Connective Tissue Disease Flashcards
Any biological tissues w/ an extensive matrix that:
- provide structure, support & defense
- transport material
- bind organs together
Consist of:
- Cell fibers (collagen -tough, & elastic -elastic)
- Ground substance (water, fluid, protein)
Connective Tissues
1) Loose Tissue
- Composed of collagen & elastin
- E.g. blood vessels, internal organs
2) Fibrous Tissue
- Mostly collagen, few elastin
- E.g. tendons, ligaments
3) Adipose Tissue
- Fat cells
4) Blood
- The cells are separated by fluid
5) Cartilage
6) Bone
Types of Connective Tissue
Any disease that targets connective tissue
Many of them feature ABNORMAL IMMUNE SYSTEM ACTIVITY (systemic autoimmune disease)
- Inflammation in tissues as a result of attack’s by one’s own immune system (autoimmunity)
Exact cause unknown
Connective Tissue Diseases
Genes
- 10-12% of SLE pt’s have 1st or 2nd degree relatives w/ SLE
- <1% in healthy people
Behaviour
- Smoking, stress
Environment
- Pollution/pesticide, infections, medications
Causes of Autoimmune Diseases
Systemic Lupus Erythematosus
Overlap Syndrome
(mixed connective tissue disorder)
Scleroderma
Polymyositis/dermatomyositis
Family of Connective Tissue Diseases
“A disease of a thousand faces”
‘butterfly-shaped malar rash’
Main subtypes:
- Discoid Lupus Erthematosus
- Systemic Lupus Erythematosus (SLE)
- Subacute Cutaneous Lupus Erthematosus
Lupus Erythematosus
Multi-system disease affecting:
- Skin (rash, hair loss)
- Lungs
- Kidney
- CNS
- Circulatory system
- Heart
- GI System
- Muscle & joints
- Oral & nasal ulcers
SLE
Prevalence: 20-150 in 100,000 worldwide
Incidence: 1-10 in 100,00 worldwide
Highest risk: women during reproductive years
Female:Male = 9:1 (post-puberty & premenopausal age)
More common in some populations (compared to Caucasians):
- African American 3-6x
- Hispanic & Native American 2-3x
- Asian 2x
SLE Epidemiology
1) Malar rash
2) Discoid rash
3) Photosensitivity
4) Oral ulcers
5) Non-Erosive Arthritis
6) Pleuritis or pericarditis
7) Renal disorder
8) Neurological disorder (seizures or psychosis)
9) Hematologic disorder
10) Immunologic disorder
11) Positive antinuclear antibody test
Classified if 4 OR MORE of the 11 criteria are present
Sensitivity: 85%
Specificity: 95%
SLE Classification Criteria
Non-Erosive arthritis
- Transient
- Symmetrical
- Affecting small joints
- Less severe than RA
- When arthritis is present, 5-40% of patients develop a non-erosive arthopathy due to ligamentous laxity & muscle contracture
SLE MSK Clinical Features
- Seizures
- Psychosis
- Behaviours/personality changes, depression
- Cognitive dysfunction
- Stroke
- Peripheral neuropathy
- Chorea (abnormal involuntary movement disorder)
- Transverse myelitis
SLE Neurologic Clinical Features
Up to 50% of pt’s develop Lupus Nephritis
10% require kidney dialysis or transplant
Usually asymptomatic
Hallmark clinical finding: Proteinuria
Severe case: Gross hematuria (visible blood in urine)
Most frequent cause of disease-related death
SLE Renal Clinical Features
15-75% of pt’s:
- Oral ulcers
- Nausea
- Vomiting
- Pancreatitis
- Hepatitis
- Mesenteric Vasculitis (severe abdominal pain)
SLE GI Features
Pain (fibromyalgia)
Severe fatigue
Memory loss
Depression
SLE Other Symptoms
Pain Management
Therapeutic Exercise & Physical Activity
Patient Education
- Energy conservation principles
- Joint protection principles
- Sun protection
SLE PT Management