Autoimmune connective tissue disorders Flashcards

1
Q

Clinical criteria of SLE

A

Acute cutaneous lupus

Chronic cutaneous lupus

Oral/nasal ulcers (unexplained, roof of mouth)

Synovitis

Serositis

Proteinuria/red cell casts

Neuro features

Haemolytic anaemia

Leuco or lymphopenia

Thrombocytopenia

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2
Q

Acute cutaneous features of lupus

A

Malar rash, sparing nasolabial folds

Photosensitive rash

Bullous lupus (like TEN)

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3
Q

Chronic cutaneous features of lupus

A

Discoid rash (90% won’t have systemic involvement)

Raised erythematous patch > pigmented hyperkeratotic oedematous papules > atrophic, depressed, lesions

Profundis: dusky, deep fat inflammation

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4
Q

Serositis features in SLE

A

Lung: pleurisy, pleural effusion, pleural rub

Heart: Pericarditis, pericardial pain, pericardial effusion, pericardial rub

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5
Q

Lab investigations for lupus

A

ANA – NOT SPECIFIC

Anti-dsDNA/Anti-Smith – MOST SPECIFIC

Antiphospholpid

Low complement

+ve Coombs test

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6
Q

Pathophysiology of SLE

A

Autoimmune reaction to nuclear components > formation (and failure of clearance) of immune complexes

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7
Q

What is Libman-Sacks syndrome?

A

Non-infective endocarditis assoc w/ SLE

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8
Q

Monitoring SLE disease activity

A

Disease activity scores

FBC, U+e

CRP/ESR (CRP nomal)

Complement

Anti-dsDNA titres

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9
Q

Features of antiphospholipid syndrome

A

Coagulopathy

Livedo reticularis

Obstetric (recurrent miscarriage)

Thrombocytopenia

Anti-cardiolipin or lupus anticoagulant (prolongation of coagulation studies not corrected w/ serum c.f. haemophilia)

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10
Q

Treatment of mild SLE

A

NSAIDs, sunblock, HCQ

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11
Q

Treatment of SLE flares

A

Immunosuppression (dose + choice depends on severity)

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12
Q

SLE and pregnancy

A

Antiphospholipid/anti-ro/anti-la: Obstetric complications

Cyclophosphamide: Infertility

Mycophenolate: Craniofacial abnormalities

Use HCQ as safe!

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13
Q

Feature of mixed connective tissue disease

A

High anti-U1 RNP antibodies

SLE, systemicsclerosis, polymyositis mixed features

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14
Q

Prognosis in SLE

A

80% survival at 15 years

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15
Q

Features of limited sytemic sclerosis (aka CREST)

A

Scleroderma (face, hands, feet, spares trunk)

Telengiectasia

Esophogeal dysmotility

Raynaud’s

Subclinical pulmonary hypertension > may become life-threatening

Anti-centromere antibodies

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16
Q

Management of pulmonary hypertension in limited systemic sclerosis

A

Sildenafil, bosentan

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17
Q

Diffuse systemic sclerosis antibody tests

A

Anti-topoisomerase-1 (SCL-70) and anti-RNA polymeraase

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18
Q

Management of systemic sclerosis

A

BP, echocardiogram, spirometry monitoring

Manage symptoms

Steroids for ILD

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19
Q

Antihistone antibodies

A

Drug-induced lupus

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20
Q

Anti-Ro antibodies

A

Sjogrens

Systemic sclerosis

SLE

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21
Q

Anti-La antibodies

A

Sjogrens

SLE

22
Q

Anti-Smith antibodies

A

SLE

23
Q

Anti-RNP

A

SLE, mixed connective tissue disease

Poor prognosis for SLE

24
Q

Anti-Jo/Anti-Mi

A

Polymyositis, dermatomyositis

Anti -Jo –> ILD

25
Q

cytoplasmic ANCA

A

PR3 +ve

GPA

Also some MPA, PAN

26
Q

Perinuclear ANCA

A

MPO +ve

MPA

Churg-Strauss

Goodpasture’s

27
Q

Complement in ANCA +ve vasculitis

A

Not consumed

28
Q

Skin manifestations in diffuse vs limited scleroderma

A

Diffuse: Widespread

Limited: Trunk-sparing, below elbow, face not affected as much

29
Q

Sclerodermal renal crisis

A

Occurs in diffuse systemic sclerosis

Acute-onset hypertension + AKI

Treated w/ ACE-i

May be ppt by high-dose steroids

30
Q

Pulmonary manifestations in limited vs diffuse systemic sclerosis

A

Limited: End arteritis –> Pulmonary HTN, R-sided heart failure, normal spirometry, reduced gas transfer

Diffuse: Fibrosis, restrictive lung disease, may get PAH

Overlapping!

31
Q

GI manifestations in systemic sclerosis

A

Affects both forms

Fibrosis > reduction in peristalsis > bacterial overgrowth +/- constipation/incontinence

Gastro-oesophogeal dysmotility > PPIs

32
Q

Skin manifestations in advanced systemic sclerosis

A

Sclerodactyly (claw-like)

Ulceration

Shiny skin, beak-like noses, small mouths

Loss of pigmentation

33
Q

Presentation of Sjogren’s

A

Primary (4th-5th decade) or secondary to e.g. SLE, SS

Exocrine gland infiltration/fibrosis:

Dry eyes (Schirmer’s)

Dry mouth

Systemic:

Polyarthritis

Myositis

Raynaud’s, vasculitis, etc…

34
Q

High risk for SLE renal manifestations

A

First 3 years of illness

Male

Non-white

Anti-Sm

Young

35
Q

Common neuro manifestations of SLE

A

Stroke

Seizures

36
Q

Risk factors for neuro SLE

A

Anti-phospholipid

Previous NPSLE

Active disease

37
Q

Features of SLE arthropathy

A

Excessive pain

Jaccoud’s deformity (avascular necrosis common)

Migratory

Short EMS

PIPJs, wrists, knees

38
Q

Secondary causes of Raynaud’s

A

IBD

SLE

Sjogren’s

Systemic sclerosis (worse in limited)

Polymyositis/dermatomyositis

39
Q

Features of polymyositis

A

Insidious

progressive

symmetrical

Proximal

muscle weakness

40
Q

Investigation of polymyositis

A

Screen for cancer (esp lung, pancreas, ovary, bowel) - CT CAP

Muscle enzyems (esp CK, LDH) raised

Fibrillation on EMG

ANA +ve

Anti-M2/Anti-Jo1

41
Q

Skin signs in dermatomyositis

A

Heliotrope (purple) rash on eyelids w/ oedema

Macular rash (shawl sign over back and soulders)

Gottron’s papules: rough, red, over knuckles/elbows/knees

42
Q

Anti-Ro and Anti-La in pregnancy

A

Congenital heart block

43
Q

HLA B27 associated disorders

A

Ankylosing spondylitis strongest association

Also Reiter’s, IBD, psoriatic arthritis

44
Q

Anticentromere Abs

A

Limited systemic sclerosis

45
Q

Anti-topoisomerase 1 Abs (Anti-Scl70)

A

Diffuse systemic sclerosis

46
Q

Management of Raynaud’s

A

Ca channel blockers

47
Q

Management of ILD in systemic sclerosis

A

Cyclophospamide

48
Q

Treatment of moderate SLE

A

Steroids + steroid-sparing e.g. azathioprine, methotrexate

49
Q

Treatment of severe SLE

A

High-dose steroids

Cyclophosphamide

Mycophenolate mofetil

50
Q

SE of cyclophosphamide

A

Permanent infertility in men

Reduced fertility in women

51
Q

Worring feature’s of Raynaud’s

A

Late-onset

Sudden increase in symptom load