Connective Tissue Diseases Flashcards
3 systemic symptoms of autoimmunity
- weight loss
- poor appetite
- fever
3 non-specific signs and symptoms of autoimmunity
- Raynaud’s
- Sicca (dry eyes & mouth)
- Diffuse lymphadenopathy (swollen lymph nodes in multiple areas of the body)
More specific clues that make us suspect autoimmunity (6)
- ARTHRITIS
- Some skin rashes
- Mucosal ulcers
- Ocular symptoms
- Lung involvement
- Renal dysfunction
- Peripheral nerve defect
Which type of arthritis is indicative of autoimmunity?
Inflammatory arthritis
Inflammatory arthritis vs Osteoarthritis
Inflammatory arthritis:
* Autoimmune disease
* Immune attack on the joints
* Affects small joints of the hands and feet
Osteoarthritis:
* Degenerative disease
* “Wear-and-tear” of joints
* Affects large joints (knees, hips, back)
2 “non-specific” laboratory tests that are usually elevated in the presence of inflammation.
- Erythrocyte sedimentation test
- C-reactive protein (CRP)
These are non-specific markers of inflammation
Specific marker (lab test) of autoimmune disease
Presence of auto-antibodies in the serum (on blood test)
True or false: Presence of auto-antibodies guarantees that the patient has an autoimmune disease
False! Their presence does not guarantee the presence of an autoimmune disease (false-positives)
True or false: Absence of auto-antibodies guarantees that the patient does not have an autoimmune disease
False! Their absence does not rule out an autoimmune disease (false negatives)
True or false: The level of auto-antibodies usually correlates with disease severity or activity
False! There is no correlation (with certain exceptions)
True or false: The role of auto-antibodies in the pathophysiology of autoimmune diseases is clear.
False! Their role is not clear!
In some diseases, autoantibodies directly cause damage. However, in other diseases, autoantibodies are present, but not the main drivers of disease. In many such cases, there’s no strong evidence they directly cause the disease. Instead, the pathophysiology might involve T cells, cytokines, or other immune mechanisms instead.
How can we obtain a clear, specific diagnosis and prognosis of a given autoimmune disease
Biopsy!
Autoimmune rheumatic diseases (definition)
A group of autoimmune inflammatory disorders that affect connective tissues and joints.
On a biopsy and pathology examination, what confirms that a process is auto-immune?
Presence of immune cells and immune complex deposits in the tissue.
Name the 4 types of autoimmune rheumatic diseases
- Connective tissue diseases
- Inflammatory arthropathies
- Inflammatory myositis
- Systemic vasculitis
4 autoimmune rheumatic diseases categorized as connective tissue diseases
- Systemic lupus erythematosus (SLE)
- Scleroderma
- Sjogren’s syndrome
- Mixed connective tissue disease (MCTD)
Scleroderma (definition)
Autoimmune disease characterized by uncontrolled fibrosis of the skin, blood vessels, and other organs.
2 patterns of scleroderma
Limited (CREST syndrome - only extremities and face)
Diffuse (upper arms and legs, trunk)
Most scleroderma patients will be positive for… (2)
- Raynaud’s syndrome
- Positive ANA (anti-nuclear antibody)
Organs most affected by scleroderma (5)
- Skin
- Blood vessels
- Lungs
- GI tract
- Kidneys
Scleroderma: Physical exam findings (5)
- Patient is unable to flex his joints and form a fist
- Ulcers develop on joints due to tightening of the skin
- Pursed lips and difficulty opening the mouth
- Shiny skin, salt-and-pepper pattern
- Telangiectasias (small dilated blood vessels)
Sjogren’s syndrome (definition)
Autoimmune disease that primarily affects the body’s exocrine glands (esp. salivary and lacrimal glands)
Sjogren’s syndrome manifestations (2)
- Dry mouth (xerostomia)
- Dry eyes (xerophthalmia)
These are also referred to as Sicca symptoms
Sjogren’s syndrome is associated with 2 auto-antibodies
anti-Ro (SSa)
anti-La (SSb)
