Connective Tissue Disease

Question 1

what are the 11 diagnostic criteria for SLE?

Answer 1

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

Question 2

what is required for a diagnosis of SLE?

Answer 2

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

Question 3

what investigations would you do if you suspected SLE?

Answer 3

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

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

Question 4

what do you look for on urinalysis in SLE?

Answer 4

proteinuria

cell casts

Question 5

what factors may increase the risk of developing SLE?

Answer 5

genetic - HLA DR2 and DR3
viruses - EBV
drugs

Question 6

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

Answer 6

it is non-erosive

Question 7

what investigations are best for monitoring disease progression in SLE?

Answer 7

anti-dsDNA
complement
ESR

also check blood pressure and urine dip

Question 8

what drugs can cause SLE?

Answer 8

hydralazine
minocycline
isoniazid
chlorpromazine
methyldopa
phenytoin

Question 9

how would you treat severe flares of SLE?

Answer 9

IV cyclophosphamide and high dose prednisolone

Question 10

what is the maintenance treatment for SLE?

Answer 10

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

Question 11

how are the cutaneous symptoms of SLE managed?

Answer 11

hydroxychloroquine
topical steroids
high factor sun block
avoid sun exposure

Question 12

what are the possible side effects of hydroxychloroquine?

Answer 12

retinopathy

Question 13

how does dermatomyositis present?

Answer 13

insidious onset
symmetrical proximal muscle weakness
myalgia
may have arthralgia

Question 14

what are the complications of dermatomyositis?

Answer 14

aspiration pneumonia
respiratory failure
dysphagia
lung fibrosis

Question 15

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

Answer 15

gottron’s papules

Question 16

what skin signs may you see in dermatomyositis?

Answer 16

gottron’s papules
heliotrope rash
macular rash
subcutaneous calcification

Question 17

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

Answer 17

heliotrope rash

Question 18

how would you investigate dermatomyositis?

Answer 18

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

Question 19

what must you screen for in a patient with dermatomyositis?

Answer 19

cancer (esp. gastric)

Question 20

how would you manage dermatomyositis?

Answer 20

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

Question 21

how does sjogrens syndrome present?

Answer 21

recurrent conjunctivitis
dry mouth, dry eyes
difficulty swallowing
dental problems

Question 22

what investigations would you do if you suspected sjogrens?

Answer 22

schirmer's test
salivary gland scintigraphy
ESR
ANA
RF

Question 23

how would you manage sjogrens?

Answer 23

artificial tears/saliva
eye ointments
treatment of secondary infections

Question 24

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

Answer 24

their children have a higher risk of congenital heart block

Question 25

what are the features of limited cutaneous systemic sclerosis?

Answer 25

``` skin involvement limited to face, hands and feet calcinosis raynauds oesophageal dysmotility sclerodactyly telangiectasia ```

Question 26

what are the features of diffuse cutaneous systemic sclerosis?

Answer 26

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

Question 27

in what condition do anticentromere antibodies occur?

Answer 27

LIMITED systemic sclerosis

Question 28

how would you manage systemic sclerosis?

Answer 28

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

Question 29

how is raynauds treated?

Answer 29

conservative: avoid cold exposure, warm gloves and socks pharmo: calcium channel blockers IV prostacyclin if severe

Question 30

how does polymyalgia rheumatica present?

Answer 30

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

Question 31

what disease may polymyalgia rheumatic be associated with?

Answer 31

temporal arteritis

Question 32

what are the symptoms of temporal arteritis?

Answer 32

headaches, scalp tendernedd, jaw claudication, visual problems

Question 33

what investigations would you do in polymyalgia rheumatica?

Answer 33

no definitive test | do ESR

Question 34

how would you treat polymyalgia rheumatica?

Answer 34

steroids (years) | consider gastric and bone protection

Question 35

what investigations would you do in temoral arteritis?

Answer 35

ESR | temporal artery biopsy

Question 36

how would you manage temporal arteritis?

Answer 36

start prednisolone ASAP 40-60mg to avoid irreversible blindness consider gastric and bone protection

Question 37

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

Answer 37

treat anyway because there may be skip lesions

Question 38

what is the classic triad of wegener's granulomatosis?

Answer 38

renal involvement upper respiratory tract involvement pulmonary involvement (ELK - ent, lungs, kidneys)

Question 39

how might wegener's granulomatosis present?

Answer 39

classic triad (ELK) - nosebleeds - rhinorrhoea - sinusitis - cough - SOB non-specific symptoms e.g. fatigue, weight loss

Question 40

what investigations would you do for wegener's?

Answer 40

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

Question 41

how would you manage wegener's granulomatosis?

Answer 41

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

Question 42

what disorder is anti-dsDNA most characteristic for?

Answer 42

SLE

Question 43

what disorder is anticardiolipin most characteristic for?

Answer 43

antiphospholipid syndrome

Question 44

what disorder is anticentromere antibody most characteristic for?

Answer 44

limited cutaneous systemic sclerosis

Question 45

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

Answer 45

dermatomyositis

Question 46

what disorder is anti-La most characteristic for?

Answer 46

sjogrens

Question 47

what disorder is anti-Ro most characteristic for?

Answer 47

sjogrens

Question 48

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

Answer 48

diffuse cutaneous systemic sclerosis

Question 49

what disorder is c-ANCA most characteristic for?

Answer 49

wegeners granulomatosis

Question 50

what disorder is p-ANCA most characteristic for?

Answer 50

vasculitis e.g. churg-strauss, microscopic polyangiitis

Question 51

what can cause a raised ESR >100?

Answer 51

vasculitis sepsis haematological malignancy polymyalgia rheumatica

Question 52

what disorder is anti jo-1 most characteristic for?

Answer 52

dermatomyositis