Rheumatology Flashcards
1
Q
3 antibodies in antiphospholipid syndrome
A
- Anti-cardiolipin
- Lupus anticoagulant
- Anti Beta-2 glycoprotein
2
Q
What is Felty’s syndrome
A
Splenomegaly
Leg ulcers
neutropenia
High risk triad seen in rheumatoid
3
Q
Cardiac complication of rheumatoid arthritis
A
constrictive pericarditis
4
Q
Which deletion mutation is protective for Ankylosing spondylitis
A
ERAP 1 deletion - as this protein is required to present peptides to HLA B27
5
Q
Antibody in Scleroderma renal crisis
A
RNA polymerase 3
6
Q
Management of GCA
A
- Steroids
- IV methyl pred 3 days if visual symptoms.
- 1mg/kg pred up to 60mg with a slow wean. - Long term prophylaxis
- Tocilizumab
- Methotrexate
7
Q
Anti SRP association
A
Necrotizing myositis
8
Q
Anti TIF 1 association
A
Myositis with high risk malignancy
9
Q
Anti MDA5/CADM
A
Rapidly progressive DM.
ILD, less muscle involvement.
10
Q
Symptoms of anti-synthetase syndrome
A
Anti-Jo (anti-tRNA), myositis, Raynaud’s, mechanics hands, heliotrope rash, ILD, non-erosive arthritis.
11
Q
Risk factor for recurrence of reactive arthritis
A
HLA B27 gene
12
Q
Lofgrens syndrome
A
Seen in Sarcoidosis
- swollen ankles
- erythema nodosum
- bilateral hilar lymphadenopathy
13
Q
Which arthropathy commonly involves the enthesis
A
Reactive arthritis
14
Q
DFS70 significance
A
indicates patient does NOT have systemic autoimmune rheumatic disease
15
Q
All rheumatological diseases have NSIP pattern of ILD except
A
RA: Has UIP pattern
16
Q
Environmental triggers for RA
A
Mucosal inflammation:
Gut microbiome
Periodontitis
Lung mucosa inflammation - smoking.
17
Q
Significance of RF positive rheumatoid arthritis
A
More severe disease if RF positive
18
Q
What is RS3PE
A
Remitting seronegative, symmetric synovitis with pitting oedema
“boxing glove appearance”
Responds well to steroids
19
Q
Hand X-ray in rheumatoid arthritis
A
- Symmetrical
- *PIP and MCP joints involved, DIPs spared.
- subchondral cysts and erosions
- *juxta-articular osteoporosis
- *ulnar deviation at MCP, radial deviation at wrist
- boutonniere, hitchhikers thumb and swan neck deformity
- erosion of medial distal radius
Other - atlantoaxial subluxation
20
Q
Hand X-ray in Gout
A
- Podagra - 1st metatarsophalangeal joint
- usually a oligoarthropathy
- joint effusion
- punched out erosions with over-hanging edge
- tophi
- double halo sign on US
Note- there is NO osteopenia
21
Q
Hand X-ray in Pseudogout
A
- involves knee, wrist, elbow, shoulder
- if hand - wrists and MCPs only
- features of osteoarthritis in non-weight bearing joints
- chondrocalcinosis
- subchondral cysts
Single halo on US as calcifications are within the cartilage itself
22
Q
Hand X-ray in Psoriatic arthritis
A
- distal joint predominance
- symmetric polyarthropathy
- asymmetric oligoarthroapthy
- enthesitis
- *pencil in cup erosions
- joint subluxation
- *periostitis - new bone formation/ thickening
- dactylitis - sausage finger
- athritis mutilans - telescoping fingers
Other:
- nail involvement
- skin psoriasis
- uveitis/ conjunctivitis
- IBD
- prostatitis
23
Q
Hand X-ray in Psoriatic arthritis
A
- distal joint predominance
- symmetric polyarthropathy
- asymmetric oligoarthroapthy
- enthesitis
- *pencil in cup erosions
- joint subluxation
- *periostitis - new bone formation/ thickening
- dactylitis - sausage finger
- athritis mutilans - telescoping fingers
Other:
- nail involvement
- skin psoriasis
- uveitis/ conjunctivitis
- IBD
- prostatitis
24
Q
Hand X-ray in reactive arthritis
A
Triad - arthritis, conjunctivitis, urethritis (cervicitis in women)
- HLAB27 positive
- lower limbs > upper limbs
- asymmetric
- Enthesitis
- floating osteophytes
- *juxta-articular osteoporosis
- *periostitis - new bone formation/ thickening
25
Hand X-ray in reactive arthritis
Reiters Triad - arthritis, conjunctivitis, urethritis (cervicitis in women)
- HLAB27 positive
- lower limbs > upper limbs
- asymmetric
- Enthesitis
- floating osteophytes
- *juxta-articular osteoporosis
- *periostitis - new bone formation/ thickening
26
RA remission score
DAS28 <2.6
ACR20/ ACR50/ ACR70
27
Cytokine involved in Gout
IL-1
28
Hand X-ray in Osteoarthritis
- Joint space narrowing
- Osteophytes
- Osteosclerosis
- erosions and subchondral cysts
- synovitis
29
Hand X-ray in lupus/SLE
- correctable ulnar subluxation
- no erosions
Also called Jaccoud's arthropathy
30
Probenecid contraindications
Dont use in PMHx of renal calculi
| Increases concentration of ABx through inhibiting tubular secretion
31
Drugs which cause Lupus
TNF inhibitors
32
Cardiac complications of Lupus
Pericarditis
Cardiomegaly
Conduction defects
Libman-Sacks (sterile) endocarditis
33
When to immunosuppress in lupus nephritis
Class 3,4,5.
| Do not immunosuppress in 1,2 (too early) or 6 (too late)
34
Immunosuppression agents in lupus nephritis - in order
1. IV pulse steroids
2. Mycophenolate
3. Belimumab (NOT RITUX) - Blocks B activating factor (BAFF)
4. Cyclophosphamide/ Azathioprine
35
Genetic associations in Scleroderma
HLADRB1
| STAT4
36
Other name for Anti-Scl70
Anti- topoisomerase
37
Best ACE-i for Scleroderma renal crisis
Captopril
38
Immune marker for inclusion body myositis
anti-CN1A
39
extra-muscular features of Myositis
Gottron's papules
Poikiloderma (shawl sign)
Heliotrope rash
40
HLA type in Ankylosing spondylitis
HLAB27
41
Extra-articular manifestations of ankylosing spondylitis
- anterior uveitis
- IBD
- psoriasis
- aortic regurg
42
Second line Mx for ankylosing spondylitis (after NSAIDs, steroids, non-pharm)
TNF- alpha blockers - Adalimumab, Etanercept, Infliximab, (Golimumab for non-radiological AS)
IL-17 inhibitors - Secukinumab
43
Artery which causes vision loss in GCA
posterior ciliary artery
44
Management of PMR
15mg/day pred for 4 months then wean
45
Leflunomide in pregnancy
Cease + cholestyramine washout
46
First site of damage in knee OA
medial tibial-femoral cartilage
47
Risk factors for future joint erosions in RA
- number of swollen joints currently
- number of erosions currently
- RF/CCP/ESR/CRP levels
48
beta-2-microglobulin is associated with which type of amyloidosis
dialysis associated
49
TTR is associated with which type of amyloidosis
senile cardiac amyloid
50
Management of Ank Spond
1. Exercise
2. NSAIDs
3. TNF-a (NO role for DMARDs) - Cannot use in multiple sclerosis.
4. IL-17 agents - Secukinumab
51
What is rheumatoid factor?
It is usually an IgM directed against the FC portion of IgG
52
Dietary factor most affecting the risk of Gout
Beer
53
Pathophysiology of Vasculitis
1. vasculitis is a T-cell driven process that is triggered by exposure to antigens, most probably infectious agents. Dendritic APCs activate CD4+ T cells, which recruit macrophages and monocytes to the vessel walls. induce systemic inflammation via the release of cytokines, IL1/IL6. T cells also release interferon-γ, which is a key proinflammatory cytokine of GCA.
Platelet-derived growth factor and vascular endothelial growth factor play key roles in GCA.
2. Immune complex formation -polyarteritis nodosa, cryoglobulinaemia and Henoch-Schönlein purpura.
54
Drug used for refractory moderate SLE after hydroxychloroquine and steroids
Belimumab (BAFF/BLYS inhibitor)
55
SE of hydroxychloroquine
Retinopathy
56
Treatment of cutaneous SLE
First-line treatment of skin disease includes topical agents (GC and/or CNIs) and antimalarials, with or without systemic GC.
57
Treatment of Lupus Nephritis
MMF and cyclophosphamide are the agents
of choice for induction treatment; low-dose CYC is preferred over highdose CYC as it has comparable efficacy and lower risk of gonadotoxicity.
Refractory disease - Rituximab
58
HLA associated with Rheumatoid arthritis
HLA-DRB1
59
What is Rheumatoid factor
An antibody against the FC portion of IgG
60
HLA for Allopurinol hypersensitivity
HLAB5801
61
T cell type and interleukin related to HLAB27 positivity
IL23, TH17.
62
Reiters Syndrome
Reactive arthritis + Conjunctivitis + Urethritis
63
Drug to avoid in IBD related arthropathy
IL17 inhibitors - Secukinumab
64
Features of Jaccouds arthropathy and diseases associated
- correctable ulnar subluxation
- no erosions
Diseases - Rheumatic fever, SLE.
65
Antibidy related to neural lupus
Ribosomal P antibodies
66
Management of antiphospholipid syndrome
Aspirin
| Hydroxychloroquine
67
Condition causing watermelon stomach
GAVE - gastric antral vascular ectasia - associated with scleroderma.
68
Management for Raynauds/ nail vasculopathies in Scleroderma
Hand warmers
CCBs
Sildenafil
Bosentan
69
PRES ( Posterior reversible encephalopathy syndrome) symptoms as associations.
(same as reversible posterior leukoencephalopathy syndrome and brain capillary leak syndrome.
Symptoms - CCCV
- cephalgia's
- convulsions
- confusion
- visual disturbance
Associations:
- HTN
- Immunosuppression
- lupus nephritis/ GN
70
Pattern of myopathy in inclusion body myositis
distal upper limb
| proximal lower limb
71
HLA in statin induced myositis
HLADRB1 11:01
72
Bechets HLA association
HLAB51
73
ACPA (CCP) is formed by which process
citrulination
74
Immunosuppressants safe in pregnancy
P - pred
A - Aza
S - Sulfasalazine (but reduces male fertility)
H - Hydroxychloroqine.
75
Contraindications to use of TNF inhibitors
- demyelinating disease
- septic arthritis <12 months
- pregnancy
- untreated TB
- malignancy within 10 years
- chronic cutaneous ulceration
- indwelling catheter
- CCF
- infected prosthesis
- recurrent chest infections.
76
Familial Mediterranean fever symptoms
intermittant fever, abdominal pain, chest pain, erysipleas like skin lesion.
77
Familial Mediterranean fever treatment
Colchicine
78
Familial Mediterranean fever cause and marker
Marker - SAA amyloid protein
| Cause - autosomal recessive mutation in pyrin gene MEFV
79
Which infection is PAN associated with?
Hep B
80
Treatment of PAN
Non-infection related --> Steroids + cyclophosphamide
| Infection related --> treat infection + PLEX
81
Which organ system does PAN not affect?
respiratory system
82
Extra-articular features of reactive arthritis
- conjunctivitis
- uveitis
- urethritis
- balanitis
- keratoderma blennorrhagicum - (scaly palm and sole rash)
- aortic incompetence
83
Good prognostic marker in Rheumatoid
Acute onset with a few large joints affected only
84
ACR criteria for SLE
MD SOAP BRAIN symptoms.
Alcopecia is NOT in the criteria
Malar rash, Discoid rash, Serositis, Oral ulcers, arthritis, photosensitive rash, Blood dyscrasias, Renal disease, ANA/ Autoantibodies, Immune markers, Neurological symptoms.
85
Antibody in drug induced lupus
Anti-histone antibody
86
AL amyloidosis features
```
Due to light chain deposition.
deposits in kidney, heart and liver.
Macroglossia (large tongue)
Autonomic symptoms.
Carpal tunnel
```
87
AA amyloidosis features
Auto-immune, infection or malignancy related.
| Produced by the LIVER.
88
ATTR amyloidosis features
Deposits in the heart - particularly in elderly men.
89
AF amyloidosis features
Inherited. Abnormal protein produced in the liver.
90
Polymyositis antibody and histology
Anti Jo - associated with antisynthetase syndrome and ILD.
| Histo - CD8 infiltration and increase MHC1 expression.
91
Dermatomyositis antibody and histology
MDA5 - Rapidly progressive DM. ILD, less muscle involvement.
Mi2
TIF-1 --> highest risk for malignancy
Histo - wedge like infarcts and reduced capillaries
92
Necrotizing myositis antibody and histology
Anti SRP
Anti HMGCoR
Histo - necrotic fibres with MACROPHAGES and COMPLEMENT.
93
Inclusion body myositis antibody and histology
anti-CN1A
Histo - CD8 infiltration and increase MHC1 expression.
Ragged reg and ragged blue staining, Amyloid deposits, VACUOLES.
94
Highest risk antibodies for mixed connective tissue disease
Anti-68 kD and A' antibodies
| U1RNP is second highest
95
DMARD which affects male fertility
Sulfasalazine
96
4 symptoms of IgA disease (HSP)
1. Palpable purpura in patients with neither thrombocytopenia nor coagulopathy
2. Arthritis/arthralgia
3. Abdominal pain
4. Renal disease
97
Rheum condition with highest risk of Lymphoma
Sjogren's
98
Risk factors for ILD progression in Scleroderma
```
Disease duration <4 years
Diffuse cutaneous systemic sclerosis
Pulmonary function tests
- FVC <65%
- DLCO <55%
- >10% change
HRCT
- Interstitial lung disease affecting >20% of the lung
Serology
- Anti-topoisomerase I (anti-Scl-70) antibodies
```
99
Most effective treatment for scleroderma ILD
Mycophenolate (better than cyclophosphamide)
100
Single gene mutation that causes lupus (rare)
TREX1
101
First cell involved in GCA
Dendritic cell - activates TLR and CD83
102
gene associated with PAN
ADA2
103
Treatment for Granulomatosis with polyangiitis
Cyclophosphamide
Azathioprine maintenance - 3 years
Prednisolone
Rituximab if treatment resistant
104
Colchicine can treat which manifestations of Familial Mediterranean fever
Fever
Amyloidosis
Serositis
--> Does not work well for arthralgias
105
Which feature of Rheumatoid arthritis is worsened by methotrexate
Rheumatoid nodules
106
When can Rituximab be used in ANCA vasculitis
1. PBS approved for induction
| 2. Trial proven for maintenance (not on PBS for maintenance)
107
Which infection is increased by the use of TKIs
herpes zoster
108
Rheumatoid antibodies - most specific, best NPV, best for monitoring disease activity.
most specific - anti Sm
best NPV - ANA
best for monitoring disease activity - dsDNA
