connective tissue disease Flashcards
what type of hypersensitivity reaction is systemic lupus erythematous?
type 3 HS
how many symptoms do you need for a diagnosis of SLE?
at least 4
what are the constitutional symptoms of SLE?
fatigue weight loss malaise fever poor appetite
what are the skin symptoms of SLE?
malar rasht hat spares the nasolabial folds (butterfly rash) photosenstivity alopecia scaly scarring discoid rash
what symptom of the mouth is common is SLE?
mouth ulcers
what sort of joint pain is common in SLE?
non erosive small joint arthritis similar to RA
Jaccouds arthritis - non erosive arthritis associated with ulnar deviation
what chest symptoms are in SLE?
pericarditis
pleural effusion/pleuritic chest pain/SOB
what affect can SLE have on your kindeys?
glomerulonephritis?
what haematological conditions can be caused by SLE?
lymphadenopathy
leukopenia
anaemia
thrombocytopaenia
what is Libman Sack in SLE?
a type of non bacterial sterile endocarditis
what is a symptom of the hands associated with SLE?
raynauds
what is the 1st screening test done for SLE? what should follow if positive?
urine analysis
renal biopsy
what immunology is associated with SLE?
anti - ANA (non specific)
anti - DsDNA
what is anti-DsDNA associated with?
it increases with disease activity
what would you expect C3/4 to do with active disease?
decrease
complement used up
is CRP used in SLE?
no
does not increase with flare ups (only raised during infection/co-exciting disease e.g. RA)
what is mild SLE?
skin disease and arthralgia
what is moderate SLE?
inflammatory arthritis and organ involvement
what is severe SLE?
organ disease
what is the treatment for mild SLE?
hydroxycloroquine
NSAIDs
topical steroids
what is the treatment for moderate SLE?
methotrexate
azathioprine
oral steroids
what is the treatment for severe SLE?
IV steroids
cyclophosphamide
rituximab
what else should be done to manage SLE?
avoid sun exposure (photosensitivity)
how does drug induced SLE present?
feature of lupus without renal or neurological involvement
- arthralgia, myalgia, malaria skin rash, ANA +ve
what drug commonly causes SLE?
hydralazine
stops on stopping the drug
what can cause anti-phospholipid syndrome?
primary
secondary to lupus
what symptoms are associated with anti-phospholipid syndrome?
venous/arterial thrombosis (DVT)
recurrent miscarriage
thrombocytopenia
what skin symptom is seen in APS?
livedo reticularis
mottled skin covered with red/purple patched in a web pattern
how is a diagnosis of APS made?
1 lab and 1 clinical finding
what are the clinical findings needed to make a diagnosis of APS?
aterial/venous thrombosis
pregnancy loss with no other explanation from 10 weeks onwards
3 pregnancy losses before 10 weeks
pregnancy loss due to pre-eclampsia/placental insufficiency
what are the lab findings needed to make a diagnosis of APS?
2 positive findings, 12 weeks apart anti-cardiolipin antibody lupus anti-coagulation test anti-b2 glycoprotein causes low platelets and prolonged APTT
what is the treatment for APS after a primary thrombosis?
low dose aspirin
what is the treatment for APS after a secondary thrombosis?
after first VTW, life long warfarin
INR 2-3
if it happens on warfarin then low dose aspirin too
what is the treatment for APS if pregnant?
aspirin between pregnancies
aspirin and heparin during pregnancy (warfarin teratogenic)
what does sjogrens increase your risk of?
lymphoma
increased B cell activity
what are the symptoms of Sjogrens syndrome?
DRYNESS kertoconjunctivitis sicca (dry eyes) wake up at night needing water, difficulty swallowing bread xerostomia (dry mouth) parotid swelling vaginal dryness fatigue, raynauds tooth decay
what test can be done for Sjogrens?
Schrimers test
filter paper on eye, access how wet it is
<5mm in 5 mins = +ve dry eyes
what immunology is positive of Sjogrens
anti-ro
anti-la
ANA +ve (non specific)
what is the treatment for Sjogrens?
1st line hydrocholoriquine (for fatigue and arthralgia)
pilocarpine - stimulate salivary glands
who should not use pilocarpine?
asthmatics
what should be monitored for in Sjogrens?
non Hodgkins B cell lymphoma
what is scleroderma?
term meaning skin thickening
what does 90% of sclerosis patients present with?
raynauds
what is localised scleroderma?
dermal fibrosis and inflammation without any organ involvement
like to be seen with raynauds
what is systemic sclerosis (CREST)?
skin fibrosis associated with sclerosis of viscera and proceeded by raynauds
what it the presentation of sclerosis?
lung fibrosis pulmonary hypertension hypertension dysphagia renal hypertensive crisis
what is the classification for slerosis?
diffuse (30%)
limited (70%)
what immunology and onset is seen in diffuse sclerosis?
anti-SCL 70
onset of wisespread skin slecrosis affecting trunk and entire limbs 1 year after raynauds
what immunology and onset is seen in limited sclerosis?
anti-centromere
onset of skin fibrosis limited to distal limbs year after raynauds
what are the classic features of limited sclerosis?
CREST + P calcinosis - calcium deposits in fingers raynauds esophageal dysfunction (acid reflux) sclerodactyly (thinking of skin) talagiectasia pulmonary hypertension
how is sclerosis managed?
annual ECHO and PFT (due to pulmonary hypertension)
control BP - ACE inhibitor
immunosuppression - cyclophosphamide
(steroids may cause hypertensive crisis)
what is mixed connective tissue disease?
features of SLE, systemic sclerosis and polymyositis
what immunology is seen with mixed connective tissue disease?
anti-RNP
what is 1st line drug treatment for raynauds?
calcium channel antagonist - nifedipine
