Connective Tissue Disease Flashcards

(36 cards)

1
Q

Who most commonly gets SLE?

A

Women (90%)
Usually presents age 20-30
More common and more severe in Afro-Caribbean, Hispanic American, Asian and Chinese

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2
Q

Which organs are most commonly affected by SLE?

A
Skin
Joints
Kidneys
Blood cells
Nervous system
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3
Q

What are the constitutional features of SLE?

A

fever
fatigue
weight loss

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4
Q

What are the musculoskeletal features of SLE?

A

Arthralgia, myalgia
Arthritis - synovitis/tenderness of at least 2 joints with early morning stiffness > 30 mins, non erosive (Jaccoud arthropathy)

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5
Q

What are the mucocutaneous features of SLE?

A
Malar/butterfly rash
Photosensitivity
Discoid lupus
Subacute cutaneous lupus
Oral/nasal ulceration 
Raynaud's phenomenon
Alopecia
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6
Q

What are the renal features of SLE and how are they diagnosed?

A

Lupus nephritis

  • proteinuria > 0.5g in 24 hours
  • red cell casts on renal biopsy
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7
Q

Which cardiorespiratory features may be seen in SLE?

A

Pleural or pericardial effusion

Acute pericarditis

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8
Q

Which haematological features are seen in SLE?

A

Leukopenia
Thrombocytopenia
Haemolytic anaemia
Lymphadenopathy

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9
Q

Which neuropsychiatric features are seen in SLE?

A
Delirium
Psychosis
Seizures
Headache
Cranial nerve disorders
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10
Q

Which investigations should be done for suspected SLE?

A
FBC (anaemia, leukopenia, thrombocytopenia)
Autoantibodies
C3 + C4
Urinalysis
Imaging for specific organ involvement
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11
Q

Which autoantibodies may be positive in SLE?

A
ANA
Anti-dsDNA
Anti-Sm
Anti-Ro
Anti-La
Anti-RNP
Antiphospholipid antibodies
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12
Q

Which autoantibody is most specific for SLE?

A

Anti-dsDNA (but varies with disease activity)

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13
Q

How is complement affected in SLE?

A

C3 and C4 are low when disease is active, especially in renal and haematological disease

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14
Q

What is the first line drug management for SLE?

A

Hydroxychloroquine + topical steroids + NSAIDs

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15
Q

Which score is used to monitor disease activity in SLE?

A

SLEDAI score

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16
Q

What monitoring should be done in a patient with SLE?

A

Monitor anti-dsDNA + complement as they vary with disease activity
Urinalysis for nephritis
Evaluate/manage CVD risks as this is major cause of mortality

17
Q

What are the clinical features of sjogren’s syndrome?

A
Dry eyes and mouth (sicca symptoms)
Gritty feeling in eyes
Vaginal dryness
Bilateral parotid enlargement
Joint pain
Fatigue
Unexplained increase in dental caries
18
Q

Risk of which cancer is increased in sjogren’s syndrome?

19
Q

How is Sjogren’s syndrome diagnosed?

A

Clinical features
Schirmer’s test –> confirm ocular dryness
+ve anti-Ro and anti-La

20
Q

What is the management for Sjogren’s syndrome?

A

Symptomatic:
- eyedrops + saliva replacement
- pilocarpine to stimulate saliva production (causes flushing)
Dental care
Hydroxychloroquine may help with arthralgia and fatigue

21
Q

Which antibodies are associated with mixed connective tissue disease?

A

Anti-RNP antibodies

22
Q

What are the clinical features of anti-phospholipid syndrome?

A
Recurrent venous or arterial thrombosis +/- foetal loss 
Stroke/MI in young patients
Libman Sacks (sterile) endocarditis
Recurrent PE --> severe pulmonary HTN
Migraine
Livedo reticularis
23
Q

What is Livedo reticularis?

A

Mottled, lace-like discolouration on skin

24
Q

Which investigations should be done for suspected anti-phospholipid syndrome?

A
Bloods: thrombocytopenia + prolonged APTT
Anti-phospholipid antibodies:
- lupus anticoagulant
- anti-cardiolipin
- anti-beta 2 glycoprotein
25
What is the management for anti-phospholipid syndrome?
Anticoagulation (do not need to treat if +ve antibodies but no episode of thrombosis) If recurrent miscarriage --> LMWH during pregnancy (warfarin is teratogenic)
26
What is another name for systemic sclerosis?
Scleroderma
27
What are the classic symptoms in systemic sclerosis?
``` Raynaud's (almost always) Skin thickening and tightening Dysphagia GORD SOB Telangiectasis Calcinosis (SC calcium deposits in fingers) Sclerodactyl ```
28
How is systemic sclerosis classified?
Diffuse cutaneous SSc: - skin involvement on extremities above AND below elbows and knees (+ face + trunk) Limited cutaneous SSc: - skin involvement only below elbows + knees on extremities (+ face)
29
Which antibodies are associated with diffuse systemic sclerosis?
Anti-Scl-70
30
Which antibodies are associated with limited systemic sclerosis?
Anti-centromere
31
Which facial features are seen in systemic sclerosis?
Small puckered mouth Beaked nose Lack of wrinkles Telangectasia
32
Apart from skin, which other clinical features may be seen in systemic sclerosis?
Pulmonary HTN, pulmonary fibrosis + accelerated HTN --> renal crisis Dysphagia, malabsorption + bacterial overgrowth of small bowel Inflammatory arthritis and myositis
33
How is Raynaud's/digital ulcers managed in systemic sclerosis?
CCBs --> nifedipine first line | Others: fluoxetine, ARBs, nitrates, iloprost, bosentan
34
How is a renal crisis treated in systemic sclerosis?
ACE inhibitors | May need dialysis
35
How is pulmonary hypertension treated in systemic sclerosis?
PDE5 inhibitor e.g. Sildenafil Bosentan Oxygen
36
How is interstitial lung disease managed in systemic sclerosis?
Mycophenolate mofetil or cyclophosphamide