Connective tissue diseases Flashcards by India Lunn

sx in systemic sclerosis

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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what is polymyositis

inflammation of PROXIMAL muscle causing muscle weakness

dermatomyositis if skin is involved

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symptoms in polymyositis

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

fever

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ix in polymyositis

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

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increased risk of what in polymyositis

malignancy - need to screen for this!!!

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rx polymyositis

prednisolone

immunosuppressant e.g. methotrexate, azathioprine

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what is sjogren’s syndrome

dry eyes and dry mouth enlarged salivary glands

also have raynauds and fatigue

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what is secondary sjogren’s

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

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ix sjogren’s

schirmer tear test

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rx sjogren’s

artificial tears and saliva

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

limited cutaenous form of systemic sclerosis

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what is included in CREST syndrome (systemic sclerosis)

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

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what 4 diseases are included in connective tissue disorders

SLE
Sjogren’s
systemic sclerosis
polymyositis/dermatomyositis

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what is systemic sclerosis (aka scleroderma)

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

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rx raynaud’s

nifedipine
IV illoprost
sidenafil
bosentran

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rx pulmonary HTN

bosentran
sidenafil
illoprost

SEs CCBs

flushing
headache
ankle oedema

clinical features of skin in dermatomyositis

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

what later symptoms may occur in polymyositis

dysphagia/aspiration - oropharyngeal muscle involvement

resp failure if resp muscles involved

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

digital ulcers

what needs to be monitored in SSc

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

subtypes of systemic sclerosis

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

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

illoprost

what mouth sign is seen in systemic sclerosis

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

what hand sign is seen in SSc

sclerodactyly

what are the differential diagnoses of polymyositis

post viral SE statin/steroid low vit D/Ca could be PMR but this doesn't usually present with weakness

what needs to be surveilled in polymyositis

breathing swallowing (in case these muscles become weak)

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

limited has long history of raynaud's | diffuse has short history

which out of limited and diffuse cutaneous SSc has: a) pulmonary HTN b) renal crisis c) cardiac involvement d) malignancy

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

what stomach thing might be seen in systemic sclerosis

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

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

sle -> pleurisy and effusions | ssc and RA -> interstitial lung disease

which two rheumatological conditions have photosensitivity

SLE | dermatomyositis