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