SLE and PSS Flashcards

1
Q

What is Systemic Lupus Erythematosus (SLE)?

A

Generalized connective tissue disorder
Inflammation in multiple organ systems
Immune hyperactivity - abnormal immune activation and loss of self-tolerance.
Chronic autoimmune disease with periods of acute exacerbations & remissions
Varying prognosis

	- Females > Men
	- M/C in women of childbearing age
	- Rare in patients >45 years
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2
Q

What are the clinical features of Systemic Lupus Erythematosus (SLE)?

A
  • Unknown Cause
    • Increased ESR
    • Environmental triggers:
      - foods
      - drugs
      - UV light
      - microorganisms (bacteria, viruses, parasites)
      • Most frequent and serious feature = glomerulitis > renal failure
      • Possible Raynaud’s phenomenon
        • Vascular spasm causing cold and numbness in the hands and feet
      • Steroids may cause > Osteoporosis, AVN, spinal Fx, and GI ulcers.
      • Prone to infection (bone)
      • Myositis
        • 30-50% diffuse muscle tenderness, weakness and atrophy

Neck of femur and spinal fractures (throacic) are m/c

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3
Q

What are the signs and symptoms of Systemic Lupus Erythematosus (SLE)?

A
  • Malaise
    • Overwhelming fatigue
    • Fever
    • Anorexia
    • Anaemia
    • Weight loss
    • Polyarthralgia
    • Erythematous skin rash - most characteristic physical feature
      - face, neck, elbows, and dorsum of hands.
      - Malar erythema on face (“Lupus”) - BUTTERFLY
    • Alopecia – common
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4
Q

What are the locations of Systemic Lupus Erythematosus (SLE)?

A

M/C in femoral and humeral heads.
- Look for in unusual locations such as carpals, metacarpals, tarsals, and metatarsals
- Spinal changes unusual but;
- Atlantoaxial instability < 5%
- compression Fxs secondary to corticosteroid therapy

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5
Q

What are the radiological features of Systemic Lupus Erythematosus (SLE)?

A
  • Transient reversibility of deformities - Most prominent in hands
    - Especially in longstanding ds
    - Little functional disability
    - Completely reducible (disappears with pressure)
    - deformity results from ligamentous laxity and contracture due
    to muscle imbalance
  • Joint space loss and erosions- not prominent feature
    - helps DDX from RA
  • Generalized osteoporosis may be prominent
  • Tuftal resorption - seen with accompanying Raynaud’s
  • Possible osteonecrosis with or without steroids.
  • Soft tissue Calcification (Ca++ ):
    - diffuse linear, streaky, or nodular Ca++ in subcutaneous and
    deeper tissues, particularly in lower extremities
    - focal or localized plaque-like Ca++
    - periarticular Ca++
    - arterial Ca++

Reduced bone density
Reduced joint space
Avascular necrosis – tissue death – most likely due to steroid use

Calcification - MC lower extremities

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6
Q

What is Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?

A

An uncommon disease marked by fibrotic increase in skin and visceral organs
Affects various organs and systems MC Oesophageal/digestive
Unknown aetiology
Generalized disorder of small arteries and micro vessels
Characterized by scarring (fibrosis) and vascular obliteration in the skin, GI tract, lungs, heart, and kidneys
Tight – tense (hidebound) skin = clinical hallmark
Organ compromise = prognostic keystone

	- Females > Males 3:1
	- 30- 50 yoa (manifests)
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7
Q

What is a key difference with SLE and PSS symptomatically?

A

SLE = Butterfly rash
PSS - Tight tense skin

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8
Q

What is the location of Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?

A
  • Hands - most common site of involvement.
    - Spine, pelvis, and large peripheral joints generally spared
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9
Q

What are the clinical features of Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?

A
  • CREST
    - Calcinosis
    - Raynaud’s phenomenon
    - Esophageal abnormality
    - Smooth muscle atrophy and fibrosis of the distal two-thirds
    - Decreased peristalsis
    - Scleroderma
    - Telangiectasia
    - Tiny blood vessels cause threadlike red lines or patterns on the skin
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10
Q

What are the radiological findings of Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?

A
  • Bone and soft tissue changes
    - Acro-osteolysis (tuftal resorption of the distal phalanges)
    - Periarticular osteopenia
    - Joint space narrowing
    - Erosions
    - Subcutaneous and periarticular calcification
    - Atrophy especially at tips of fingers
    - Flexion contractures
    - Rib resorption, mandibular angle resorption, radius and ulna resorption
    - Terminal phalangeal sclerosis
    DDX: RA

Calcifications MC Hands

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11
Q

Radiographically, what is the difference between RA and SLE?

A

RA = erosions. Morning stiffness lasts hours
SLE = No erosions. Morning stiffness last minutes

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