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MSK Systems > 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
Photosensitivity
Oral ulcers
Arthritis
Serositis - pleurisy or pericarditis
Renal involvement
Neurological
Hematological 
Immunological
ANA
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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 4 hematological features of SLE

A

Low WCC
Low platelets
Lymphocytes
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
CREST syndrome
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)

A

Up to elbows
Distal to knees
No trunk involvement or proximal limbs

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14
Q

What is sclerodactyly

A

localized thickening and tightness of skin of fingers/toes

often leads to skin ulceration of distal digits, commonly accompanied by atrophy of underlying soft tissues

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15
Q

Complications of CREST syndrome

A

Pulmonary hypertension

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16
Q

Diffuse systemic scleroderma
Affected areas
Progression
Complications (3)

A

Affected areas of diffuse systemic scleroderma - skin, trunk
Rapidly progressive - progressing to visceral organs e.g. kidney, heart, lungs, GIT
Complications
1. Pulmonary hypertension and fibrosis
2. Small bowel bacterial overgrowth
3. Renal crisis

17
Q

Treatment of scleroderma (2)

A

Beta blockers - peripheral vasodilation

Sildenafil to treat pulmonary HTN and Raynaud’s phenomenon

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

A

Schimer’s test - to measure tear production

21
Q

Autoimmune myositis
Prevalence
3 defining features

A

Rare as it is 6 per million incidence
Muscle weakness
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
3 imaging
4 lab

A

Imaging: EMG, MRI, muscle biopsy
Workup: elevated CK, elevated aldolase, elevated liver enzymes, elevated lactate dehydrogenase (LDH)

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

A

prednisolone

methylprednisolone - 20% stronger

27
Q

Complications of giant cell arteritis

A

Loss of vision - irreversible

28
Q

Autoimmune myositis complications (2)

A

Cancer and interstitial lung disease

MSK Systems (7 decks)

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