SLE and PSS Flashcards
What is Systemic Lupus Erythematosus (SLE)?
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
What are the clinical features of Systemic Lupus Erythematosus (SLE)?
- 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
What are the signs and symptoms of Systemic Lupus Erythematosus (SLE)?
- 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
What are the locations of Systemic Lupus Erythematosus (SLE)?
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
What are the radiological features of Systemic Lupus Erythematosus (SLE)?
- 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
What is Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?
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)
What is a key difference with SLE and PSS symptomatically?
SLE = Butterfly rash
PSS - Tight tense skin
What is the location of Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?
- Hands - most common site of involvement.
- Spine, pelvis, and large peripheral joints generally spared
What are the clinical features of Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?
- 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
What are the radiological findings of Progressive Systemic Sclerosis (PSS) (AKA Scleroderma)?
- 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
Radiographically, what is the difference between RA and SLE?
RA = erosions. Morning stiffness lasts hours
SLE = No erosions. Morning stiffness last minutes