardiotliptin
E) Anti-lupus anticoagulant

Answer 6

ANSWER
C) Anti-beta2-glycoprotein I
EXPLANATION
Among many similar-sounding antibodies beta-2 glycoprotein I is the correct. It is a cofactor of cardiolipin, autoantibodies are formed against it. There is anti-annexin V, but there is no anti-apexin V, and cardiotliptin is an obviously mistake, too.

Question 7

INT - 14.7
Which autoantibody is specific for MCTD?
A) Anti-PM-Scl
B) Anti-U1 RNP
C) ANCA
D) Anti-SS-A
E) Anti-dsDNA

Answer 7

ANSWER
B) Anti-U1 RNP
EXPLANATION
Anti-U1-RNP is the most specific (near 100%) autoantibody of MCTD with a diagnostic value. Others can occur too in a small number of MCTD patients, but they are not specific.

Question 8

INT - 14.8
Which is true about immunofluorescent staining of antinuclear antibody?
A) The staining is nucleolar in SLE
B) The staining is homogenous in MCTD
C) The staining is nucleolar in systemic sclerosis
D) The staining is homogenous in RA
E) The staining is against centromer in myositis

Answer 8

ANSWER
C) The staining is nucleolar in systemic sclerosis
EXPLANATION
In systemic sclerosis, truly nucleolar ANA staining is specific. ANA staining in SLE is usually homogenous, in MCTD is granular or nucleolar, in RA is mixed. Centromere staining is typical in scleroderma.

Question 9

INT - 14.10
Which statement is NOT typical in systemic autoimmune diseases?
A) Familiar aggregation
B) Sometimes medicines or chemicals can trigger them
C) Antinuclear autoantibodies are frequent
D) Males are affected mainly
E) The disease can worsen or improve during pregnancy

Answer 9

ANSWER
D) Males are affected mainly
EXPLANATION
Systemic autoimmune diseases mostly occur in women. Family aggregation is typical, medicines (e.g. SLE) or chemicals (e.g. systemic sclerosis) can trigger them, any kind of ANA can be detected in almost all of them and during pregnancy they can worsen (e.g. SLE) or improve (e.g. RA).

Question 10

INT - 14.11
Which genetic constellation is typical in autoimmune diseases?
A) HLA-DQ2-DR4
B) HLA-A1-B8-DR3
C) HLA-A8-B27-DR4
D) HLA-B7-B27-DR3
E) HLA-A1-B7-DR4

Answer 10

ANSWER
B) HLA-A1-B8-DR3
EXPLANATION
The HLA-A1-B8-DR3 genetic constellation is typical in many autoimmune diseases. DQ2 is typical in celiac disease, B27 in spondylarthropathies, B7 in psoriasis.

Question 11

INT - 14.12
Which of the following is the most frequently occurring systemic autoimmune disease?
A) SLE
B) Polymyositis
C) Systemic sclerosis
D) MCTD
E) Sjögren’s syndrome

Answer 11

ANSWER
E) Sjögren’s syndrome
EXPLANATION
Prevalence of Sjögren syndrome can approach 0,5-1%, the others are much less common.

Question 12

INT - 14.13
Which autoimmune disease is NOT commonly associated with B-cell lymphoma?
A) SLE
B) Systemic sclerosis
C) Rheumatoid arthritis
D) Polymyositis
E) Sjögren’s syndrome

Answer 12

ANSWER
D) Polymyositis
EXPLANATION
Polymyositis is mostly T-cell mediated, B-cell lymphomas are not more common associated with it. Secondary tumors are associated rather with dermatomyositis. In the other diseases B-cell stimulation is typical, which causes an increased risk of B-cell hematologic tumor development.

Question 13

INT - 14.14
Which is NOT a symptom of antiphospholipid syndrome?
A) Habitual abortion
B) Frequent migraine
C) Thrombocytosis
D) Increased risk of thromboembolism
E) Livedo reticularis

Answer 13

ANSWER
C) Thrombocytosis
EXPLANATION
In case of antiphospholipid syndrome there is thrombocytopenia despite the recurrent thrombosis. Migraine is rare, but typical.

Question 14

INT - 14.15
Which medicine DO NOT causes SLE?
A) Etanercept
B) Procainamide
C) Infliximab
D) Hydralazine
E) Rituximab

Answer 14

ANSWER
E) Rituximab
EXPLANATION
Rituximab is a B-cell inhibitor which doesn’t cause SLE, moreover in off-label indication it can be appropriate for the therapy of certain SLE cases. Etanercept and infliximab are TNF inhibitors, they can cause SLE. Procainamide and hydralazine are classic triggers of drug-induced lupus.

Question 15

INT - 14.16
In what percent of patients with undifferentiated autoimmune diseases (NDC) develops definitive autoimmune disease?
A) 5-10 %
B) 15-20 %
C) 30-40 %
D) 50-60 %
E) 70-80 %

Answer 15

ANSWER
D) 50-60 %
EXPLANATION
Half or two-thirds of NDC cases transform into definitive disease, in 10-15 % the symptoms regress, in 30-35% the NDC stadium is long-lasting (more than 5 years).

Question 16

INT - 14.17
In which case can be NDC diagnosed?
A) In the presence of three typical clinical symptoms
B) In the presence of one typical clinical symptom and two autoantibody positivity’s
C) In the presence of two clinical symptoms and two autoantibodies
D) In the presence of two clinical symptoms and one autoantibody
E) In the presence of one clinical symptom and three typical autoantibodies

Answer 16

ANSWER
D) In the presence of two clinical symptoms and one autoantibody
EXPLANATION
There must be two of the typical autoimmune clinical symptoms (polyarthritis, sicca symptom, Raynaud phenomenon etc.) and an immune serology positivity (e.g. ANA) must be associated with them.

Question 17

INT - 14.18
Which process is NOT typical in the pathogenesis of SLE?
A) DNA repair disorder
B) DNA methylation
C) Tissue citrullination
D) Increased B-cell activity
E) Decreased regulatory T-cell activity

Answer 17

ANSWER
C) Tissue citrullination
EXPLANATION
Tissue citrullination can be observed mainly in rheumatoid arthritis, its importance in SLE is minimal.

Question 18

NT - 14.20
Accelerated atherosclerosis and increased cardiovascular mortality are NOT typical in which disease?
A) Rheumatoid arthritis
B) SLE
C) Sjögren’s syndrome
D) Antiphospholipid syndrome
E) Systemic sclerosis

Answer 18

ANSWER
C) Sjögren’s syndrome
EXPLANATION
In RA, SLE, antiphospholipid syndrome and scleroderma we have a lot of data about accelerated atherosclerosis, increased cardiovascular morbidity and mortality. In Sjögren’s syndrome, it is not proved yet.

Question 19

INT - 14.21
Which is NOT a real histological type of lupus nephritis?
A) Diffuse lupus nephritis
B) Sclerotizing glomerulonephritis
C) Membranous lupus nephritis
D) Mesangial proliferative lupus nephritis
E) Focal minimal lupus nephritis

Answer 19

ANSWER
E) Focal minimal lupus nephritis
EXPLANATION
There are focal and minimal lupus nephritis, but not associated together.

Question 20

INT - 14.23
Which is NOT a typical cause of death in SLE?
A) Cardiovascular disease
B) Kidney failure
C) Central nervous system lupus
D) Pulmonary fibrosis
E) Secondary tumors

Answer 20

ANSWER
D) Pulmonary fibrosis
EXPLANATION
Pulmonary fibrosis is not typical in SLE, it usually occurs in systemic sclerosis or MCTD.

Question 21

INT - 14.24
Which pathogenetic process DOES NOT plays a role in the development of systemic sclerosis?
A) Microvascular disorders
B) Immunopathological disorders
C) Tissue citrullination
D) Collagen deposition in the organs
E) Macrovascular disorders

Answer 21

ANSWER
C) Tissue citrullination
EXPLANATION
Tissue citrullination occurs mainly in rheumatoid arthritis.

Question 22

INT - 14.26
Which is NOT a diagnostic criterion of systemic sclerosis?
A) Sclerodactylia
B) Paroxysmal scleroderma
C) Star-shaped scars on the fingertips
D) Bibasilar pulmonary fibrosis
E) Esophageal dismotility

Answer 22

ANSWER
E) Esophageal dismotility
EXPLANATION
Esophageal dismotility is truly an organic symptom of systemic sclerosis, but is not among the major and minor ACR diagnostic criteria.

Question 23

INT - 14.27
Which medicine is NOT appropriate for treating the vascular symptoms of systemic sclerosis?
A) Nitroglycerin
B) Calcium antagonists
C) Angiotensin II receptor antagonists
D) Beta receptor blockers
E) Sildenafil

Answer 23

ANSWER
D) Beta receptor blockers
EXPLANATION
All agents are vasculoprotective, but beta-blockers enhance Raynaud’s phenomenon and microvascular disorders, therefore they are contraindicated in scleroderma.

Question 24

INT - 14.28
Which is NOT among the clinic pathological subtypes of inflammatory muscle diseases?
A) Adult polymyositis
B) Tumor-associated myositis
C) Juvenile poly-dermatomyositis
D) Inclusion body myositis
E) Ossification myositis

Answer 24

ANSWER
E) Ossification myositis
EXPLANATION
Myositis ossificans is probably not autoimmune originated, it causes local muscle calcification.

Question 25

INT - 14.29
Which is NOT among the diagnostic criteria of inflammatory muscular diseases according to Bohan and Peter?
A) Elevated levels of CK and LDH enzymes
B) Intensifying weakness of distal muscles
C) Positive muscle biopsy with inflammation, necrosis
D) EMG disorders
E) Skin symptoms specific for dermatomyositis

Answer 25

ANSWER
B) Intensifying weakness of distal muscles
EXPLANATION
In poly-dermatomyositis, intensifying weakness of proximal muscles is typical.

Question 26

INT - 14.31
Which agent is NOT appropriate for treating glandular symptoms of Sjögren’s syndrome?
A) N-acetylcysteine
B) Tear point occlusion
C) Methylcellulose containing artificial tear
D) Infliximab
E) Pilocarpine

Answer 26

ANSWER
D) Infliximab
EXPLANATION
Infliximab is a TNF inhibitor, there were clinical tests using it in Sjögren’s syndrome, but it was ineffective.

Question 27

INT - 14.32
Which disease is NOT an element of MCTD?
A) Rheumatoid arthritis
B) NDC
C) Polymyositis
D) SLE
E) Systemic sclerosis

Answer 27

ANSWER
B) NDC
EXPLANATION
It is frequent to mistake NDC (which is an immature autimmune predisease) for MCTD (which is a separate autoimmune disease with determined clinical criteria and specific autoantibody /anti-U1-RNP/). MCTD consists of RA, polymyositis, SLE and systemic sclerosis symptoms.

Question 28

INT - 14.33
Which disease constitutes diagnostic criteria for MCTD according to Alarcon-Segovia?
A) Pulmonary fibrosis
B) Swelling of the back of the hand
C) Pulmonary arterial hypertension
D) Esophageal dismotility
E) Swelling of the fingers

Answer 28

ANSWER
B) Swelling of the back of the hand
EXPLANATION
Swelling on the back of the hand is in this criteria system. Swelling of the fingers is in the Kahn-system, the others are parts of MCTD, but are not in any criteria system.

Question 29

INT - 14.35
Which systemic vasculitis affects the medium-sized vessels?
A) Churg-Strauss syndrome
B) Takayasu arteritis
C) Kawasaki disease
D) Cryoglobulinemic vasculitis
E) Polymyalgia rheumatica

Answer 29

ANSWER
C) Kawasaki disease
EXPLANATION
Medium-sized vessels are affected by Kawasaki disease and Polyarteritis nodosa.

Question 30

INT - 14.36
Histopathological forms of lupus nephritis:
1) Focal lupus nephritis
2) Membranous lupus nephritis
3) Sclerotizing glomerulonephritis
4) Minimal mesangial nephritis
5) Diffuse lupus nephritis
A) 1. and 3. are correct
B) 2. and 4. are correct
C) only 5. is correct
D) 1., 3. and 5. are correct
E) all answers are correct

Answer 30

ANSWER
E) all answers are correct
EXPLANATION
These histological types all occur in lupus nephritis.

Question 31

INT - 14.37
Classification criteria of SLE:
1) Iron deficiency anemia
2) ANA positivity
3) Anti-ENA antibody
4) Leukopenia <4G/l
5) Lymphocytosis
A) answers 1 and 3 are correct
B) answers 2 and 4 are correct
C) only answer 5 is correct
D) answers 1, 3 and 5 are correct
E) all of the answers are correct

Answer 31

ANSWER
B) answers 2 and 4 are correct
EXPLANATION
ANA positivity and leukopenia (lower than 4 G/l) are in the SLE criteria system according to ACR. In SLE there are hemolytic and noniron deficiency anemia and lymphopenia among the criteria, not lymphocytosis. Anti-ENA positivity may occur, but it is not a criterion.

Question 32

INT - 14.39
Diagnostic criteria of Sjögren’s syndrome according to the American-European Consensus system:
1) Recurrent foreign body sensation in the eyes
2) Positive ultrasound of salivary glands
3) Dry eyes for more than 3 months
4) Schirmer-test <3mm/3min
5) Positive sialography
A) 1. and 3. answers are correct
B) 2. and 4. answers are correct
C) only 5. answer is correct
D) 1., 3. and 5. answers are correct
E) all of the answers are correct

Answer 32

ANSWER
D) 1., 3. and 5. answers are correct
EXPLANATION
Foreign body sensation in the eyes, dry eyes and positive sialography are among the criteria in Sjögren’s syndrome. Salivary gland ultrasound is not among the criteria and the pathological value of Schirmer-test is less than 5mm/5 minutes.

Question 33

INT - 14.41
Classification criteria of Henoch-Schönlein purpura:
1) Palpable purpura
2) ANCA positivity
3) Abdominal angina
4) Lymphocytes in the wall of small vessels
5) Age <21 years at the beginning of the disease
A) 1. and 3. are correct
B) 2. and 4. are correct
C) only 5. is correct
D) 1., 3. and 5. are correct
E) all answers are correct

Answer 33

ANSWER
D) 1., 3. and 5. are correct
EXPLANATION
For Henoch-Schönlein vasculitis palpable purpura, abdominal (intestinal) angina and age under 21 years are typical. This form of vasculitis is ANCA-negative and there are neutrophil granulocytes in the wall of the small vessels.

Question 34

INT - 14.42
Which is true?
1) Wegener-granulomatosis is a vasculitis affecting the small vessels
2) Polyarteritis nodosa is an ANCA-associated vasculitis
3) Microscopic polyangiitis is an ANCA-associated vasculitis
4) Takayasu-arteritis affects the medium-sized vessels
5) Churg-Strauss syndrome is not ANCA-associated
A) 1. and 3. are correct
B) 2. and 4. are correct
C) only 5. is correct
D) 1., 3. and 5. are correct
E) all answers are correct

Answer 34

ANSWER
A) 1. and 3. are correct
EXPLANATION
Wegener granulomatosis (granulomatous vasculitis) affects the small vessels, MPA is ANCA-associated. PAN is not ANCA-associated, Takayasu-arteritis affects the large vessels and Churg-Strauss syndrome is ANCA-associated.

Question 35

INT - 14.44
Appropriate medicines for therapy of ANCA-associated vasculitides:
1) Cyclophosphamide
2) Azathioprine
3) Sulfamethoxazole
4) Methotrexate
5) Rituximab
A) 1. and 3. are correct
B) 2. and 4. are correct
C) only 5. is correct
D) 1., 3. and 5. are correct
E) all answers are correct

Answer 35

ANSWER
E) all answers are correct
EXPLANATION
All the listed medicines are appropriate for treating vasculitis. We use cyclophosphamide for induction treatment, and then azathioprine and methotrexate are appropriate for sustaining therapy. The clinical base of the sulphamethoxazole therapy is the supposed pathogenic role of Pseudomonas jirovecii infection. Several studies proved the efficiency of rituximab, especially in Wegener granulomatosis. In vasculitis it can be used with special permission except for indication (off-label).

Question 36

INT - 14.45
Organ symptoms of systemic sclerosis:
1) Pulmonary arterial hypertension
2) Apical pulmonary fibrosis
3) Watermelon-stomach
4) Discoid skin symptoms
5) Subcutaneous calcinosis
A) 1. and 3. are correct
B) 2. and 4. are correct
C) only 5. is correct
D) 1., 3. and 5. are correct
E) all answers are correct

Answer 36

ANSWER
D) 1., 3. and 5. are correct
EXPLANATION
Pulmonary hypertension, watermelon-stomach and calcinosis truly can be symptoms of scleroderma. However, pulmonary fibrosis is located bibasilarly and discoid skin symptoms are specific for SLE (DLE).

Question 37

INT - 14.9
Which is NOT a polysystemic autoimmune disease?
A) Polymyositis
B) Systemic sclerosis
C) Sclerosis multiplex
D) Sjögren’s syndrome
E) MCTD

Answer 37

ANSWER
C) Sclerosis multiplex
EXPLANATION
Sclerosis multiplex affects the nervous system, the others are truly polysystemic.

Question 38

INT - 14.19
Which biological therapy is appropriate for the therapy of SLE?
A) Etanercept
B) Infliximab
C) Belimumab
D) Adalimumab
E) Tocilizumab

Answer 38

ANSWER
C) Belimumab
EXPLANATION
Belimumab is the only agent for SLE therapy which inhibits B-cell activating factor (BAFF, BLyS). Etanercept, infliximab and adalimumab are TNF inhibitors which can cause lupus-like symptoms therefore they are contraindicated in therapy of SLE. Tocilizumab is an interleukin-6 receptor inhibitor which may be appropriate for therapy of SLE, but there is no evidence for it yet.

Question 39

INT - 14.25
Against which antigen is oriented Anti-Scl70 in scleroderma?
A) RNA polymerase I
B) DNA polymerase I
C) DNA topoisomerase III
D) RNA polymerase III
E) DNA topoisomerase I

Answer 39

ANSWER
E) DNA topoisomerase I
EXPLANATION
The several similar-sounding antibodies truly occur in systemic sclerosis, but the autoantigen of anti-Scl70 is DNA topoisomerase I.

Question 40

INT - 14.30
Which is NOT among the American-European Consensus Criteria for Sjögren’s syndrome?
A) Anti-fodrin antibody
B) Anti-SS-A and/or anti-SS-B antibody
C) Positive Rose-Bengal test
D) Schirmer-test <5 mm/5 min
E) Positive salivary gland scintigraphy

Answer 40

ANSWER
A) Anti-fodrin antibody
EXPLANATION
Anti-fodrin antibody was a hit for a long time, it was considered to be very specific for Sjögren’s syndrome. Later this enthusiasm decreased, now it is not among the diagnostic criteria.

Question 40

INT - 14.40
B-cell inhibitor appropriate for therapy of SLE:
1) Rituximab
2) Tocilizumab
3) Certolizumab pegol
4) Infliximab
5) Belimumab
A) 1. and 3. are correct
B) 2. and 4. are correct
C) only 5. is correct
D) 1., 3. and 5. are correct
E) all answers are correct

Answer 40

ANSWER
C) only 5. is correct
EXPLANATION
Belimumab is the only agent for SLE therapy which inhibits B-cell activating factor (BAFF, BLyS). Rituximab is a B-cell inhibitor too, but its effectivity in SLE is not proven. Certolizumab pegol and infliximab are TNF inhibitors, tocilizumab is an IL-6 receptor inhibitor antibody.