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MSK > Connective tissue diseases > Flashcards

Connective tissue diseases Flashcards

SLE Scleroderma Sjogren's syndrome Autoimmune myositis Giant cell arteritis

1
Q

SLE definition (4)

A

a systemic autoimmune disorder
widespread deposition of immune complexes containing autoantigen and autoantibody
type III hypersensitivity
predilection for skin, joints, kidney

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2
Q

What autoantibody is present in >95% of SLE patients?

A

ANA is present in >95% of SLE patients

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3
Q

Explain the sensitivity and specificity of ANA for SLE

A

High sensitivity for SLE but low specificity for SLE

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4
Q

SLE: epidemiology
Gender distribution
Ethnicity
Onset

A

SLE epidemiology
F:M 9:1
Afro-Caribbeans > Asian > White
young - 15-50 yo

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5
Q

Classification criteria for SLE [11]

A 
Malar rash
Discoid rash
Non-scarring alopecia
Oral/nasal ulcers
Synovitis
Serositis - pleurisy or pericarditis
Renal involvement
Neurological
Hematological 
Immunological
Lab criteria
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6
Q

Name 2 renal features of SLE part of the classification criteria

A

Significant proteinuria

Cellular casts in urine

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7
Q

Name 3 hematological features of SLE

A

Low WCC
Low platelets
Hemolytic anemia

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8
Q

Scleroderma: epidemiology

A

F:M 3:1

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9
Q

Scleroderma: onset

A

30-50 yo

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10
Q

Scleroderma define

A

Chronic

Thickening and hardening of skin

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11
Q

Name 3 types of scleroderma

A

Scleroderma morphea/ localized scleroderma
Limited cutaneous scleroderma (subtype CREST)
Diffuse systemic scleroderma

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12
Q

Scleroderma morphea characteristics (3)

Prognosis and mortality

A

Localised patches of hardened skin - smooth and shiny
On trunk
No internal organ involvement
Good prognosis, low mortality

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13
Q

CREST syndrome - affected areas (2)

What antibodies to test for [2]

A

Face, distal limbs
No trunk involvement or proximal limbs
Mild pulmonary HTN
ANA, Anti-Centromere antibody

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14
Q

What is sclerodactyly [3]

A

localized thickening and tightness of skin of fingers/toes [1]
often leads to skin ulceration of distal digits, [1] commonly accompanied by atrophy of underlying soft tissues [1]

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15
Q

Complications of
CREST syndrome [1]
Diffuse systemic [3]

A

Pulmonary hypertension

Diffuse systemic

  1. Pulmonary hypertension and fibrosis
  2. Small bowel bacterial overgrowth
  3. Renal crisis
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16
Q

Diffuse systemic scleroderma
Affected areas
Progression
Autoabs to test for [2]

A

Affected areas: trunk and proximal limbs
Rapidly progressive
Progressing to organs e.g. kidney, heart, lungs, GIT
ANA, Anti-SCL70

17
Q

Treatment of scleroderma
Symptomatic [4]
Diffuse [1]

A

Symptomatic:

  • Raynaud’s: CCB, sildenafil
  • Myositis: CCS
  • Renal crisis: ACEI
  • Cardiac: anti-arrhythmic

Diffuse:
- late stage pencillamine

18
Q

Sjogren’s syndrome
Epidemiology - gender
Onset

A

F:M 9:1

Onset - 40-50 yo

19
Q

Clinical features of Sjogren’s syndrome

A

Dry eyes, mouth
Parotid gland enlargement
Systemic symptoms: fatigue, fever, myalgia, arthralgia

20
Q

Ix Sjogren’s [2]

Lab diagnostic tests [2]

A 
Schirmer's test - to measure tear production
Minor salivary gland biopsy
Lab:
ANA, Anti-Ro, Anti-La
RF positive
21
Q

Autoimmune myositis
Prevalence
2 types

A

Rare as it is 6 per million incidence
Polymyositis
Dermatomyositis

22
Q

Autoimmune myositis

Characteristics of muscle weakness (3)

A

Muscle weakness in autoimmune myositis is symmetrical, diffuse, proximal

23
Q

Autoimmune myositis

Characteristics of dermatomyositis (5)

A 
Gottron's papules - scaly rash over MTP joints
Heliotrope rash
Shawl's sign
Nail fold erythema
Subcut calcifications
24
Q

Autoimmune myositis

Investigations [4]

A

Muscle enzymes: CK, AST, ALT, LDH, aldolase (elevated)
Immunology: ANA (+ve), anti-Jo1 antibodies (+ve)
EMG: characteristic fibrillation potentials
Muscle biopsy: confirms dx and excl. other dx
MRI: oedema in acute myositis

25
Q

Giant cell arteritis
ACR criteria for classification for GCA
What are the 5 criteria

A
  1. Age at onset 50 years or more
  2. New onset localized headache - unilateral
  3. Temporal artery tenderness - reduced pulsation
  4. ESR more or equal to 50
  5. Abnormal temporal biopsy
26
Q

Giant cell arteritis: Treatment [3]

A

prednisolone + low dose aspirin

methylprednisolone - 20% stronger

27
Q

Complications of giant cell arteritis

A

Loss of vision - irreversible

28
Q

Autoimmune myositis complications (2)

A

Cancer
-May be paraneoplastic phenomenon so screen for cancers

Interstitial lung disease

29
Q

Name 4 neurological features of SLE as part of its classification

A

Seizures, psychosis, myelitis, peripheral neuropathy

30
Q

Lab criteria for SLE as part of its classification

A 
ANA
				□ Anti-dsDNA
				□ Anti-Sm
				□ Low complement
				□ APS
Positive DCT
31
Q

Two other investigations to do for SLE

A

ESR high, CRP normal

BP

32
Q

Drug induced lupus

Which organ systems are more commonly involved? [2]

A

Skin and lung signs are more common

Renal and CNS rarely affected

33
Q

Management SLE [6]

A 
High factor sunblock
Hydroxychloroquine
Topical steroids
NSAIDs unless renal disease
Steroid sparing agents
Biologic
34
Q

Management SLE
Name 3 steroid sparing agents
Name 1 biologic used in maintenance
4 Rx for severe flares

A 
Azathioprine
Methotrexate
Mycophenolate
Biologic: belimumab
Severe flares:
- Urgent high dose steroids
- Mycophenolate
- Rituximab
- Cyclophosphamide
35
Q

Management of lupus nephritis [3]

A

Steroid sparing agents - step up
Renal replacement therapy
Renal transplant

36
Q

Mixed CTD

Lab test [1]

A

Anti-u1RNP

37
Q 
APS
Recurrent miscarriages are a common feature
Autoabs to test for [3]
CLOT
Rx [1]
A

Anti-cardiolipin ab, lupus anticoagulant ab, anti-beta2 glycoprotein1

Coagulation defect
Livedo reticularis
Obstetric - recurrent misc
Thrombocytopenia

Rx: lifelong warfarin + aspirin

38
Q

Autoimmune myositis mx [3]

A

Prednisolone
Hydroxychloroquine
Topical tacrolimus

MSK (28 decks)

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