Sjrogens, SLE, APS Flashcards
Symptoms of sjrogens
Sicca - dry eyes (keratoco njunctivitis sicca) and mout (xerostomia)
Fatigue
Joint involvement - arthralgias and myalgias
Peripheral neuropathies
Extra glandular involvemet - resp and GI disease, renal tubular acidosis
Typesof sjrogens
primary, seconndary
Who does sjrogens affect
0.1-4% of population
Female 9:1 male
40s and 50s
What complications are people with sjrogens syndrome monitored for
non hodgkins lymphoma
MALT (mucosal ass lymphoid tissue) lymphoma - 20x more likley
What antibodies is sjrogens ass with
ANA
RF
ENA - Ro, La
What is sjrognes syndrome
Chronic inflammatory and AI disorder characterised by diminished lacrimal and salivary gland secretion - sicca complex
Diagnosis of sjrogens
ANA, anti-60kD (SS-A), Ro + anti-La antibodies
Decreased slaiva and tear production
Lymphocytic infiltration in labial salivary gland biopsy
What other AI diseases can be ass w sjrogens making it seconary
Systemic sclerosis, rheumatoid arthritis, SLE
Schirmers test
Sterlie pieve of paper put on surface of the eye for 5 minutes
Positive = <5ml (healthy = 15ml)
What use to screen or deceolpment of lymphoma
Bloods - ESR, complement, immunoglobulins/electrophoresis
Investigations for sjrogens
Antiboody tests - ANA etc
Bloods, EP
Schirmers test
Slaivary flow - spit as much saliva as can then weighed
Parotid and submandibular gland USS
Minor labial gland biopsy
Treatments for sjrogens syndrome
Eye drops
Artificial saliva
Importance of good dental hygeine
Hydroxycholoquine for fatigue
Screen for Hepatitis Cand HIV
What can comes under mixed tissue disease
RA
SLE
Myositis
Scleroderma
Presentation of mixed CTD
Raynauds, digital ulcers
Puffy hands
Fatigue
Muscle involvement
Skin
Arthritis
Interstitial LD
Pulmon. arterial HPTN
What antibody see in Mixed CTD
RNP
What look for onceconfimed AI (multiple) CTD?
ILD
Pulm. art. HPTN
Erosive arthritis
What is antiphospholipid syndrome
aquired AI disorder RECURRENT VENOUS OR ARTERIAL THROMBOSIS and /or foetal loss
Thrombocytopenia
What can hypercoaguability in APS affect
virtually any organ
Peripheral venous system - DVT, PE
CNS - stroke, seizures, chorea, sinus thrombosis
Skin - livedo reticularis, purpura, infarcts, ulceration
Eyes - Amourosis fugax, adrenal infarcts
What is most common disease ass with APS
SLE 30% have + APS antibodies
Features of APS
venous/arterial thrombosis
recurrent miscarriages
livedo reticularis
other features: pre-eclampsia, pulmonary hypertension
Antibodies in APS
Anticardiolipin
Anti beta2 glycoproteins abs
lupus anticoagulant
Investigations fro APS
Antibodies
Thrombocytopenia
Prolonged APTT
Management of APS
Primary thromboprophylaxis - low dose aspirin
2ndry thromboprophylaxis - Initial VTE/arterial thrombosis - lifelong warfarin, INR2-3
Recurrent VTE - add aspirin to warfarin, INR 3-4
What is livedo reticularis
Mottled appearance on skin in APS
What need for a diagnosis of APS
One clinical and one lab criteria
Clinical criteria for APS
Vascualr thrombosis
Pregnancy morbidity
Lab criteria ASP
Elevated IgG or IgM anticardiolipin (aCL), anti beta 2 glycoprotein I
lupus anticoagulant
What does lupus anticaogulant need to be for diagnosis of APS
at least 2 occasions at least 12 weeks apart
Pregnancy morbidity criteria in APS
> 1 late term spontaneous abortions
1 prem birth or morphologically healthy neonate at or before 34 weeks due to sev preeclampsia, eclampsia or sev placental insufficiency
.3 unexplained consecutive spontaneous abortions (<10 weeks gestation)
What pregnancy complications are APS patients more at risk of
Preeclampsia/eclampsia
Preterm delivery
FGR
What pre pregnancy planning do for APS
Disease quiescence fora t least 6 monhts prior to conception
Appropritae counselling
What contraceptive for APS
COCP
What should be monitored in pregnancy with APS
Disease activity - baseline and regular intervals
Maternal Ro/La - foetal cardiac monitoring
SLE - test for APS in early preg
What does maternal Ro/La antibodies in APS increase the risk of in pregnancy
Foetal congenital Heart block
If positive do foetal cardiac scanning
Treatment of APS in pregnancy
Low dose aspirin therapy
Assess need for therapeutic/prophylactic LMWH
Flares - prompt lowest effective dose prednisolone
What antibody is the strongest predictor for adverse outcomes in APS in pregnancy
LAC
When advise against pregnancy in APS
Sev pulm arterial HPTN
Stage 4/5 CKD
Known teratogens DMARDs
Methotrexate, mycophenolate, cyclophosphamide
What management should be given in pregnancy in SLE
Hydroxychloroquine
Azathioprine is safe
What drugs give in severe refractory maternal disease SLE in pregnancy
Pulsed IV methylprednisolone,
IVIG or 2/3rd trimester cyclophosphamide if v severe
Affect of rituximab in pregnancy
Not teratogenic BUT
Causes neonatal B cell depletion in second or third trimesters
What causes neonatal lupus
Transplacental passage of maternal antibodies
Non severe, lupus like rash
