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My MRCP > Rheum: SLE, CTD > Flashcards

Rheum: SLE, CTD Flashcards

1
Q

What is the cause of discoid lupus?

A

Follicular keratin plugs

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2
Q

What is the treatment of discoid lupus?

A

Topical steroids –> hydroxychloroquine

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3
Q

Which antibodies are most specific for SLE?

A

Anti-SM

Anti-dsDNA

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4
Q

Which antibody is most sensitive for SLE

A

ANA

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5
Q

Why are C3 and C4 low during active lupus?

A

Formation of immune complexes causes consumption of complement

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6
Q

What is the pathogenesis of SLE?

A

Dysregulation of IgD

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7
Q

What is the treatment of SLE?

A

Hydroxychloroquine maintenance therapy
Internal organ involvement –> AZA, CYC, ritux
Prednisolone to induce remission

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8
Q

Which inflammatory markers are used to monitor SLE?

A

ESR

As CRP may be normal during active disease; if raised may indicate underlying infection

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9
Q

Apart from ESR, how else is SLE monitored?

A

Low complement

Anti-dsDNA titres

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10
Q

What antibody is associated specifically with neonatal lupus erythematosus?

A

Anti-Ro

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11
Q

What are the complications/features of congenital lupus?

A

Heart block
Skin rashes
Hepatosplenomegaly

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12
Q

What percentage of psoriasis patients develop arthritis?

A

10-20%

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13
Q

What are the 5 types of psoriatic arthritis?

A 
Symmetrical (similar to RA)
Asymmetrical oligo
Sacroilitis
DIP joint disease
Arthritis mutilans
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14
Q

What are other signs of psoriatic arthropathy?

A

Tenosynovitis
Dactylitis
Enthesitis
Nail pitting and onycholysis

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15
Q

What are the X-Ray appearances of psoriatic arthritis?

A

Co-existence of erosive changes and new bone formation
Periostitis
Pencil in cup appearance

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16
Q

What is rheumatoid factor?

A

IgM against IgG

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17
Q

Which HLA is RA associated with?

A

DR4

Especially Felty’s syndrome

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18
Q

What is Felty’s syndrome?

A

RA
Splenomegaly
Neutropaenia

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19
Q

What are the X-Ray features of RA?

A

Loss of joint space
Juxta-articular osteoporosis
Soft tissue swelling
Late - periarticular erosions, subluxation

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20
Q

What percentage of normal people have +ve HLA-B27?

A

10%

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21
Q

What percentage of ankylosing spondylitis patients have +ve HLA-B27?

A

90%

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22
Q

What is Schober’s sign?

A

An increase of less than 5cm on anterior spinal flexion when measuring the distance from 10cm above and 5cm below the left posterior iliac spine

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23
Q

What are the X-Ray features of ankylosing spondylitis?

A

Bamboo spine
Sacroilitis (subchondral erosions, sclerosis of vertebral corners)
Squaring of vertebrae
Syndesmophytes

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24
Q

What is bamboo spine?

A

Ossification of outer fibres of annulus fibrosus

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25
Q

What are the complications of ankylosing spondylitis?

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

What is the treatment of ankylosing spondylitis?

A

Exercise and physio
NSAIDs
DMARDS if peripheral joint involvement
TNF inhibitors - do not improve radiological progression

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27
Q

What is the most common organism causing septic arthritis?

A

S.aureus

Young sexually active adults - neisseria gonorrhoea

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28
Q

What is the cause of reactive arthritis?

A

Post STI form most common form in men, post-dysenteric form equal sex incidence

Shigella flexneri, salmonella typhi/enteritidis, yersinia, campylobacter, chlamydia trachomaatis

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29
Q

What is the triad of reactive arthritis?

A

Urethritis
Conjunctivitis/anterior uveitis
Arthritis - asymmetrical, lower limbs

Also dactylitis, circinate balanitis, keratoderma blenorrrhagica

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30
Q

What is the prognosis of reactive arthritis?

A

Symptoms last 4-6 months

25% have recurrent episodes, 10% chronic disease

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31
Q

In gout, where are the urate crystals deposited?

A

Superficial portions of the articular cartilage

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32
Q

What is the most common cause of gout?

A

Decreased excretion of uric acid - diuretics, CKD, lead toxicity

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33
Q

What are other causes of gout?

A

Increased production of uric acid - myeloproliferative disorder, cytotoxic drugs, severe psoriasis

Lesch-Nyhan syndrome

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34
Q

What are the X-Ray features of gout?

A 
Joint effusion
"Punched out" erosions with sclerotic margins in a juxta-articular distribution, often with overhanging edges
Eccentric erosions
No periarticular osteopenia
Soft tissue tophi
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35
Q

What is the double contour sign in gout?

A

Hyperechoic irregular band over the superficial margin of the joint cartilage, produce by deposition of monosodium urate crystals on the surface of the hyaline cartilage

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36
Q

Where do calcium pyrophosphate crystals deposit in pseudogout?

A

The middle layer of the hyaline cartilage, parallel to the bony cortex as a hyper-echoic irregular line embedded in the cartilage (with a normal hyaline cartilage surface)

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37
Q

What are risk factors for pseudogout if it develops at a younger age?

A 
Haemochromatosis
Hyperparathyroidism
Low magnesium
Low phosphate
Acromegaly
Wilson's disease
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38
Q

What is seen on joint aspiration in pseudogout?

A

Weakly positive birefringent rhomboid shaped crystals

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39
Q

What are the 3 types of systemic sclerosis?

A

Limited cutaneous
Diffuse cutaneous
Scleroderma

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40
Q

What are the features of limited cutaneous systemic sclerosis?

A

Raynaud’s may be first sign
Scleroderma of face an distal limbs
Subtype: CREST syndrome (calcinosis, Raynaud’s, oesophageal dysmotility, sclerodactyly, telangiectasia)

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41
Q

What are the features of diffuse cutaneous systemic sclerosis?

A

Scleroderma of trunk and proximal limbs

Respiratory (ILD and PAH) involvement, renal disease

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42
Q

Which antibodies are associated with systemic sclerosis?

A

Limited cutaneous: ACA
Diffuse cutaneous: Anti-Scl-70

Both: ANA in 90%, RF in 30%

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43
Q

What are the features of antiphospholipid syndrome?

A

Venous and arterial thombosis
Recurrent foetal loss
Thrombocytopenia
Livedo reticularis

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44
Q

Why is APTT high in APS?

A

Ex vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade

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45
Q

What is the treatment of antiphospholipid syndrome?

A

Primary thromboprophylaxis - low dose aspirin
Secondary thromboprophylaxis - lifelong warfarin, target INR 2-3
Recurrent VTE on warfarin - add low dose aspirin, target INR 3-4

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46
Q

What are the features of dermatomyositis?

A

Heliotrope rash around the eyelids
Gottron’s papules
Fatigue and weakness

47
Q

Which antibodies are associated with dermatomyositis?

A

ANA most common (60%)

Anti-Mi2 most specific (only seen in 25%)

48
Q

How is dermatomyositis diagnosed?

A

Elevated CK
EMG
Muscle biopsy

49
Q

What is Sharp’s syndrome?

A

Mixed connective tissue disease

50
Q

What are the features of mixed connective tissue disease?

A

Raynaud’s
Polyarthralgia
Myalgia
Dactylitis

Also photosensitive rash, scleroderma-like changes, oesophageal dysfunction, pleurisy/PAH/ILD, glomerulonephritis

51
Q

Which antibodies are positive in mixed connective tissue disease?

A

Anti-U1 ribonucleotide antibodies

ANA

52
Q

What is Raynaud’s disease?

A

Exaggerated vasoconstrictive response of the digital arteries and cutaneous arteriole to the cold or emotional stress

53
Q

What are the secondary causes of Raynaud’s?

A 
CTDs
Leukaemia
Type 1 cryoglobulinaemia
Vibrating tools
COCP
Cervical rib
54
Q

Which features of Raynaud’s suggest underlying CTDs?

A

Onset after 40
Unilateral
Rash, digital ulcers, calcinosis
Autoantibodies

55
Q

What is the management of Raynaud’s?

A

CCB - nifedipine

2nd line IV prostacyclin infusion (lasts several weeks/months)

56
Q

What is the cause of Marfan’s syndrome?

A

Mutation on FBN1 gene on chromosome 15 that codes for the protein fibrillin 1.

57
Q

What are the eye symptoms of Marfan’s?

A

Supero-temporal ectopia lentis
Blue sclera
Myopia

58
Q

What is dural ectasia?

A

Ballooning of the dural sac at the lumbrosacral level
60% Marfan’s patients
Lower back pain, headaches, bladder and bowel dysfunction

59
Q

What are the cardiac symptoms of Marfan’s?

A

MV prolapse

Aortic dissection

60
Q

What is the management of Marfan’s?

A

Regular ECHOs

BB/ACEIs

61
Q

What is the cause of polymyositis?

A

T cell mediated cytotoxic process against muscle fibres

Idiopathic, CTD, malignancy

62
Q

How is polymyositis diagnosed?

A

Elevated CK, LDH, aldolase, AST, ALT

EMG
Muscle biopsy

63
Q

Which antibodies are associated with polymyositis?

A

Anti-synthetase

Anti-Jo 1 (associated with more severe form associated with lung involvement)

64
Q

What is polyarteritis nodosa?

A

Vasculitis of medium sized arteries leading to aneurysm formation

65
Q

What is PAN associated with?

A

Hepatitis B

Middle aged men

66
Q

What are the features of PAN?

A 
Fever etc
Mononeuritis multiplex
Hypertension
Livedo reticularis
Haematuria and renal failure
67
Q

Which antibody is associated with PAN?

A

pANCA in 20%

68
Q

What is familial mediterranean fever and who gets it?

A

Autosomal recessive
Presents in 2nd decade
Turkish, Armenian, Arabic descent

69
Q

What are the features of FMF?

A 
Pyrexia
Abdo pain from peritonitis
Pleurisy
Pericarditis
Arthritis
Erysipeloid rash
70
Q

What is the management of FMF?

A

Colchicine

71
Q

What is the cause and pathophysiology of Langerhans Cell Histiocytosis?

A

Abnormal proliferation of dendritic cells/histiocytes

Formation of multiple granulomas

72
Q

What are the features of Langerhans Cell Histiocytosis?

A

Bone pain (skull or proximal femur)
Rash
Recurrent otitis media/mastoiditis

73
Q

What is seen on electron microscopy in LCH?

A

Tennis racket shape Birbeck granules

74
Q

What are the features of Still’s disease?

A

Arthralgia
Swinging pyrexia that peaks early evening
Salmon pink maculopapular rash
Lymphadenopathy

75
Q

What is the diagnostic criteria of Still’s disease?

A

Yamaguchi

76
Q

How is Still’s disease diagnosed?

A

RF and ANA negative

Elevated ferritin

77
Q

What is osteopetrosis?

A

Failure of osteoclastic bone resorption –> cortical thickening and sclerosis –> thick and brittle bones

Autosomal dominant

78
Q

What are the features of osteopetrosis?

A

Fractures

79
Q

How is osteopetrosis diagnosed?

A

XR: symmetrical and diffuse skeletal sclerosis - stone bone

Normal Ca, phosphate, ALP, PTH

80
Q

What is the cause of osteogenesis imperfecta?

A

Autosomal dominant inheritance of mutation in gene coding for type 1 collagen

Decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides

81
Q

What are the features of osteogenesis imperfecta?

A

Fractures
Blue sclerae
Deafness secondary to otosclerosis
Dental imperfections

82
Q

What types of collagen form cell surfaces and hairs, and the basal lamina?

A

Cell surfaces, hairs - Collagen type 5 - Classic Ehlers-Danlos

Basal lamina - Type 4 - Alport’s and Goodpasture’s syndrome

83
Q

What is the cause of Paget’s disease?

A

Excessive osteoclastic resorption followed by increased osteoclastic activity - uncontrolled bone turnover

84
Q

How is Paget’s disease diagnosed?

A

Normal Ca, PO4, PTH

Isolated raised ALP

85
Q

What is the treatment of Paget’s disease?

A

Bisphosphonates

86
Q

What are the features of spinal stenosis?

A

Gradual onset uni/bilateral leg pain, with or without back pain

Numbness and weakness

Worse on walking, resolves when sitting/leaning forwards/crouching down

Normal examination

87
Q

What is osteomalacia?

A

Softening of the bones secondary to low vitamin D

88
Q

What are the symptoms of osteomalacia?

A

Bone pain
Fractures
Proximal myopathy

89
Q

How is osteomalacia diagnosed?

A

High ALP
Low calcium and phosphate
Translucent bands (Looser’s zones) on X-Ray

90
Q

What are the complications of Paget’s disease?

A 
Cranial nerve entrapment
Bone sarcoma
Fractures
Skull thickening
High output cardiac failure
91
Q

What are the risk factors for avascular necrosis of the hip?

A

Long term steroid use
Chemotherapy
Alcohol excess
Trauma

92
Q

What is seen on X-Ray in avascular necrosis of the hip?

A

Osteopenia
Microfractures
Crescent sign (collapse of the articular surface)

93
Q

How do bisphosphonates work?

A

Analogues of pyrophosphate, a molecular which decreases demineralization in bone

Inhibits osteoclasts by reducing recruitment and promoting apoptosis

94
Q

What are 3 side effects of bisphosphonates?

A

Oesophagitis
Osteonecrosis of the jaw
atypical stress fractures of the proximal femoral shaft

95
Q

What type of retinopathy is seen in hydroxychloroquine use?

A

Bull’s eye

96
Q

How does sulfasalazine work?

A

Prodrug for 5-ASA

Decreases neutrophil chemotaxis, suppresses proliferation of lymphocytes and pro-inflammatory cytokines

97
Q

In which patients should use of sulfasalazine be avoided?

A

G6P deficiency

Allergy to aspirin/sulphonamides (cross-sensitivity)

98
Q

What are 6 side effects of sulfasalazine?

A 
Oligospermia
Stevens-Johnson syndrome
Pneumonitis
Myelosuppression
Heinz body/megaloblastic anaemia
Coloured tears
99
Q

What are the side effects of leflunomide?

A 
Diarrhoea
Hypertension
WL
Peripheral neuropathy
Myelosuppression
Pneumonitis
100
Q

What are the rules when stopping leflunomide?

A

Wash out period of a year

Requires co-administration of cholestyramine

101
Q

In what condition are anti-yo antibodies seen?

A

Patients with breast cancer and paraneoplastic syndrome

Associated with cerebellar degeneration and ataxia

102
Q

What is DeQuervain’s tenosynovitis?

A

Sheath containing the extensor pollicis brevis and abductor pollicis longus tendons is inflamed

103
Q

What are the features of De Quervain’s tenosynovitis?

A

Pain on radial side of wrist
Tenderness over radial styloid process
Painful abduction of thumb against resistance
Finkelstein’s test - examiner pulls thumb in ulnar deviation and longitudinal traction - causes pain over radial styloid process

104
Q

Which nerve is compressed in carpal tunnel syndrome?

A

Median

105
Q

What are 6 causes of carpal tunnel syndrome?

A 
Pregnancy
Lunate fracture
RA
Hypothyroid
Acromegaly
Idiopathic
106
Q

What is seen on examination in Carpal Tunnel syndrome?

A

Tinel’s and Phalen’s sign
Weakness of thumb abduction (abductor pollicis longus)
Wasting of thenar eminence

107
Q

What is surgical decompression of Carpal tunnel syndrome?

A

Division of flexor retinaculum

108
Q

What are the general features of prolapsed disc?

A

Dermatomal leg pain associated with neurological deficits

Leg pain > back pain
Pain worse when standing

109
Q

Which vertebral disc is prolapsed when a positive femoral stretch test is seen, with weak quadriceps, reduced knee reflex, and sensory loss over anterior knee?

A

L3 or L4

110
Q

What is seen in L5 disc prolapse?

A

Sensory loss over dorsum of foot
Weakness in foot and big toe dorsiflexion
Reflexes intact
Positive sciatic nerve stretch test

111
Q

What is seen in S1 disc prolapse?

A

Positive sciatic nerve stretch test
Weakness in plantar flexion of foot
Reduced ankle reflex
Sensory loss over posterolateral aspect of leg and lateral aspect of foot

112
Q

What level of rheumatoid factor and ANA titre is significant?

A

> 1:160

>1:80

113
Q

What skin lesions are seen in Behcet’s disease?

A

Erythema nodosum
Acneiform
Migratory thrombophlebitis
Vasculitic

114
Q

What is reflex sympathetic dystrophy?

A

Complex regional pain syndrome
Disproportionate pain secondary to injury/surgery
Skin changes from autonomic dysfunction

My MRCP (43 decks)

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