connective tissue autoimmune disease Flashcards

1
Q

who normally gets SLE

A

white women pre menopause

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what exacerbates lupus

A

smoking, dust, UV light

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is the pathogenesis of lupus

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what systems does SLE commonly affect (7)

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what is a distinctive cutaneous symptom of lupus

A

butterfly malar rash on face

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what are cutaneous SLE symptoms (5)

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what symptoms of arthritis are common in lupus

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what neurological symptoms are common in SLE

A

delirium, psychosis, seizure, headaches

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are common heart/ lung symptoms of SLE

A

pleural/ pericardial effusion, pericarditis, Interstitial lung disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are common haemotological symptoms of SLE

A

leukopenia, thrombocytopenia, haemolytic anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what renal symptoms are seen in SLE

A

proteinurea, blood in urine, nephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what autoantibodies are seen in SLE

A

Anti DsNA, ANA (anti SM)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

how do you diagnose SLE

A

1/11 SLICC criteria - at leasr one lab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what treatment is given to all lupus patients

A

sun protection, hydroxychloroquine, steroids, NSAIDs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what treatment is given to severe organ involvement lupus patients

A

cyclophosphamide, IV steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what treatment is given to ALP positive lupus patients

A

antiplatelets, anticoag

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what is sjorgen’s disease

A

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

18
Q

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

A

lymphocytic infiltration of exocrine organs - lymphoma

19
Q

what are the symptoms of sjorgen’s (5)

A

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

20
Q

what test is done for Sjorgen’s dry eyes

A

Schirmer’s test

21
Q

what auto-antibodies are present in sjorgen’s

A

Anti - ro, Anti - La (ANA)

22
Q

what immunoglobin can be found in sjorgens

A

IgG

23
Q

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

A

pilocarpine (saliva replacement), hydroxychloroquine, cyclophosamide

24
Q

what is systemic sclerosis/ scleroderma

A

multi-systemic autoimmune disease where immune system becomes over active

25
Q

what causes scarring in systemic sclerosis

A

too much collagen

26
Q

what is limited cutaneous involvement of systemic sclerosis

A

extremities below elbows and knees +/- face

27
Q

what is diffuse cutaneous involvement of systemic sclerosis

A

extremities above AND below elbows and knees AND face AND trunk

28
Q

what is the main cause of death in systemic sclerosis

A

pulmonary hypertension

29
Q

what are the symptoms of systemic sclerosis (8)

A

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

30
Q

what is Reynaud’s

A

blanching/ whitness of digits and acrocyanosis - sensitive to cold

31
Q

how do you manage reynaud’s

A

CCB’s, PDE5 inhibitor

32
Q

what face changes can be seen in Reynauds

A

small puckered mouth, beaked nose, skin tightening, telangiectasia

33
Q

what GI symptoms can be seen in Reynauds

A

calcinosis, small intestinal bacterial overgrowth, malabsorption, incontinence

34
Q

what cardio/ resp symptoms can be seen in Reynauds

A

interstitial lung disease eg FIBROSIS, pulm hypertension, myocardial disease

35
Q

what is used to treat scleroderma renal crisis

A

ACEi

36
Q

what auto-antibodies can be seen in systemic sclerosis

A

Anti-scl-70, ANA , anticentromere

37
Q

what imaging can be done i systemic sclerosis

A

C Xray, echo

38
Q

what treatments are used for systemic sclerosis

A

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

39
Q

what is anti-phospholipid syndrome often secondary to

A

lupus

40
Q

what are the symptoms of anti-phospholipid syndrome

A

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

41
Q

what are the increased risks with anti-phospholipid syndrome

A

stroke, MI, PE

42
Q

how do you diagnose anti-phospholipid syndrome

A

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