Connective tissue diseases Flashcards
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)
what is polymyositis
inflammation of PROXIMAL muscle causing muscle weakness
dermatomyositis if skin is involved
symptoms in polymyositis
progressive muscle weakness - difficult reaching overhead, climbing stairs, standing up
fever
ix in polymyositis
raised muscle enzymes ESR/CRP raised (differentiates from PMR) ANA antibody positive EMG muscle biopsy
increased risk of what in polymyositis
malignancy - need to screen for this!!!
rx polymyositis
prednisolone
immunosuppressant e.g. methotrexate, azathioprine
what is sjogren’s syndrome
dry eyes and dry mouth enlarged salivary glands
also have raynauds and fatigue
what is secondary sjogren’s
sjogren’s + other disease e.g. SLE, RA, SSc, PBC
ix sjogren’s
schirmer tear test
rx sjogren’s
artificial tears and saliva
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
what is included in CREST syndrome (systemic sclerosis)
calcinosis (calcium depsosits, particularly at fingertips) Raynaud's Esophageal dysmotility -> GORD sclerodactyly telangectasia
what 4 diseases are included in connective tissue disorders
SLE
Sjogren’s
systemic sclerosis
polymyositis/dermatomyositis
what is systemic sclerosis (aka scleroderma)
autoimmune connective tissue disease characterised by thinking of the skin due to accumulation of collagen
rx raynaud’s
nifedipine
IV illoprost
sidenafil
bosentran
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
