Connective Tissue Disease Flashcards

1
Q

what are the 11 diagnostic criteria for SLE?

A
serositis
oral ulcers
arthritis
photosensitivity
blood dyscrasia
renal involvement
ANA 
immune disorder
neurological symptoms
malar rash
discoid rash
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2
Q

what is required for a diagnosis of SLE?

A

4 out of the 11 diagnostic criteria (SOAP BRAIN MD)

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3
Q

what investigations would you do if you suspected SLE?

A

FBC, LFT, U&E
ANA, anti-dsDNA
ESR
urinalysis!!

depending on what systems are affected: renal biopsy, MRI head, echo

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4
Q

what do you look for on urinalysis in SLE?

A

proteinuria

cell casts

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5
Q

what factors may increase the risk of developing SLE?

A

genetic - HLA DR2 and DR3
viruses - EBV
drugs

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6
Q

how does the arthritis in SLE differ from that of RA etc?

A

it is non-erosive

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7
Q

what investigations are best for monitoring disease progression in SLE?

A

anti-dsDNA
complement
ESR

also check blood pressure and urine dip

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8
Q

what drugs can cause SLE?

A
hydralazine
minocycline
isoniazid
chlorpromazine
methyldopa
phenytoin
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9
Q

how would you treat severe flares of SLE?

A

IV cyclophosphamide and high dose prednisolone

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10
Q

what is the maintenance treatment for SLE?

A

hydroxychloroqine
simple analgesia
NSAIDs
low dose steroids or steroid sparing agents (aza, metho, myco)

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11
Q

how are the cutaneous symptoms of SLE managed?

A

hydroxychloroquine
topical steroids
high factor sun block
avoid sun exposure

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12
Q

what are the possible side effects of hydroxychloroquine?

A

retinopathy

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13
Q

how does dermatomyositis present?

A

insidious onset
symmetrical proximal muscle weakness
myalgia
may have arthralgia

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14
Q

what are the complications of dermatomyositis?

A

aspiration pneumonia
respiratory failure
dysphagia
lung fibrosis

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15
Q

what is the name given to the papular rash seen over the knuckles and knees/elbows in dermatomyositis?

A

gottron’s papules

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16
Q

what skin signs may you see in dermatomyositis?

A

gottron’s papules
heliotrope rash
macular rash
subcutaneous calcification

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17
Q

what is the name given to the lilac/purple rash over the eyelids?

A

heliotrope rash

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18
Q

how would you investigate dermatomyositis?

A
muscle enzymes (CK, AST, ALT, LDH)
electromyography
muscle biopsy (definitive)
autoantibodies - anti Jo-1, anti Mi-2
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19
Q

what must you screen for in a patient with dermatomyositis?

A

cancer (esp. gastric)

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20
Q

how would you manage dermatomyositis?

A

prednisolone
hydroxychloroquine for skin problems
immunosuppresives e.g. azathioprine, methotrexate, ciclosporin

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21
Q

how does sjogrens syndrome present?

A

recurrent conjunctivitis
dry mouth, dry eyes
difficulty swallowing
dental problems

22
Q

what investigations would you do if you suspected sjogrens?

A
schirmer's test
salivary gland scintigraphy
ESR
ANA
RF
23
Q

how would you manage sjogrens?

A

artificial tears/saliva
eye ointments
treatment of secondary infections

24
Q

what is the clinical significance of positive anti-Ro and anti-La antibodies in women?

A

their children have a higher risk of congenital heart block

25
Q

what are the features of limited cutaneous systemic sclerosis?

A
skin involvement limited to face, hands and feet
calcinosis
raynauds
oesophageal dysmotility
sclerodactyly
telangiectasia
26
Q

what are the features of diffuse cutaneous systemic sclerosis?

A

diffuse skin involvement and organ fibrosis lung - fibrosis, pulmonary hypertension
bowel - dysmotility on larger scale than the limited form
heart - cardiomyopathy, pericardial effusions
kidneys - failure, renal crisis

27
Q

in what condition do anticentromere antibodies occur?

A

LIMITED systemic sclerosis

28
Q

how would you manage systemic sclerosis?

A

control BP - ACEi, ATII blockers
immunosuppression if organ involvement
treat raynauds
treat the complications e.g. nutritional support if severe GI dysmolity

29
Q

how is raynauds treated?

A

conservative: avoid cold exposure, warm gloves and socks

pharmo:
calcium channel blockers
IV prostacyclin if severe

30
Q

how does polymyalgia rheumatica present?

A

pain and morning stiffness in the neck, shoulder and pelvic girdle

31
Q

what disease may polymyalgia rheumatic be associated with?

A

temporal arteritis

32
Q

what are the symptoms of temporal arteritis?

A

headaches, scalp tendernedd, jaw claudication, visual problems

33
Q

what investigations would you do in polymyalgia rheumatica?

A

no definitive test

do ESR

34
Q

how would you treat polymyalgia rheumatica?

A

steroids (years)

consider gastric and bone protection

35
Q

what investigations would you do in temoral arteritis?

A

ESR

temporal artery biopsy

36
Q

how would you manage temporal arteritis?

A

start prednisolone ASAP 40-60mg to avoid irreversible blindness

consider gastric and bone protection

37
Q

what should you do if a temporal artery biopsy comes back negative in suspected temporal arteritis and why?

A

treat anyway because there may be skip lesions

38
Q

what is the classic triad of wegener’s granulomatosis?

A

renal involvement
upper respiratory tract involvement
pulmonary involvement

(ELK - ent, lungs, kidneys)

39
Q

how might wegener’s granulomatosis present?

A

classic triad (ELK)

  • nosebleeds
  • rhinorrhoea
  • sinusitis
  • cough
  • SOB

non-specific symptoms e.g. fatigue, weight loss

40
Q

what investigations would you do for wegener’s?

A
FBC
ESR
U&E
C-ANCA, P-ANCA 
urinalysis
lung function tests and CXR
may need biopsies - nasal mucosa, lung, kidney
41
Q

how would you manage wegener’s granulomatosis?

A

cyclophosphamide and prednisolone initially
once in remission, replace cyclo with azathioprine
methotraxate and leflunomide can also be used

42
Q

what disorder is anti-dsDNA most characteristic for?

A

SLE

43
Q

what disorder is anticardiolipin most characteristic for?

A

antiphospholipid syndrome

44
Q

what disorder is anticentromere antibody most characteristic for?

A

limited cutaneous systemic sclerosis

45
Q

what disorder is anti-Jo-1 most characteristic for?

A

dermatomyositis

46
Q

what disorder is anti-La most characteristic for?

A

sjogrens

47
Q

what disorder is anti-Ro most characteristic for?

A

sjogrens

48
Q

what disorder is anti-Scl-70 most characteristic for?

A

diffuse cutaneous systemic sclerosis

49
Q

what disorder is c-ANCA most characteristic for?

A

wegeners granulomatosis

50
Q

what disorder is p-ANCA most characteristic for?

A

vasculitis e.g. churg-strauss, microscopic polyangiitis

51
Q

what can cause a raised ESR >100?

A

vasculitis
sepsis
haematological malignancy
polymyalgia rheumatica

52
Q

what disorder is anti jo-1 most characteristic for?

A

dermatomyositis