Connective Tissue Disease Flashcards Preview

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Flashcards in Connective Tissue Disease Deck (45):
1

Constitutional Symptoms of SLE

Fever
Malaise
Poor appetite
Weight loss
Fatigue

2

SLE Environmental Factors

Environmental factors:
Viruses eg Epstein-Barr Virus
UV light may stimulate skin cells to secrete cytokines stimulating B-cells
Silica dust (found in cleaning powders, cigarette smoke and cement) may increase risk of developing SLE

3

SLE Mucocutaneous Features

Photosensitivity
Malar rash
may or may not be associated with sun exposure
Discoid lupus erythematosus
Subacute cutaneous lupus

4

SLE Musculoskeletal Features

Non-deforming polyarthritis/polyarthralgia
RA distribution but no radiological erosion
Deforming arthropathy - Jaccoud’s arthritis
Erosive arthritis - rare
Myopathy - weakness, myalgia & myositis

5

Swan neck deformities

These result from recurrent synovitis and inflammation of the joint capsule, tendons and ligaments

6

SLE Pulmonary Features

Pleurisy
Infections
Diffuse lung infiltration and fibrosis
Pulmonary hypertension
Pulmonary infarct

7

SLE cardiac features

Pericarditis
Cardiomyopathy
Pulmonary hypertension
Libman Sach endocarditis (non-bacterial endocarditis)

8

SLE Glomerulonephritis Presentation

Proteinuria
Urine sediments
Urine RBC and casts
Hypertension
Acute renal failure
Chronic renal failure

9

What are casts?

Cylindrical structures produced by the kidneys, present in some disease states

10

SLE Neurological Features

Depression/psychosis
Not always related to disease activity
Migranous headache
Cerebral ischaemia
TIAs or stroke
Cranial or peripheral neuropathy
Cerebellar ataxia

11

SLE Haematological Features

Lymphadenopathy
~25% of all patients during their course of illness
Leucopenia (low white cells)
Anaemia (haemolytic, normochromic normocytic)
Thrombocytopenia (low platelets)

12

Screening Tests for Suspected SLE

Full blood count
Renal function tests including urine examination
Anti-nuclear antibody
Anti-double stranded DNA antibodies
ENA
Complement levels

13

Conditions that ANA is found in

SLE, RA, hepatitis C, HIV, other autoimmune conditions

14

Anti-SM and what it is likely to be involved with?

SLE
Neurological association

15

Anti-La

Sjogrens

16

Anti-Ro

Sjogrens

17

Anti-Scl70

Scleroderma

18

Anti-RNP

Mixed Connective Tissue disease
Overlap features – sclerodermatous skin lesions, Raynaud’s phenomenon, low grade myositis

19

In SLE, what titre correlates with overall disease activity?

Anti-double stranded DNA antibody
May be associated with lupus nephritis

20

What antibody may be associated with lupus nephritis in SLE?

Anti-double stranded DNA antibody

21

In SLE, what is anti-Ro associated with?

Cutaneous manifestations

22

What could happen with anti-Ro and a foetus?

Neonatal heart block, neonatal LE

23

Other investigations for SLE

Depend on symptoms
CXR
Pulmonary function tests
CT chest
Urine protein quantification
Renal biopsy
Echocardiogram
Nerve conduction studies
MRI brain

24

What could you measure that negatively correlated with SLE activity?

Complement C3/C4
(anti-double stranded DNA antibody correlates WITH activity)

25

Monitoring SLE activity

Thorough clinical assessment including BP
Anti-dsDNA level positively correlates with activity
C3/C4 levels negatively correlate with activity
Urine examination including protein, cells and casts
Full blood count
Blood biochemistry

26

SLE general management

Counselling - patients, spouse and relatives
Regular monitoring
Avoid excessive sun-exposure
Pregnancy issues

27

SLE drug treatment

NSAID and simple analgesia
Anti-malarials – chloroquine and hydroxychloroquine
Useful for arthritis, cutaneous manifestations and constitutional symptoms
May reduce systemic complications

28

SLE - Biologics

Anti-CD20 (Rituximab)
Anti-Blys (Belimumab)

29

SLE - Immunosuppressives

Azathioprine
Cyclophosphamide
Methotrexate
Mycophenolate mofetil
(All can cause bone marrow suppression
All can cause increased susceptibility to infection
Potentially teratogenic)

30

What do SLE patients die of?

Infection important cause of death in the early / medium stage of disease
Cardiovascular disease the major the cause of death in the late stages

31

What is often the first sign of systemic sclerosis?

Raynauds

32

Treatment for sjogrens syndrome

-eye drops/ punctal plugs
-Pilocarpine
-artificial saliva
-steroids & immunosuppression
-hydrochloroquinine
-ATTENTION TO CARDIOVASCULAR RISK FACTORS

33

Name some CVS risks associated with sjogrens

Heart attack
Stroke
Hypertryglyceridemia
Hypertension

(this is because of acute inflammatory state in sjogrens)

34

The older name for limited sceloderma

The older term for limited scleroderma is CREST syndrome (= Calcinosis, Raynaud's disease, (O)Esophageal dysmotility, Sclerodactyly, Telangiectasia).
PULMONARY HYPERTENSION IN 30%

35

Which sjogrens antibody is associated with neonatal complete heart block?

Anti-Ro

36

Which antibodies are associated with limited scleroderma?

Anti-centromere

37

Which antibodies are associated with diffuse scleroderma?

Anti-Scl-70

38

How quickly do skin changes occur in diffuse systemic sclerosis?

Within one year of raynauds

39

Which organs are commonly involved in diffuse systemic sclerosis?

Kidneys, lungs, gut, muscle, joints, heart

40

What is juvenile sclerosis?

When patient under 16 presents with 1 major and at leas two of the 20 minor criteria of systemic sclerosis

41

Respiratory organ involvement in systemic sclerosis

ILD
Pumonary hypertension
CHEST WALL RESTRICTION

42

GI organ involvement in systemic sclerosis

Oesophageal hypomobility
Small bowel hypomobility, bacterial overgrowth
Pancreatic insufficiency
Rectal hypomobility

43

Name a medication used to treat pulmonary arterial hypertension

Bosentan, sildenifil

44

Treatment of systemic sclerosis

Calcium channel blockers
Prostacyclin (Iloprost)
ACE inhibitors
Prednisolone
Immunosuppression
Bosentan, Sildenafil

45

Trigeminal neuralgia

Extreme severe facial pain that tends to come and go in sudden shock like attacks
-like an electric shock