Connective Tissue Disease Flashcards

1
Q

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)
A

Connective Tissues

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2
Q

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

A

Types of Connective Tissue

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3
Q

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

A

Connective Tissue Diseases

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4
Q

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

A

Causes of Autoimmune Diseases

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5
Q

Systemic Lupus Erythematosus

Overlap Syndrome
(mixed connective tissue disorder)

Scleroderma

Polymyositis/dermatomyositis

A

Family of Connective Tissue Diseases

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6
Q

“A disease of a thousand faces”

‘butterfly-shaped malar rash’

Main subtypes:

  • Discoid Lupus Erthematosus
  • Systemic Lupus Erythematosus (SLE)
  • Subacute Cutaneous Lupus Erthematosus
A

Lupus Erythematosus

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7
Q

Multi-system disease affecting:

  • Skin (rash, hair loss)
  • Lungs
  • Kidney
  • CNS
  • Circulatory system
  • Heart
  • GI System
  • Muscle & joints
  • Oral & nasal ulcers
A

SLE

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8
Q

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
A

SLE Epidemiology

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9
Q

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%

A

SLE Classification Criteria

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10
Q

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
A

SLE MSK Clinical Features

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11
Q
  • Seizures
  • Psychosis
  • Behaviours/personality changes, depression
  • Cognitive dysfunction
  • Stroke
  • Peripheral neuropathy
  • Chorea (abnormal involuntary movement disorder)
  • Transverse myelitis
A

SLE Neurologic Clinical Features

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12
Q

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

A

SLE Renal Clinical Features

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13
Q

15-75% of pt’s:

  • Oral ulcers
  • Nausea
  • Vomiting
  • Pancreatitis
  • Hepatitis
  • Mesenteric Vasculitis (severe abdominal pain)
A

SLE GI Features

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14
Q

Pain (fibromyalgia)
Severe fatigue
Memory loss
Depression

A

SLE Other Symptoms

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15
Q

Pain Management

Therapeutic Exercise & Physical Activity

Patient Education

  • Energy conservation principles
  • Joint protection principles
  • Sun protection
A

SLE PT Management

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16
Q

Prioritize:

  • What activities are the most important to you?
  • Are friends/family able to help?

Plan:

  • Self-monitoring: Know when & why you have less energy
  • Balance exercise w/ rest
  • Eat a well-balanced diet & avoid heavy meals

Pace:

  • Balance between rest & activity
  • Regular breaks during the day
  • Adopt a regular routine
  • Good sleep

Posture:

  • Maintain good posture
  • Break up sitting by periods of standing
A

Energy Conservation Principles

17
Q

1) Respect for pain
2) Balance activity & rest
3) Avoid repetitive activities (reduce joint stress)
4) Use larger, stronger joints for activities
5) Avoid staying in one position for a prolonged period

6) Maintain use of joints in good alignment
- Avoid tight grips
- Use large handles (MCP joints)

7) Maintain a healthy body weight
8) Maintain a good posture during daily activities

A

Joint Protection Principles

18
Q

Connective tissue disease is a complex condition

Pain control, physical activity, & patient education are the main non-pharmacologic interventions in the management of connective tissue disease such as SLE

Exercise & physical activity are safe effective tx. for people w/ SLE

A

Key Messages