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Rheum > Autoimmune connective tissue disease > Flashcards

Autoimmune connective tissue disease Flashcards

1
Q

Scleroderma presentation

A 
Calcinosis
Reynauds
oEsophageal dysmotility
Sclerodactyly
Telangectasia
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2
Q

Limited scleroderma Abs

A

anti-centromere in 70-80%

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3
Q

Diffuse scleroderma Abs

A

anti-topoisomerase-I (scl-70) in 40%

anti-RNA polymerase III in 20% (renal crisis)

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4
Q

Scleroderma complications

A

Pulmonary hypertension
Lung/cardiac fibrosis
Renal failure (Rheum emergency)

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5
Q

Scleroderma management

A

Iloprost/sildenafil for vascular problems
Immunosuppression e.g. IV cyclophosphamide for organ involvement

ACE inhibitors/ARBs reduce risk of renal crisis
Monitor BP and renal function regularly

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6
Q

What is relapsing polychondritis and treatment

A

Recurrent episodes of cartilage inflammation and destruction, affects ears, nasal septum, larynx and joints
Treated with steroids/DMARDs/airway management e.g. tracheostomy

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7
Q

What is polymyositis

A

Progressive symmetrical proximal muscle weakness + autoimmune-mediated striated muscle inflammation, can be from neoplastic causes

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8
Q

What is dermatomyositis

A

Myositis with skin signs

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9
Q

Dermatomyositis skin signs

A

Macular rash (shawl sign)
Heliotrope rash on eyelids with oedema
Gottron’s papules (rough red papules over knuckles)

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10
Q

Myositis extramuscular signs

A 
Fever
Arthralgia
Raynaud's
Interstitial lung fibrosis
Myocarditis/arrythmias
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11
Q

Dermato/polymyositis investigations

A

Muscle enzymes increased in plasma
EMG shows fibrillation potentials
Muscle biopsy confirms diagnosis
MRI shows muscle oedema in acute myositis

Auto-Abs: anti-Mi2, anti-Jo1 with interstitial lung fibrosis

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12
Q

Dermatomyositis management

A

Prednisolone
Immunosuppressives + cytotoxics in resistant cases
Hydroxychloroquine/topical tacrolimus for skin disease

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13
Q

What is Sjögren’s

A

Chronic inflammatory autoimmune disorder (1˚ at age 40-60)

Lymphocytic infiltration and fibrosis of exocrine glands causes most symptoms

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14
Q

Sjögren’s presentation

A

Dry eyes, dry mouth, dry cough
Tough to swallow
Polyarthritis/arthralgia

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15
Q

Sjögren’s investigations

A

Anti-Ro and Anti-La Abs in 40% and 26% respectively, ANA in 74%, RF in 38%
Biopsy shows focal lymphocyte aggregation

Schirmer’s test measures conjunctival dryness
Rose Bengal staining may show keratitis

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16
Q

Sjögren’s management

A

Treat dryness (hypromelose drops for eyes, frequent drinks, gum)
NSAIDs + hydroxychloroquine for arthralgia
Immunosuppressants in severe

17
Q

What is SLE

A

Systemic lupus erythematosus - Autoantibodies made against many autoantigens, forming immune complexes which are not cleared well so host immune response causes wide-spread symptoms

18
Q

SLE diagnosis clinical criteria

A

Typically women of child-bearing age:
Malar/Discoid rash
Non-scarring alopecia

Oral/nasal ulcers
Synovitis
Serositis (pleura or pericardium)

Proteinurea/red cell casts in urine
Neuro features
Haemolytic anaemia/Leucopenia/Thrombocytopenia

19
Q

SLE diagnosis laboratory criteria

A

ANA +ve (in 95%)
Anti-dsDNA
Anti-Smith
Antiphospholipid

Low C3/C4/C50 (therefore C3d/C4d raised as these are breakdown products)
Direct Coombs test +ve (Abs to RBCs)

CRP usually normal but ESR raised

20
Q

Drug induced lupus characteristics

A

Anti-histone Abs in >95%
Skin and lung signs mainly
Disease remits when drug stopped

21
Q

SLE management general

A

High-factor sunblock
Hydroxychloroquine for joint + skin
NSAIDs (unless renal disease)
Azathioprine, methotrexate, mycophenolate steroid sparing treatments
Belimumab (B-cell stimulating factor blocker)

22
Q

SLE management mild flares

A

Hydroxychloroquine/low dose steroid

23
Q

SLE management moderate flares

A

DMARDs or mycophenolate

24
Q

SLE management severe flares

A

High-dose steroids

Mycophenolate
Cyclophosphamide

Rituximab

Neurological testing also needed

25
Q

SLE management lupus nephritis

A

Steroids + cyclophosphamide/mycophenolate for immunosuppression

26
Q

Anti-phospholipid syndrome issues

A

Coagulation defects
Livedo reticularis (purple rash on face)
Obstetric (recurrent miscarriage)
Thrombocytopenia

27
Q

Anti-phospholipid syndrome treatment

A

Anti-coagulation therapies

Seek advice in pregnancy

28
Q

Lupus associations

A

Increased risk of CVD
Osteoporosis
Anti-phospholipid syndrome

29
Q

Anti-phospholipid antibody association

A

anti-cardiolipin

30
Q

Wegener’s granulomatosis antibody association

A

cANCA antibodies

Rheum (4 decks)

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