Rheumatology 2 Flashcards

1
Q

What is ankylosing spondylitis?

A

Seronegative spondyloarthropathy

Chronic autoimmune inflammatory disease of the axial skeleton

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2
Q

What is the typical onset for AS?

A

20s-30s

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3
Q

Give four risk factors for AS?

A

HLA-B27 positivity
Male gender
FH of AS or psoriasis
Recent GU infection

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4
Q

Give three aspects of the pathogenesis of AS.

A

Lymphocyte and plasma cell infiltration
Local bone erosion and enthesitis
Syndesmophytes and bamboo spine

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5
Q

What is the typical presentation of AS?

A

Pain before the age of 35 (lower back, buttocks, anterior chest)Morning stiffness relieved by exercise
Can wake patient from night
Asymptomatic between episodes

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6
Q

What is the characteristic of peripheral joint involvement in AS?

A

Asymmetrical

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7
Q

Give three signs of AS.

A

Question mark posture - thoracic kyphosis with retention of lumbar lordosis
reduced chest expansion
Paraspinal muscle wasting
Schober’s test<5cm

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8
Q

What are the 4As of other manifestations of AS?

A
Achilles tendonopathy
Anterior uveitis
Apical lung fibrosis
Aortic regurgitation
Atlanto-axial subluxation
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9
Q

What is the initial investigation in AS and what is found?

A

X-Ray

Sacroilitis, sclerosis and fusion

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10
Q

Why is MRI used in AS?

A

Shows sacroiliitis and enthesitis better

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11
Q

What medications are used in AS?

A

Adalimumab SC
Certolizumab pegol SC
Etanercept SC
Infliximab IV

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12
Q

What is the name of the heterogenous group of heritable connective tissue disorders?

A

Ehlers-Danlos Syndrome

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13
Q

How many subtypes of EDS are there?

A

13

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14
Q

What are the most important types of EDS?

A
  1. Classical
  2. Classical-like
  3. Cardiac-valvular
  4. Vascular
  5. Hypermobile (50%)
  6. Kyphoscoliotic
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15
Q

What are the skin symptoms of EDS?

A

Increased skin elasticity and fragility
Easy bruising
Widening scars
Skin splitting on forehead, knees, and elbows

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16
Q

What are the join symptoms of EDS?

A

Laxity and hypermobility
Spontaneous dislocations and subluxations - shoulder, knee, TMJ
Pes planus

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17
Q

Give some other symptoms of the various types of EDS.

A

Cardio - dizziness, palpitations, dysautonomia
MSK - herniation, hypotonia, kyphoscoliosis
Other - uterocervical prolapse, fatiguability

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18
Q

How is EDS diagnosed?

A

Hypermobile type - clinical

Other types - molecular genetic testing

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19
Q

What is the management of EDS?

A

Physiotherapy
Pain management and CBT
Certain forms use celiprolol.

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20
Q

What is celiprolol?

A

Beta-1 adrenoreceptor antagonist

Prevents arterial dissections and ruptures

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21
Q

What is the mutation in Marfan’s syndrome?

A

Fibrillin 1 gene
Found on chromosome 15q21
2/3 familial transmission, 1/3 sporadic mutations associated with advanced paternal age

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22
Q

What are signs of Marfan’s syndrome?

A

Dolichostenomelia (long arms and legs compared to trunk)
Tall and thin
Arachnodactyly
Thoracolumbar and sacral striae

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23
Q

What are patients with Marfan’s at risk of?

A

Pleural rupture and pneumothorax
Cardio - AAA/dissection/aortic or mitral valve problems
Eyes - Lens dislocation, closed angle glaucoma
Hypermobility and arthralgia

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24
Q

How are Marfan’s patients monitored?

A

Annual echo

Cardiac MRI/CT every 5 years

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25
Q

Apart from managing the complications, how are Marfan’s patients treated?

A

Prophylactic beta-blockers

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26
Q

What is the name of the inflammatory condition characterized by severe bilateral pain and morning stiffness of the shoulder, neck, and pelvic girdle?

A

Polymyalgia rheumatica

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27
Q

What is the main risk factor for PMR?

A

Giant cell arteritis

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28
Q

What is the prodrome of PMR?

A

Flu-like

Sudden, over 1-2 weeks

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29
Q

What is the diagnostic criteria for PMR?

A

1) Age over 50
2) Symptoms > 2 weeks
3) Bilateral shoulder and/or pelvic girdle aching WITHOUT WEAKNESS
4) Morning stiffness > 45m
5) Raised ESR/CRP

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30
Q

What bloods are required in diagnosis of PMR and exclusion of other conditions?

A
ESR, CRP, viscosity
FBC, UEs, LFTs, TFTs, Bone profile
Protein electrophoresis
Creatinine kinase
RF
ANA and anti-CCP
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31
Q

What other investigations are required in PMR?

A

Urinalysis and Bence Jones
CXR
USS shoulders/hips

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32
Q

What is the treatment of PMR?

A

PMR is extremely sensitive to steroids.

Steroid regimen - PO prednisolone gradually decreasing over 1-2 years

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33
Q

What should be done if a PMR patient is high risk for fragility fractures?

A

Perform DEXA

Give bisphosphonates with calcium and Vit D supplements

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34
Q

What is myositis?

A

CTD involving inflammation of striatal muscle

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35
Q

What are the types of polymyositis?

A

Dermatomyositis
Polymyositis
Necrotizing autoimmune myopathy
Sporadic inclusion body myositis

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36
Q

What are the main symptoms of myositis?

A

Diffuse proximal weakness
Fatigue and myalgia
Distal muscles spared until late in disease

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37
Q

How does diffuse proximal weakness present?

A

Difficulty rising from chair
Climbing steps
Lifting objects
Combing hair

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38
Q

What can myositis progress to?

A

Pharyngeal weakness - dysphagia
Laryngeal weakness - dysphonia
Respiratory failure and lung fibrosis

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39
Q

What are the signs of dermatomyositis, excluding the muscle symptoms?

A

Heliotrope discolouration of eyelids and periorbital oedema
Gottron’s papules
Ulcerative vasculitis
Photosensitive macular rash in shawl pattern
Scales and shininess

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40
Q

What do lab tests show in myositis?

A

Elevated CK, aldolase, LDH
PM: anti-Jo1
DM: ANA, anti-Mi2

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41
Q

How is myositis diagnosed?

A

EMG - spontaneous fibrillation potentials at rest
Muscle biopsy - fibre necrosis and inflammatory infiltrate
MRI - inflammation

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42
Q

What should be excluded in elderly patients with dermatomyositis?

A

Malignancy

43
Q

What is the treatment of myositis or dermatomyositis?

A

High dose systemic steroids
Immune suppression - azathioprine, cyclophosphamide, mycophenolate mofetil
IVIG

44
Q

What is pseudogout?

A

Joint inflammation caused by deposition of calcium pyrophosphate dihydrate crystals

45
Q

Attacks of pseudogout can be precipitated by….

A

Dehydration
Intercurrent illness
Dysregulation of calcium-phosphate homeostasis
Surgery or trauma

46
Q

How does pseudogout present?

A

Acute monoarticular/oligoarticular arthritis
Typically knees or wrists
Similar but milder than gout
There may be fever

47
Q

How does Chronic CPP arthritis present?

A

Destructive changes more severe than OA

Neuropathic joint

48
Q

How is pseudogout present?

A

X-Ray - linear opacification of articular cartilage

Joint aspiration - purulent, neutrophils, crystals.

49
Q

What are the characteristics of joint crystals in pseudogout?

A

Intracellular and extracellular weakly positive birefringent rhomboid crystals.

50
Q

How is pseudogout managed?

A

Symptomatic - ice packs, rest, therapeutic aspiration, NSAIDs, colchicine

51
Q

What percentage of psoriasis patients develop a seronegative spondyloarthropathy?

A

5-25%

52
Q

Apart from an inflammatory arthritis, how does psoriatic arthritis present?

A
Dactylitis
Enthesitis
Nail changes
Anterior uveitis
Sacroilitis
Aortitis
53
Q

What are the different types of psoriatic arthritis?

A
  • Symmetrical polyarthritis rheumatoid pattern (DIPs>MCPs)
  • Oligoarthritis
  • Lone DIP disease
  • Arthritis mutilans
  • Spondylitic pattern and sacroilitis
54
Q

How is psoriatic arthritis diagnosed?

A

Increased ESR/CRP
HLA-B27 and IgA
Joint aspiration shows neutrophils
X-Ray for erosions

55
Q

How is psoriatic arthritis managed?

A

Methotrexate address both skin and joint disease

2nd line: sulfasalazine, leflunomide

56
Q

Why should hydroxychloroquine be avoided in psoriatic arthritis?

A

May cause an exfoliative dermatitis

57
Q

What biologics should be considered in psoriatic arthritis?

A

TNF inhibitors

After inadequate response to one DMARD, or patients with axial disease

58
Q

What are the subgroups of reactive arthritis?

A

Post-enteric - campylobacter, salmonella, shigella, yersinia

Post-venereal - HIV or chlamydia

59
Q

How long after infection does reactive arthritis develop?

A

Minimum of 1 and maximum of 6 weeks

60
Q

What is the typical arthritis in reactive arthritis?

A

Asymmetrical, predominantly lower extremity oligoarthritis

61
Q

What are the other symptoms of reactive arthritis?

A
Malaise and fever
Reiter's syndrome
Nail changes
Mouth ulcers
Erythema nodosum/circinate balanitis
62
Q

What is seen on bloods in reactive arthritis?

A

High CRP and ESR
HLA-B27 pos, RF and ANA neg
Normochromic normocytic anaemia, leucocytosis and thrombocytosis

63
Q

What does joint aspiration show in reactive arthritis?

A

Polymorphonuclear leukocytes

64
Q

What are important investigations in reactive arthritis?

A

Culture of stools, throat, and urogenital tract samples

65
Q

What is the management of reactive arthritis?

A

Corticosteroids (IA or PO)
Abx
Limited use for sulfasalazine

66
Q

What is the pathophysiology of systemic sclerosis?

A

Increased fibroblast activity resulting in the abnormal growth of connective tissue and collagen, causing vascular damage and fibrosis

67
Q

What are the types of scleroderma?

A

Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Overlap systemic sclerosis
Systemic sclerosis sine scleroderma

68
Q

What is the difference between limited and diffuse cutaneous systemic sclerosis?

A

Limited - skin affected only on face, forearms, and lower leg. Milder disease and slow progression.

Diffuse - skin also involves upper arms, thighs, or trunk. Rapid progression and high mortality

Both have symptoms included in CREST acronym

69
Q

What does CREST stand for?

A
Calcinosis
Raynaud's
Esophageal dysmotility
Sclerodactyly
Telangiectasia
70
Q

What are five signs of scleroderma?

A
Sclerodactyly
Fingertip pitting and ulceration
Calcinosis
Microstomia
Telangiectasia
Joint pain
71
Q

What are the pulmonary features of scleroderma?

A

80% pulmonary fibrosis

Pulmonary arterial hypertension is the leading cause of death

72
Q

What are the GI features of scleroderma?

A

GORD
Watermelon stomach and GI bleed from gastric antral vascular ectasia
Reduced small bowel motility

73
Q

What autoantibodies are associated with scleroderma?

A

Anti-topoisomerase 1 - diffuse
Anti-centromere - limited
Anti-RNA polymerase
ANA

74
Q

What is the main management of scleroderma?

A

Immunotherapy - MTX, MMF, CYC

75
Q

How is the following managed in scleroderma?

1) Raynaud’s
2) Pulmonary fibrosis
3) PAH
4) Renal crisis
5) Contractures/calcinosis

A

1) Nifedipine
2) CYC or MMF infusion
3) Bosentan, ambrisentan, sildenafil
4) ACEIs plus dialysis
5) Surgical procedures, aluminium hydroxide

76
Q

What is scleroderma associated with?

A

Breast, lung, and haematological malignancies

Sjogrens syndrome

77
Q

What are five risk factors for septic arthritis?

A
Increasing age
Diabetes mellitus
Prior joint damage e.g. RA
Joint surgery
Immunodeficiency
78
Q

What are the features of gonococcal septic arthritis?

A

Fever
Arthralgia
Multiple skin lesions
Tenosynovitis

79
Q

What are the symptoms of septic arthritis?

A

Single swollen, red, hot joint
Severe pain on active or passive movement
Fever and rigors

80
Q

How is septic arthritis diagnosed?

A

Blood cultures
Increased CRP and WCC
Synovial fluid: leukocytes, gram staining and culture, polarising microscopy
-X-ray: osteomyelitis, fat pad displacement, swelling of capsule and soft tissue, joint space widening due to effusion

81
Q

What is the treatment of septic arthritis?

A

Surgical drainage

High dose IV abx that cover s.aureus and strep A

82
Q

What is Sjogren’s syndrome?

A

Chronic lymphocytic infiltration of exocrine glands

83
Q

What are two risk factors for Sjogren’s syndrome?

A

HLA B8 or DR3

Vitamin D deficiency

84
Q

How does Sjogren’s syndrome present?

A
Xerophthalmia
Xerostemia
Recurrent bilateral parotitis
Dry cough and dysphagia
Fatigue
85
Q

How is Sjogren’s syndrome diagnosed?

A

RF and ANA, anti-Ro and anti-La

Schirmer test <5mm (normal - 15mm)

86
Q

How is Sjogren’s syndrome managed?

A

Artificial tears and saliva replacement
Avoid anti-cholinergics
Pilocarpine tablets

87
Q

What are the two main aspects of pathophysiology of vasculitis?

A

Vessel wall destruction leading to aneurysm or rupture

Stenosis leading to tissue ischaemia and necrosis

88
Q

Name the large and medium vessel vasculitides?

A

Large: GCA, Takayasu’s
Medium: PAN, Kawasaki

89
Q

Name the ANCA-associated vasculitides.

A

Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis

90
Q

Apart from those associated with ANCA, name three other small vessel vasculitides.

A

Cryoglobulinaemic vasculitis
Henoch-Schonlein Purpura (IgA vasculitis)
Hypocomplementaemic urticarial vasculitis

91
Q

What are the two forms of ANCA and which conditions are they found in?

A

Cytoplasmic (c-ANCA) - GPA

Perinuclear (p-ANCA) - MPA and EGPA

92
Q

Name five presentations of a large vessel vasculitis.

A
Arm claudication 
Diminished pulses
Jaw claudication
Aortic arch aneurysm
Visual loss
93
Q

Which arteries does Takayasu arteritis affect?

A

Aorta and great vessels

94
Q

How can Takayasu arteritis present?

A

Systemic upset including arthralgia and myalgia
Arm claudication and loss of pulses
Hypertension from renal artery stenosis
Stroke and visual change

95
Q

How is Takayasu arteritis diagnosed?

A

F-FDG PET-CT scan

96
Q

How is Takayasu arteritis and PAN managed?

A

PO prednisolone

MTX/AZA/MMF if steroid resistant

97
Q

What is polyarteritis nodosa?

A

Necrotizing vasculitis of medium sized arteries

98
Q

What is a risk factor for PAN?

A

Hepatitis B

99
Q

How does PAN present?

A
Systemic upset
Vasculitis rash and livedo reticularis, bullous and vesicular lesions
GI haemorrhage
Mononeuritis multiplex
Kidney and pancreas
100
Q

What is GPA?

A

Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract.

101
Q

How does GPA present?

A
Sinusitis, epistaxis, crusts, septal perforation, saddle nose, gingivitis
Haemoptysis
Glomerulonephritis and haematuria
Vasculitic skin change
MNM
102
Q

How is GPA diagnosed?

A

Lung/nose biopsy
c-ANCA, CRP, ESR
CXR and high resolution CT

103
Q

What is the treatment of GPA?

A

CYC
AZA maintenance
RTX relapse

104
Q

What is the triad of features of Still’s disease?

A

Persistent high spiking fevers
Joint pain
Salmon coloured bumpy rash