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Rheumatology > Autoimmune connective tissue disorders > Flashcards

Autoimmune connective tissue disorders Flashcards

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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

22
Q

Anti-Smith antibodies

23
Q

Anti-RNP

Study These Flashcards
A

SLE, mixed connective tissue disease

Poor prognosis for SLE

24
Q

Anti-Jo/Anti-Mi

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A

Polymyositis, dermatomyositis

Anti -Jo –> ILD

25
cytoplasmic ANCA
PR3 +ve GPA Also some MPA, PAN
26
Perinuclear ANCA
MPO +ve MPA Churg-Strauss Goodpasture's
27
Complement in ANCA +ve vasculitis
Not consumed
28
Skin manifestations in diffuse vs limited scleroderma
**Diffuse:** Widespread **Limited:** Trunk-sparing, below elbow, face not affected as much
29
Sclerodermal renal crisis
Occurs in diffuse systemic sclerosis Acute-onset hypertension + AKI Treated w/ ACE-i May be ppt by high-dose steroids
30
Pulmonary manifestations in limited vs diffuse systemic sclerosis
**Limited:** End arteritis --\> Pulmonary HTN, R-sided heart failure, normal spirometry, reduced gas transfer **Diffuse:** Fibrosis, restrictive lung disease, may get PAH Overlapping!
31
GI manifestations in systemic sclerosis
Affects both forms Fibrosis \> reduction in peristalsis \> bacterial overgrowth +/- constipation/incontinence Gastro-oesophogeal dysmotility \> PPIs
32
Skin manifestations in advanced systemic sclerosis
Sclerodactyly (claw-like) Ulceration Shiny skin, beak-like noses, small mouths Loss of pigmentation
33
Presentation of Sjogren's
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
High risk for SLE renal manifestations
First 3 years of illness Male Non-white Anti-Sm Young
35
Common neuro manifestations of SLE
Stroke Seizures
36
Risk factors for neuro SLE
Anti-phospholipid Previous NPSLE Active disease
37
Features of SLE arthropathy
Excessive pain Jaccoud's deformity (avascular necrosis common) Migratory Short EMS PIPJs, wrists, knees
38
Secondary causes of Raynaud's
IBD SLE Sjogren's Systemic sclerosis (worse in limited) Polymyositis/dermatomyositis
39
Features of polymyositis
Insidious progressive symmetrical Proximal muscle weakness
40
Investigation of polymyositis
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
Skin signs in dermatomyositis
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
Anti-Ro and Anti-La in pregnancy
Congenital heart block
43
HLA B27 associated disorders
Ankylosing spondylitis strongest association Also Reiter's, IBD, psoriatic arthritis
44
Anticentromere Abs
Limited systemic sclerosis
45
Anti-topoisomerase 1 Abs (Anti-Scl70)
Diffuse systemic sclerosis
46
Management of Raynaud's
Ca channel blockers
47
Management of ILD in systemic sclerosis
Cyclophospamide
48
Treatment of moderate SLE
Steroids + steroid-sparing e.g. azathioprine, methotrexate
49
Treatment of severe SLE
High-dose steroids Cyclophosphamide Mycophenolate mofetil
50
SE of cyclophosphamide
Permanent infertility in men Reduced fertility in women
51
Worring feature's of Raynaud's
Late-onset Sudden increase in symptom load

Rheumatology (6 decks)

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