SLE and PSS

Question 1

What is Systemic Lupus Erythematosus (SLE)?

Answer 1

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

Question 2

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

Answer 2

• 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

Question 3

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

Answer 3

• 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

Question 4

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

Answer 4

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

Question 5

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

Answer 5

• 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

Question 6

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

Answer 6

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)

Question 7

What is a key difference with SLE and PSS symptomatically?

Answer 7

SLE = Butterfly rash
PSS - Tight tense skin

Question 8

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

Answer 8

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

Question 9

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

Answer 9

• 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

Question 10

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

Answer 10

• 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

Question 11

Radiographically, what is the difference between RA and SLE?

Answer 11

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