Sjrogens, SLE, APS

Question 1

Symptoms of sjrogens

Answer 1

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

Question 2

Typesof sjrogens

Answer 2

primary, seconndary

Question 3

Who does sjrogens affect

Answer 3

0.1-4% of population
Female 9:1 male
40s and 50s

Question 4

What complications are people with sjrogens syndrome monitored for

Answer 4

non hodgkins lymphoma
MALT (mucosal ass lymphoid tissue) lymphoma - 20x more likley

Question 5

What antibodies is sjrogens ass with

Answer 5

ANA
RF
ENA - Ro, La

Question 6

What is sjrognes syndrome

Answer 6

Chronic inflammatory and AI disorder characterised by diminished lacrimal and salivary gland secretion - sicca complex

Question 7

Diagnosis of sjrogens

Answer 7

ANA, anti-60kD (SS-A), Ro + anti-La antibodies
Decreased slaiva and tear production
Lymphocytic infiltration in labial salivary gland biopsy

Question 8

What other AI diseases can be ass w sjrogens making it seconary

Answer 8

Systemic sclerosis, rheumatoid arthritis, SLE

Question 9

Schirmers test

Answer 9

Sterlie pieve of paper put on surface of the eye for 5 minutes
Positive = <5ml (healthy = 15ml)

Question 10

What use to screen or deceolpment of lymphoma

Answer 10

Bloods - ESR, complement, immunoglobulins/electrophoresis

Question 11

Investigations for sjrogens

Answer 11

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

Question 12

Treatments for sjrogens syndrome

Answer 12

Eye drops
Artificial saliva
Importance of good dental hygeine
Hydroxycholoquine for fatigue
Screen for Hepatitis Cand HIV

Question 13

What can comes under mixed tissue disease

Answer 13

RA
SLE
Myositis
Scleroderma

Question 14

Presentation of mixed CTD

Answer 14

Raynauds, digital ulcers
Puffy hands
Fatigue
Muscle involvement
Skin
Arthritis
Interstitial LD
Pulmon. arterial HPTN

Question 15

What antibody see in Mixed CTD

Answer 15

RNP

Question 16

What look for onceconfimed AI (multiple) CTD?

Answer 16

ILD
Pulm. art. HPTN
Erosive arthritis

Question 17

What is antiphospholipid syndrome

Answer 17

aquired AI disorder RECURRENT VENOUS OR ARTERIAL THROMBOSIS and /or foetal loss
Thrombocytopenia

Question 18

What can hypercoaguability in APS affect

Answer 18

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

Question 19

What is most common disease ass with APS

Answer 19

SLE 30% have + APS antibodies

Question 20

Features of APS

Answer 20

venous/arterial thrombosis
recurrent miscarriages
livedo reticularis
other features: pre-eclampsia, pulmonary hypertension

Question 21

Antibodies in APS

Answer 21

Anticardiolipin
Anti beta2 glycoproteins abs
lupus anticoagulant

Question 22

Investigations fro APS

Answer 22

Antibodies
Thrombocytopenia
Prolonged APTT

Question 23

Management of APS

Answer 23

Primary thromboprophylaxis - low dose aspirin
2ndry thromboprophylaxis - Initial VTE/arterial thrombosis - lifelong warfarin, INR2-3
Recurrent VTE - add aspirin to warfarin, INR 3-4

Question 24

What is livedo reticularis

Answer 24

Mottled appearance on skin in APS

Question 25

What need for a diagnosis of APS

Answer 25

One clinical and one lab criteria

Question 26

Clinical criteria for APS

Answer 26

Vascualr thrombosis Pregnancy morbidity

Question 27

Lab criteria ASP

Answer 27

Elevated IgG or IgM anticardiolipin (aCL), anti beta 2 glycoprotein I lupus anticoagulant

Question 28

What does lupus anticaogulant need to be for diagnosis of APS

Answer 28

at least 2 occasions at least 12 weeks apart

Question 29

Pregnancy morbidity criteria in APS

Answer 29

>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)

Question 30

What pregnancy complications are APS patients more at risk of

Answer 30

Preeclampsia/eclampsia Preterm delivery FGR

Question 31

What pre pregnancy planning do for APS

Answer 31

Disease quiescence fora t least 6 monhts prior to conception Appropritae counselling

Question 32

What contraceptive for APS

Answer 32

COCP

Question 33

What should be monitored in pregnancy with APS

Answer 33

Disease activity - baseline and regular intervals Maternal Ro/La - foetal cardiac monitoring SLE - test for APS in early preg

Question 34

What does maternal Ro/La antibodies in APS increase the risk of in pregnancy

Answer 34

Foetal congenital Heart block If positive do foetal cardiac scanning

Question 35

Treatment of APS in pregnancy

Answer 35

Low dose aspirin therapy Assess need for therapeutic/prophylactic LMWH Flares - prompt lowest effective dose prednisolone

Question 36

What antibody is the strongest predictor for adverse outcomes in APS in pregnancy

Answer 36

LAC

Question 37

When advise against pregnancy in APS

Answer 37

Sev pulm arterial HPTN Stage 4/5 CKD

Question 38

Known teratogens DMARDs

Answer 38

Methotrexate, mycophenolate, cyclophosphamide

Question 39

What management should be given in pregnancy in SLE

Answer 39

Hydroxychloroquine Azathioprine is safe

Question 40

What drugs give in severe refractory maternal disease SLE in pregnancy

Answer 40

Pulsed IV methylprednisolone, IVIG or 2/3rd trimester cyclophosphamide if v severe

Question 41

Affect of rituximab in pregnancy

Answer 41

Not teratogenic BUT Causes neonatal B cell depletion in second or third trimesters

Question 42

What causes neonatal lupus

Answer 42

Transplacental passage of maternal antibodies Non severe, lupus like rash