Connective Tissue Diseases Flashcards
name the algorithm used to name the different types of CT diseases
Samii And Sarah Patted My Sausage Dog
name the types of CT disease
sjogrens syndrome anti-phospholipid syndrome SLE polymyositis mixed CT disease systemic sclerosis dermatomyositis
are CT diseases diseases of connective tissue
no
what characterises CT diseases
all involve the presence of spontaneous over activity of the immune system usually by auto antibodies
which gender is lupus more common in
women
which ethnicity is lupus most common in
asians
afro-americans
afro-caribbeans
hispanic americans
is there a genetic element in lupus?
yes, common in identical twins
are there hormonal factors in lupus?
yes, increased oestrogen eg HRT/contraception increases risk
pathogenesis of lupus?
delayed clearance of dead cells
T cells provoke B cells to make ab’s via cytokines to them (thinks its an ag)
why should you worry about renal disease in lupus in particular?
shows no symptoms
why are the kidneys and skin infected in lupus?
antibody and antigen form an immune complex that deposits in kidney and skin; activate complement and get inflammatory reaction
clinical criteria in the classification of lupus?
cutaenous lupus ulcers arthritis serositis haemolytic anaemia leukopaenia thrombocytopaenia
immunologic criteria in the classification of lupus?
presence of: ANA Anti-DNA Antiphospholipid Ab low complement
general symptoms of lupus
fever malaise poor appetite weight loss fatigue
mucocutaneous features of lupus
photosensitivity
malar rash
mouth ulcers
alopecia
MSK features of lupus
polyarthritis/arthralgia
myopathy
jaccouds arthritis
4 P’s of serositis
pericarditis
pleurisy
pleural effusion
pericardial effusion
what do blood and protein in urine indicate?
glomerulonephritis
neurological features of lupus
depression
seizures
confusion
cranial/peripheral neuropathy
haematological features of lupus
lymphademopathy leucopenia lymphopaenia haemolytic anaemia thombocytopaenia
female patient with recurrent miscarriages and recent DVT?
anti-phospholipid syndrome
main clinical features of APS
venous and arterial thrombosis
livido reticularis
miscarriage
thrombocytopaenia
what titre is most often positive in ANA testing of patients
1:160
what conditions cause a raised ANA
RA
HIV
hep c
CT disease
when should be the only time you take anti ANA into account with CT disease
if other autoantibodies are positive and the patient has CTD features
most specific autoantibody test for lupus
anti double stranded antibody
4 forms of anti ENA antibody
anti-ro
anti-la
anti-sm
anti-rnp
which 2 neonatal issues are common when the mother is anti-ro +ve
neonatal lupus
foetal heart block
name the 3 anti phospholipid antibodies
anti-cardiolipin antibody
lupus anticoagulant
anti-beta 2 glycoprotein
what process ensures anti phospholipid antibody tests are accurate
repeat 3 months later to see if its still positive
first investigation in suspected lupus?
urinalysis
treatment for lupus?
NSAIDs
analgesia
hydroxychloroquine
steroids on a short course
best drug for lupus
hydroxychloroquine
what immunosuppressives can be used in lupus
azathioprine
methotrexate
common symptoms that span all autoimmune diseases?
arthralgia arthritis raynauds sicca (dryness) myalgia alopecia ulcers thrombosis miscarriage
when would you suspect APS?
young patient with unexplained venous and arterial events or thrombocytopaenia
treatment for APS?
lifelong anticoags
aspirin
hydroxychlorine
why does APS cause miscarriages?
clotsblood vessels to placenta so baby doesnt get nutrients or blood
most common sex and age group for sjogrens
females in 50s and 60s
clinical presentation of sjogrens
dry eyes, mouth due to diminished lacrimal and salivary gland function
gland enlargement
joint pain
fatigue
treatment for sjogrens
tear supplements eye drops saliva supplements hydrocychloroquine pilocarpine methotrexate
what would you use to anticoagulate a pregnant woman?
aspirin
fragmin
why are sjogrens patients more likely to get lymphoma
rapidly multiplying B cells
2 main effects of systemic sclerosis
vascular endothelial changes
fibrosis
most common secondary disease affecting lungs in diffuse cutaneous systemic sclerosis
pulmonary fibrosis
which parts of the skin are involved in diffuse cutaneous systemic sclerosis
torso
proximal to forearms
areas of skin affected in systemic sclerosis
distal to elbows only
name the CREST symptoms that arise in systemic sclerosis
calcinosis raynauds esophageal dysmotility sclerodactyly telangiectasia
what is sclerodactyly
localized thickening and tightness of the skin of the fingers or toes
what is calcinosis
deposition of calcium in the skin, subcutaneous tissue, muscles and visceral organs
common lung complication in systemic sclerosis
pulmonary hypertension
what would treat the raynauds element of SS?
ca channel blockers
phosphodiesterase inhibitors
what would treat the lung complications of SS?
immunosuppression
what would treat the pulmonary hypertension complication of SS?
calcium channel blocker
endothelin receptor antagonists
oxygen
what would treat the reflux complication of SS
PPIs
H2 receptor antagonists
which auto antibody does mixed CT disease test positive to?
anti RNP
what 3 diseases does mixed CT disease encompass
systemic sclerosis
lupus
polymyositis
autoantibodies present in lupus
ana
anti-dna binding
anti-sm
anti-ro
autoantibodies in systemic sclerosis
anti-centromere
anti-scl-70
autoantibodies in polymyositis?
anti-jo-1
