connective tissue autoimmune disease

Question 1

who normally gets SLE

Answer 1

white women pre menopause

Question 2

what exacerbates lupus

Answer 2

smoking, dust, UV light

Question 3

what is the pathogenesis of lupus

Answer 3

loss of immune regulation, apoptosis, dead cells release materials seen as auto-antigens, auto-antibodies formed, necrosis and scarring

Question 4

what systems does SLE commonly affect (7)

Answer 4

cutaneous, arthritis, neurological, blood, lungs, heart, kidneys

Question 5

what is a distinctive cutaneous symptom of lupus

Answer 5

butterfly malar rash on face

Question 6

what are cutaneous SLE symptoms (5)

Answer 6

malar rash, discoid rash, non-scarring alopecia, oral ulcers, photosensitive

Question 7

what symptoms of arthritis are common in lupus

Answer 7

synovitis/ tenderness in 2+ joints with >30mins morning stiffness

Question 8

what neurological symptoms are common in SLE

Answer 8

delirium, psychosis, seizure, headaches

Question 9

what are common heart/ lung symptoms of SLE

Answer 9

pleural/ pericardial effusion, pericarditis, Interstitial lung disease

Question 10

what are common haemotological symptoms of SLE

Answer 10

leukopenia, thrombocytopenia, haemolytic anaemia

Question 11

what renal symptoms are seen in SLE

Answer 11

proteinurea, blood in urine, nephritis

Question 12

what autoantibodies are seen in SLE

Answer 12

Anti DsNA, ANA (anti SM)

Question 13

how do you diagnose SLE

Answer 13

1/11 SLICC criteria - at leasr one lab

Question 14

what treatment is given to all lupus patients

Answer 14

sun protection, hydroxychloroquine, steroids, NSAIDs

Question 15

what treatment is given to severe organ involvement lupus patients

Answer 15

cyclophosphamide, IV steroids

Question 16

what treatment is given to ALP positive lupus patients

Answer 16

antiplatelets, anticoag

Question 17

what is sjorgen’s disease

Answer 17

autoimmune causing lack of secretions in eyes (lacrimal), mouth (salivary) and vagina

Question 18

what is the pathogenesis of sjorgen’s and an associated risk

Answer 18

lymphocytic infiltration of exocrine organs - lymphoma

Question 19

what are the symptoms of sjorgen’s (5)

Answer 19

dry/ gritty eyes (keratoconjunctivitis sicca), dry mouth/ vagina, bilateral parotid swelling, fatigue, dental deterioration

Question 20

what test is done for Sjorgen’s dry eyes

Answer 20

Schirmer’s test

Question 21

what auto-antibodies are present in sjorgen’s

Answer 21

Anti - ro, Anti - La (ANA)

Question 22

what immunoglobin can be found in sjorgens

Answer 22

IgG

Question 23

how do you treat sjorgen’s (mild –> severe)

Answer 23

pilocarpine (saliva replacement), hydroxychloroquine, cyclophosamide

Question 24

what is systemic sclerosis/ scleroderma

Answer 24

multi-systemic autoimmune disease where immune system becomes over active

Question 25

what causes scarring in systemic sclerosis

Answer 25

too much collagen

Question 26

what is limited cutaneous involvement of systemic sclerosis

Answer 26

extremities below elbows and knees +/- face

Question 27

what is diffuse cutaneous involvement of systemic sclerosis

Answer 27

extremities above AND below elbows and knees AND face AND trunk

Question 28

what is the main cause of death in systemic sclerosis

Answer 28

pulmonary hypertension

Question 29

what are the symptoms of systemic sclerosis (8)

Answer 29

CREST: calcinosis (calcium deposits), reynauds, oesophageal dysfunction, sclerodacytlyly, telangiectasia. digital ischaemia, GORD, SOB

Question 30

what is Reynaud's

Answer 30

blanching/ whitness of digits and acrocyanosis - sensitive to cold

Question 31

how do you manage reynaud's

Answer 31

CCB's, PDE5 inhibitor

Question 32

what face changes can be seen in Reynauds

Answer 32

small puckered mouth, beaked nose, skin tightening, telangiectasia

Question 33

what GI symptoms can be seen in Reynauds

Answer 33

calcinosis, small intestinal bacterial overgrowth, malabsorption, incontinence

Question 34

what cardio/ resp symptoms can be seen in Reynauds

Answer 34

interstitial lung disease eg FIBROSIS, pulm hypertension, myocardial disease

Question 35

what is used to treat scleroderma renal crisis

Answer 35

ACEi

Question 36

what auto-antibodies can be seen in systemic sclerosis

Answer 36

Anti-scl-70, ANA , anticentromere

Question 37

what imaging can be done i systemic sclerosis

Answer 37

C Xray, echo

Question 38

what treatments are used for systemic sclerosis

Answer 38

CCB's, ACEi (if renal), cyclophosphamide (no steroids)

Question 39

what is anti-phospholipid syndrome often secondary to

Answer 39

lupus

Question 40

what are the symptoms of anti-phospholipid syndrome

Answer 40

recurrent venous/ arterial thrombosis, recurrent miscarriages, superficial thrombophelbitis, migraines, livedo reticularis

Question 41

what are the increased risks with anti-phospholipid syndrome

Answer 41

stroke, MI, PE

Question 42

how do you diagnose anti-phospholipid syndrome

Answer 42

+ive on 2 occasions 12 weeks apart: anti-cardiolipin, lupus anti-coag test, anti-B2 glycoprotein