Connective Tissue Diseases Flashcards
are all connective tissue diseases autoimmune
yes
gender more affected by lupus and why
women since increased estrogen
race more affected by lupus
asian
virus linked to lupus
epstein barr
most common age of lupus presentation
20 - 30
what type hypersensitivity is lupus
iii
systemic symptoms in lupus
malaise, weight loss, fatigue, fever
dermatological symptoms of lupus
malar butterfly rash, photosensitivity, non-scarring alopecia, discoid lupus
cardiovascular symptoms of lupus
> PE and MI risk
serositis due to lupus can present as what
pericarditis, pleurisy, pleural effusion
rheumatological symptoms of lupus
synovitis, polyarthritis
what is the distribution of arthritis in lupus
same as RA
1st investigation for lupus
urinalysis
imaging for lupus
CXR, CT chest, MRI brain
blood tests for lupus
FBC, U&E, complement, autoantibodies
what autoantibodies are found in lupus
ANA anti-dsDNA anti-Sm antiphospholipid RNP anti-Ro
which autoantibody is specific to lupus
anti-Sm (however low sensitivity)
medications for all lupus patients
hydroxychloroquine + analgesia
side effect of hydroxychloroquine
retinopathy
management of skin disease in lupus
TOP CCS
management of arthritis in lupus
PO CCS, azathioprine / methotrexate
management of severe lupus (kidney or CNS involvement)
IV CCS, cyclophosphamide
last line management of lupus
IV immunoglobulin, rituximab
what is used to monitor lupus that positively correlates with disease activity
anti-dsDNA
what is used to monitor lupus that negatively correlates with disease activity
complement C3 / C4
red flag for antiphospholipid syndrome
recurrent miscarriage
pathology of antiphospholipid syndrome
recurrent venous and arteriol thrombosis
gender thats a risk factor for antiphospholipid syndrome
women
what sign is seen on the skin in antiphospholipid syndrome
livedo reticularis
autoantibodies in antiphospholipid syndrome
anti-cardiolipid Ab
lupus anticoagulant
anti-beta 2 glycoprotein
management of antiphospholipid syndrome
lifelong anticoagulant warfarin
conditions associated with sjogren’s syndrome
RA, SLE, Raynaud’s
pathology of sjogren’s syndrome
lymphocytic infiltration of exocrine glands
ages that are risk factors for sjogren’s syndrome
40 - 60
gender thats a risk factor for sjogren’s syndrome
female
general presentation of sjogren’s syndrome
arthralgia, dry mouth/eye/skin/vagina (sicca)
which specific organs can sometimes be affected by sjogren’s syndrome
lung, kidney, liver
investigations for sjogren’s syndrome
schirmer’s test, autoantibodies, salivary gland biopsy
what does schirmer’s test measure
tear production
what autoantibodies are found in sjogren’s syndrome
anti-ro
anti-la
ANA
what is the name of the limited (cutaneous) form of systemic sclerosis
CREST syndrome
what does CREST stand for in CREST syndrome
calcinosis raynaud's eosphageal dysmotility schlerodactyly telangiectasia
what autoantibodies are present in the limited form of systemic slerosis (CREST syndrome)
anti-centromere
ANA
what gender is more commonly affected by systemic slerosis
women
what ages are risk factors for systemic sclerosis
middle age
how does the presentation of the limited and diffuse form of systemic sclerosis differ
limited: only cutaneous in peripheral limbs.
diffuse: cutaneous over whole body, rapid onset, can affect lung, heart, kidney, pancreas
what autoantibodies are found in diffuse type systemic sclerosis
anti-Scl-70
ANA
maangement of raynaud’s disease
CCB
management of renal disease in diffuse systemic sclerosis
ACEI
management of pulmonary disease in diffuse systemic sclerosis
cyclophosphamide, prednisolone
what is mixed connective tissue disease
mixed features of systemic sclerosis, lupus and polymyositis
autoantibodies in mixed connective tissue disease
anti-U1-RNP
ANA
managment of sjogrens syndrome
eyedrop, saliva replacement, hydroxychloroquine, pilocarpine
drug for sjogren’s syndrome if organ involvement
DMARD
