Rheumatology Flashcards

1
Q

What is the inheritance pattern of Marfan’s syndrome?

A

Autosomal dominant

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2
Q

Which autoantibodies would you expect to find in Limited Cutaneous Systemic Sclerosis?

A
ANA (90%) -> anti-centromere
Rheumatoid factor (30%)
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3
Q

Which autoantibodies would you expect to find in Diffuse Cutaneous Systemic Sclerosis?

A
ANA (90%) -> anti-scl-70
Rheumatoid factor (30%)
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4
Q

What is the mechanism of action of methotrexate?

A

Inhibits dihydrofolate reductase, an enzyme essential for the synthesis of purines and pyrimidines.

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5
Q

What are the indications for methotrexate?

A
  • Inflammatory arthritis, especially rheumatoid arthritis
  • psoriasis
  • some chemotherapy acute lymphoblastic leukaemia
  • UC
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6
Q

What are the adverse affects associated with methotrexate?

A
Mucositis
Myelosuppression
Pneumonitis
Pulmonary fibrosis
Liver cirrhosis
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7
Q

How often is methotrexate taken?

A

Weekly

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8
Q

What drugs should be avoided in patients taking methotrexate?

A

Trimethoprim

Cotrimoxazole

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9
Q

What autoantibodies would you expect to find in SLE?

A
99% have ANA:
- anti-dsDNA (99%)
- anti-Smith (99%)
(also Ro and La)
20% have RF

Also -low C3 and 4

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10
Q

What monitoring is required in SLE?

A

ESR during active disease (CRP is often normal)
Complement levels are low during active disease
Anti-dsDNA can be used to disease monitoring (but not present in everyone)

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11
Q

What is osteomalacia?

A

Normal bony tissue, but dec mineral content

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12
Q

What are the causes of osteomalacia?

A
Vitamin D deficiency
Renal failure
Drug-induced - anticonvulsants
Vitamin D resistant (inherited)
Liver disease
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13
Q

What are the features of osteomalacia?

A

Bone pain
Fractures
Muscle tenderness
Proximal myopathy

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14
Q

What blood results would you expect in osteomalacia?

A

Low Ca
Low phos
Low Vit D
High ALP

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15
Q

What is the treatment for osteomalacia?

A

Calcium with vitamin D

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16
Q

What autoantibodies would you expect in drug-induced lupus?

A

ANA - 100%

  • anti-histone (85%)
  • anti-Ro and -Smith (5%)

dsDNA NEGATIVE (vs SLE where it’s +ve)

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17
Q

Give two drugs that can cause drug-induced lupus

A

procainamide

hydralazine

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18
Q

What is important to check with patients on hydroxychloroquine?

A

Vision - can cause Bull’s eye retinopathy

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19
Q

What is a common side effect of Leflunomide?

A

HTN

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20
Q

What are the requirements before allopurinol treatment can be initiated?

A

> = 2 attacks in 12m

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21
Q

What autoantibodies would you expect in RA?

A

Anti-CCP
RF
(Around 20% are seronegative)

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22
Q

What autoantibodies would you expect in Sjogren syndrome?

A

ANA - Ro and La (La is more specific)

RF

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23
Q

What blood results would you expect in anti-phospholipid syndrome?

A

Prolonged APTT

Dec platelets

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24
Q

What percentage of patients with psoriasis develop an associated arthropathy?

A

10-20%

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25
Q

Give the 6 As that are associated with ankylosing spondylitis

A
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
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26
Q

What causes splenomegaly in RA patients?

A

Felty’s syndrome

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27
Q

An allergy to which drug may be a contra-indication for the prescription of sulfasalazine?

A

Aspirin

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28
Q

What are the adverse effects of azathioprine?

A
  • bone marrow depression
  • N+V
  • pancreatitis
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29
Q

What is the first-line treatment for fibromyalgia?

A

CBT

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30
Q

What is the first-line treatment for osteoarthritis?

A

Paracetamol + topical NSAID (if hands or knees)

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31
Q

What are the characteristic features of scleroderma?

A
CREST syndrome
Calcinosis
Raynaud's
oEsophageal dysmotility
Sclerodactyly
Telangasia
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32
Q

What are Type 1 hypersensitivity reactions? Describe the skin changes you might expect

A

Mast cell degranulation (IgE bound)

Skin changes include urticaria

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33
Q

What are Type 3 hypersensitivity reactions?

A

Caused by the formation and deposition of antibody-antigen complexes. E.g. post-strep glomerulonephritis, SLE

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34
Q

What are Type 4 hypersensitivity reactions? What skin changes may occur?

A

Cell-mediated
E.g. contact dermatitis, T1DM, Hashimotos, IBD, MS
Skin changes include erythema multiforme (target lesions affecting palms, soles and face)

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35
Q

Give 3 side effects of methotrexate

A

Myelosuppression
Liver cirrhosis
Pneumonitis

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36
Q

Give 4 side effects of sulfasalazine

A

Rashes
Oligospermia
Heinz body anaemia
Interstitial lung disease

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37
Q

For 3 side effects of leflunomide

A

Liver impairment
Interstitial lung disease
HTN

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38
Q

Give 2 side effects of hydroxychloroquine

A

Retinopathy

Corneal deposits

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39
Q

Give 5 side effects of prednisolone

A
Cushingoid features
Osteoporosis
Impaired glucose tolerance
HTN
Cataracts
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40
Q

Give two side effects of NSAIDs

A

Bronchospasm in asthmatics

Dyspepsia/peptic ulceration

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41
Q

Describe the XR features in ank spond

A

sacroilitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
‘bamboo spine’ (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis

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42
Q

Initial therapy for newly diagnosed RA

A

Combination of DMARDs (incl methotrexate and one other DMARD, plus short-term glucocorticoids)

43
Q

Monitoring required when using methotrexate? Why?

Any other SEs?

A

FBC + LFTs
Risk of myelosuppression and liver cirrhosis

Other SEs: pneumonitis

44
Q

Name some DMARDs used in RA

A

Methotrexate
Sulfasalazine
Leflunomide
Hydroxychloroquine

45
Q

Name some TNF-inhibitors

A

Etanercept
Infliximab
Adalimumab

46
Q

Indications for TNF-inhibitors in RA

A

An inadequate response to at least 2 DMARDs incl metho

47
Q

55yo pt with 1m Hx of fever, arthralgia and lethargy. He recently developed haemoptysis and dyspnoea. Investigations show that he has an AKI. ANCA is negative. What is the diagnosis?

A

Goodpastures syndrome

48
Q

What positive investigation results would you expect in Goodpasture syndrome?

A

Anti-glomerular BM antibodies

49
Q

Outline the three stages of Churg-Strauss disease

A

1) Allergy - asthma or allergic rhinitis (may lead to nasal polyps)
2) Eosinophilia
3) Vasculitis - small and medium vessels leading to organ damage

50
Q

What underlying condition must be ruled out before a diagnosis of dermatomyositis can be made?

A

Malignancy - may be paraneoplastic disease and not dermato

51
Q

Which types of complement are low in SLE?

A

C3 and C4

52
Q

Describe pseudogout crystals

A

Positively bifefringent rhomboid shaped crystals

53
Q

Diagnostic marker in carcinoid

A

Urinary 5HIAA (24hr collection)

54
Q

Investigation to diagnose amyloidosis

A

Rectal biopsy

55
Q

Cause of reduced vision in temporal arteritis

A

Anterior ischaemic optic neuropathy

56
Q

Difference between limited and diffuse cutaneous systemic sclerosis

A

Limited - CREST

Diffuse - affects trunk and prox limbs. HTN, lung fibrosis, renal involvement

57
Q

Name the main four CT diseases in rheumatology

A

Scleroderma
Polymyalgia rheumatica
SLE
Polymyositis/dermatomyositis

58
Q

Name the main three bone metabolic diseases in rheum

A

Osteoporosis
Pagets disease
Osteomalacia/Ricketts

59
Q

Name the seronegative arthropathies

A

Reactive arthritis
Ankolysing spondylitis
Psoriatic arthritis
Enteropathic arthritis

60
Q

Name the crystal arthropathies

A

Pseudogout

Gout

61
Q

Which rheumatological conditions are often monoarticular

A

Trauma
Septic arthritis
Crystal arthritis
Monoarticular presentation of polyarticular disease

62
Q

Which rheumatological conditions are often oligoarticular (<5)

A

OA
Crystal arthritis
Seronegative spondyloarthropathies

63
Q

Which rheumatological conditions are often symmetrical and polyarticular

A

RA

OA

64
Q

Which rheumatological conditions are often asymmetrical and polyarticular

A

OA

Seronegative spondyloarthopathies

65
Q

Which rheumatological conditions are polyarticular, but can be either symmetrical or asymmetrical

A

AI CT diseases

Vasculities

66
Q

Investigations for rheum disorders

A

Bedside - obs, ECG
Bloods - FBC, UE, LFT, TFT, glucose, CRP/ESR, Autoantibodies, calcium/urate
Micro - joint aspiration, blood cultures
Imaging - XR joint, CT/MRI joint, CXR

67
Q

General management of rheum conditions

A

Conservative - RICE, physio, braces
Medical - NSAIDs, steroid injections, steroids, DMARDs, biologicals
Surgical - replacement, release

68
Q

What role do DMARDs play in rheum disorders

A

Control symptoms, delay progression of disease, and improve extra-articular manifestations

69
Q

First and second line DMARDs used in rheum disorders

A

1st line: methotrexate, sulfasalazine, hydroxychloroquine

2nd line: azathioprine, cyclosporin, leflunomide, mycophenolate mofetil

70
Q

Monitoring required for a pt on DMARDs

A

FBCs, UE, LFT regularly

Baseline, weekly until stable, every 2-3m

71
Q

When are biological agents indicated in rheum? Give examples

A

Used to treat mod-severe RA that’s not responded well to 1st/2nd line DMARDs
E.g. anti-TNF, rituximab, tocilizumab

72
Q

What is giant cell arteritis?

A

Large vessel vasculitis
Segmental granulomatous inflammation of ext carotid artery and branches
Associated with polymyalgia rheumatica
Causes irreversible visual loss if not treated

73
Q

3 immediate things to do in GCA

A

ESR/CRP
High-dose steroids (pred 40-80mg/d PO)
Urgent same day ophthalmology r/v

74
Q

Further management of GCA

A

Temporal artery biopsy within 1wk of starting steroids (skip lesions)
Cont steroids for 1-2yrs
Taper dose based on ESR and symptoms
PPI + bisphosphonate cover

75
Q

Investigations for gout

A
Bedside - obs
Bloods - FBC, UE, ESR/CRP
Micro - blood cultures
Imaging - XR
Special tests - joint aspiration (send for MC+S)
76
Q

Describe the crystals seen in gout

A

-ve biferingent needle-shaped urate crystals

77
Q

Acute management of gout

A

Conservative - remove cause, rest, inc fluids, avoid alcohol
Medical - NSAIDs, colchicine (if NSAIDs C/I), steroids (if NSAIDs and colchicine C/I)
Remain on allopurinol if pt is already on it

78
Q

Secondary prevention of gout

A

Conservative - W/L (if obese), dec alcohol intake, avoid high-purine foods (red meat, liver, oily fish), avoid drug causes (diuretics, cytotoxic drugs)
Medical - allopurinol

79
Q

When should allopurinol be started after the first attack of gout?

A

Start >2wks after attack under NSAID/colchicine cover

80
Q

Life-threatening drug reaction to watch out for in pts with gout?

A

Azathioprine + allopurinol!

Causes neuropenia

81
Q

Features of SLE

A
SOAP BRAIN MD
S erositis (pleuritis, pericarditis)
O ral ulcers
A rthritis
P hotosensitivity
B lood (anaemia, leukopenia, thrombocytopenia, APS)
R enal (protein, lupus nephritis)
A NA
I mmunologic (dsDNA)
N euro (psych, seizures)

M alar rash
D iscoid rash

Others - Raynaud’s, alopecia, livedo reticularis

82
Q

Describe the rashes you might see in SLE

A

Malar - facial erythematous rash sparing nasal folds, photosensitive

Discoid - well-demarcated scaly erythematous rash in sun-exposed areas, photosensitive

83
Q

Blood tests for SLE

A

1) Autoantibodys:
- ANA (sens)
- Anti-dsDNA (spec)
- Anti-histone (drug-ind)
- Anti-cardiolipin (APS)

2) ESR (inc = disease activity)
3) C3/4 (dec = disease activity)
4) FBC (dec Hb = AIHA, dec WCC, dec plats = APS)
5) UE (lupus nephritis)
6) Clotting (paradox inc APTT in APS)

84
Q

Management of SLE

A

Conservative - stop drug causes, smoking cess, advice about sun exposure
Medical:
- acute relapse -> high-dose pred, IV cyclophosphamide
- maintenance -> NSAIDs, hydroxychlorquinine ± low-dose pred
- treat complications -> sun cream, ACEi for proteinuria

85
Q

What is antiphospholipid syndrome?

A

Acquired AI disorder characterised by production of anti-cardiolipin/lupus anticoagulant autoantibodies, which interact with phospholipids involved in the coagulation cascade causing thrombophilia

86
Q

What are the cardinal features of antiphospholipid syndrome?

A
CLOT
C lotting disorder (thrombosis)
L ivedo reticularis
O bstetric complications (recurrent 1st T miscarriage)
T hrombocytopenia
87
Q

6 features of rheumatoid hands

A

1) Ulnar deviation
2) Boutonniere’s deformity (PIP flex, DIP hyperex)
3) Swan neck (PIP ex, DIP hyperflex)
4) Z-thumb (MCP flex, IP hyperex)
5) Loss of knuckle guttering
6) Dorsal subluxation of ulnar styloid process

88
Q

Extra-articular manifestations of RA

A
  • Rheum/MSK - CTS, De Quervain’s tenosynovitis, atlantoaxial sublux, Raynaud’s, osteoporosis
  • Resp - fibrosis, nodules, effusion, bronchiolitis obliterans, drug-related
  • Cardiac - IHD, pericarditis
  • Ophthalamic - keratoconjunctivitis sicca (Sjogren’s), episcleritis/scleritis, drug-related (cararacts)
  • Haem - Felty’s syndrome, AIHA, amyloidosis
89
Q

What is Felty’s syndrome?

A

RA + splenomegaly + dec WCC

90
Q

Investigations for RA

A

Bedside - obs, lung function test
Bloods - autoantibodies (RF, Anti-CCP), ESR/CRP, FBC
Imaging - XR, CXR

91
Q

Management of RA

A

Conservative - OT/PT
Medical:
- early maintenance: combo DMARDs and ST steroids
- further biologicals: TNF-a inhib (infliximab), Rituximab
- symptomatic: NSAIDs, steroids
- cover: PPI, bisphosphonates

92
Q

Specific signs of OA

A

Herberden’s nodes
Bouchard’s nodes
Squaring of C-MC joint
Absence of systemic features

93
Q

Specific signs of septic arthritis

A

Signs of systemic upset
Red hot swollen joint
Tender to palpate, warm to touch, effusion
Marked limitation of movement, inability to weight bear

94
Q

Specific signs of gout

A

Red hot swollen tender joint

Joint asp + synovial fluid analysis shows -ve birefrigent needle-shaped crystals

95
Q

Specific signs of psudogout

A

Acutely tender, red, hot, swollen joint

Joint asp + synovial fluid analysis shows inc WCC, +ve birefringent rhomboid shaped crystals

96
Q

Specific signs of RA

A

Early - swollen MCP + PIP joints, with wrist/MTP joint involvement
Late - Boutonniere, swan neck, Z thumb, ulnar dev

97
Q

Specific signs of psoriatic arthritis

A
Dactylitis
Enthesitis (pain, stiffness + tenderness of tendon insertions into bone)
Extra-articular features (plaques, nail changes, uveitis)
98
Q

Specific signs of ank spond

A

Schober’s test

Chest circumference expansion <5cm

99
Q

Specific signs of polymyalgia rheumatica

A

Normal muscle strength at initial presentation

Muscle tenderness proximally

100
Q

Specific signs of polymyositis

A

Proximal muscle weakness and atrophy
Difficulty arising from sitting position
Muscles are tender on palpation

101
Q

Specific signs of dermatomyositis

A

Gottron’s papules and heliotrope rash

Photosensitivity

102
Q

Specific signs of Sjogren’s syndrome

A
Dry eyes
Dry mouth
Parotid swelling
Vaginal dryness and dyspareunia
Dry cough
Dysphagia
103
Q

Specific signs of limited scleroderma

A
CREST
Calcinosis
Raynaud's
oEsophageal dysmotility
Sclerodactyly
Telangiectasia
104
Q

Specific signs of diffuse scleroderma

A

CREST + internal organs

  • pulmonary HTN/ILD
  • RHF, renal impairment