Connective tissue diseases Flashcards

1
Q

sx in systemic sclerosis

A
skin fibrosis and vascular disease
raynaud's
GI dysmotility -> GORD
sclerodactyly
telangiectasia
pulmonary hypertension + lung fibrosis
kidney problems -> HTN
calcinosis (calcium deposits at fingertips)
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2
Q

what is polymyositis

A

inflammation of PROXIMAL muscle causing muscle weakness

dermatomyositis if skin is involved

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3
Q

symptoms in polymyositis

A

progressive muscle weakness - difficult reaching overhead, climbing stairs, standing up

fever

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4
Q

ix in polymyositis

A
raised muscle enzymes
ESR/CRP raised (differentiates from PMR)
ANA antibody positive
EMG
muscle biopsy
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5
Q

increased risk of what in polymyositis

A

malignancy - need to screen for this!!!

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6
Q

rx polymyositis

A

prednisolone

immunosuppressant e.g. methotrexate, azathioprine

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7
Q

what is sjogren’s syndrome

A

dry eyes and dry mouth enlarged salivary glands

also have raynauds and fatigue

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8
Q

what is secondary sjogren’s

A

sjogren’s + other disease e.g. SLE, RA, SSc, PBC

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9
Q

ix sjogren’s

A

schirmer tear test

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10
Q

rx sjogren’s

A

artificial tears and saliva

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11
Q

what is CREST syndrome (NB this isn’t used anymore but it’s still useful to remember the symptoms

A

limited cutaenous form of systemic sclerosis

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12
Q

what is included in CREST syndrome (systemic sclerosis)

A
calcinosis (calcium depsosits, particularly at fingertips)
Raynaud's
Esophageal dysmotility -> GORD
sclerodactyly
telangectasia
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13
Q

what 4 diseases are included in connective tissue disorders

A

SLE
Sjogren’s
systemic sclerosis
polymyositis/dermatomyositis

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14
Q

what is systemic sclerosis (aka scleroderma)

A

autoimmune connective tissue disease characterised by thinking of the skin due to accumulation of collagen

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15
Q

rx raynaud’s

A

nifedipine
IV illoprost
sidenafil
bosentran

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16
Q

rx pulmonary HTN

A

bosentran
sidenafil
illoprost

17
Q

SEs CCBs

A

flushing
headache
ankle oedema

18
Q

clinical features of skin in dermatomyositis

A

purple rash on eyelids and fingers
Gottron’s papules over knees
rash on dorsum of hands and feet
puffy around eyes

19
Q

what later symptoms may occur in polymyositis

A

dysphagia/aspiration - oropharyngeal muscle involvement

resp failure if resp muscles involved

20
Q

what do patients get on fingers in systemic sclerosis due to raynaud’s

A

digital ulcers

21
Q

what needs to be monitored in SSc

A

pulmonary function -> fibrosis
echo: pulmonary HTN
NB 50% chance of death if have pulmonary HTN

22
Q

subtypes of systemic sclerosis

A

limited cutaenous form: skin only affected on face and distally on limbs. slower progression
diffuse cutaneous form: skin affected everywhere. faster progression at the start, then comes down

23
Q

what is given to treat raynaud’s if pt has digital ulcers

A

illoprost

24
Q

what mouth sign is seen in systemic sclerosis

A

microstomia (can’t open mouth properly because skin is tight)

25
Q

what hand sign is seen in SSc

A

sclerodactyly

26
Q

what are the differential diagnoses of polymyositis

A

post viral
SE statin/steroid
low vit D/Ca

could be PMR but this doesn’t usually present with weakness

27
Q

what needs to be surveilled in polymyositis

A

breathing
swallowing
(in case these muscles become weak)

28
Q

difference in raynaud’s hx between limited and diffuse cutaneous SSc

A

limited has long history of raynaud’s

diffuse has short history

29
Q

which out of limited and diffuse cutaneous SSc has:

a) pulmonary HTN
b) renal crisis
c) cardiac involvement
d) malignancy

A

a) limited
b) diffuse
c) diffuse
d) diffuse anti-RNA polymerase 3 antibodies - remember essay!!!

30
Q

what stomach thing might be seen in systemic sclerosis

A

watermelon stomach

Gastric Antral Vascular Ectasia (GAVE) - blood vessels in the lining of the stomach become fragile and become prone to rupture and bleeding

31
Q

associated lung disease in a) sle, b) ssc, c) RA

A

sle -> pleurisy and effusions

ssc and RA -> interstitial lung disease

32
Q

which two rheumatological conditions have photosensitivity

A

SLE

dermatomyositis