Screening for Autoimmune Disorders Flashcards
What is the definition of autoimmune disorders
- conditions in which the immune response is directed against the body
- self-destructive
- > 56 autoimmune disease
- organ specific
- systemic
What are the different types of autoimmune disorders?
- Organ-specific
- systemic
What are the potential etiologies of Autoimmune disorders
- idiopathic
- genetic
- hormonal
- environmental
What are some common lab test results common with autoimmune diseases
- thrombocytopenia
- leukopenia
- increased or decreased immunoglobin (not used alone)
- rheumatoid factor
- antinuclear antibodies: abnormal protein
- increase muscle enzymes
Common clinical features of autoimmune disorders
- pleuritis
- myocarditis
- endocarditis
- pericarditis
- vasculitis
- synovitis
- myositis
- skin rash
- altered connective tissue
- peritonitis
- constitutional symptoms (usually initially)
A lot of inflammation
What is intravenous immunoglobin therapy
- individual immunoglobulin G (IgG) has antibodies against many types of proteins and auto-antibodies
- IVIg: pooled IGg from donors
- modulates immune response: acute and chronic use
What is polymyositis/dermatomyositis and what is it assoicated with
- most common idiopathic disease of muscle
- can be idiopathic
- can be assoicated with cancer (breast, lung, ovarian, colon, gastric)
- can be assoicated with other diseases: RA, lupus, scleroderma
What is the pathophysiology for polymyositis/dermatomyositis and symptoms of the disease
- Suspected mechanism: T-helper cells attack muscle fibers
symptoms:
- weakness, usually starts proximally; symmetrical (often starts in LE)
- can be rapid but is usually insidious
- malaise and fatigue more common than weakness
- may eventually have cardiac and pulmonary involvement
What is the difference between polymyositis/dermatomyositis
- dermatomyositis = skin involvement
- rash is typically on eyelids, face, and extremities/exentsor surface
polymyositis/dermatomyositis Diagnostic testing
- increase in CPK, CK, or CK-MB
- positive antibody test
- muscle inflammation can be seen on an MRI
- EMG changes related to fibrotic changes, muscle membrane irritability, weakness, denervation/reinnervation
What are likely EMG findings for polymyositis/dermatomyositis
- increased insertional activity
- spontaneous activity at rest
- with activity: reduced amplitude of M waves, polphasic waves, complex repetitive discharge
polymyositis/dermatomyositis treatment
- medication: corticosteriods, immunosuppressants, intravenous immoglobulin therapy
- exercise/rest: distrubted practice works well
- prognosis improves with good medical management
Polymyalgia rheumatic
who does it affect and what is it assoicated with
- affects people over 55 esp over 80 years old
- related to virus, stress, autoimmune
- associated with high ESR
- muscle pain and stiffness vs joint pain
- usually self-limiting
- assoicated condition: giant cell arteritis; inflammation of medium arteries (may require medication with this)
Polymyalgia rheumatic
signs and symptoms
- appear suddenly
- proximal muscles
- muscle stiffness in morning
- weakness, fatigue, malaise
- headahces
- vision changes
- depression
- weight loss
- jaw pain dysfunction but not TMJ
Chronic Inflammatory demyelinating polyneuropathy (CIDP) cause/assoicated with
- cause is unknown
- appears to be related to a trigger such as acute illness or surgery, ICU, trauma
- assoicated with chronic disease: diabetes, HIV, lupus, Lymphoma, chronci hepatitis, thryrotoxicosis
Chronic Inflammatory demyelinating polyneuropathy (CIDP) signs and symptoms
- weakness or paralysis
- paresthesias
- Ms atrophy
- fatigue
- cranial nerve dysfunction
- impaired motor control
- areflexia
LMN probelm
Chronic Inflammatory demyelinating polyneuropathy (CIDP) treatment
- medical treatment: steriods, immunosuppressants, plasma exchange or plasmapheresis, IVIg
- PT may have a longer duration of care or multiple epsiodes
- electrodianostic findings: NCV = increased latency or EMG abnormal findings
medical interventions for autoimmune disease
- intravenous immunoglobulin therapy
- plasma exchnage plasmapheresis (not from donors from ones own blood)
What is lambert-eaton myasthenic syndrome (LEMS)
- frequently assoicated with autoimmune disorders
- antibodies attack Ca++ channels at presynaptic junction, impairing ACh release
- Assoicated with cancer esp small cell lung cancer
LEMS symptoms
- proximal weakness usually affecting legs first
- may see slight increase in strength with repeated contractions
- hyporeflexia
- autonomic dysfunction: dry mouth, constipation
- electrodiagnostic findings
LEMS treatments
Lambert-Eaton myasthenic syndrome
- plasmapheresis/plasma exchange
- intravenous immunoglobulin therapy (IVIg)
- prednisone
- acetylcholinesterase inhibitors
- aminopyridines (potassium channel blockers)*
*K+ and Ca++ work together so blocking K+ allows C++ to be released
PT exam and intervention for autoimmune disease contains?
- outcome measure: functional endruance
- strength exam
- functional mobility
- aerobic capacity training
Guillain Barre Syndrome: what is it
- acute inflammatory polyneuropathy
Guillain Barre Syndrome
incidence
- annual incidence 1-2 per 100,000 world wide
- males and females equally affected
- most common in people ages 30-50 years
- 20% of case < 20 years
GBS etiology
- recent infection thought to cause an auto-immune reponse
- ie: respiratory virus or GI virus
- auto-immune respones directed towards: myelin or axon of peripheral nerve
GBS: what can it be refered to as when it is directed towards myelin vs axon
- myelin: acute inflammatory demyelinating polyneuropathy
- axon: acute motor axonal neuropathy or acute motor and sensory axonal neuropathy
Guillain Barre Syndrome: signs and symptoms
- paresthesia in hands and feet
- bilateral, symmetrical weaknes; most often progresses distal to proximal
- usually starts in lower legs and progresses to arms and face; cranial nerve involvement
- respiratory muscles can be affected; may need mechanical ventilation
Guillain Barre Syndrome: diagnosis
- diagnosis based on progressive BUE and BLE weakness with areflexia
- electrodiagnostic findings
GBS progression
timeline
- symptoms usually progress over ~ 2 weeks
- duration of illness usually about 12 weeks
- most patients start to improve about 2-4 weeks after onset
- most recovery occurs in first 2 years
- may show improvement for up to 10 years
Guillain Barre Syndrome: prognosis
- 80% of patients are able to walk after 6 months
- 50% have some residual neurological deficits
- 15% have continued activity limitations
- ~5% die of secondary organ failure or complications
Guillain Barre Syndrome
PT exam and intervention
- outcome measures
- complications related to immobility
- pain fatigue
- overwork weakness
Myasthenia Gravis
- autoimmune syndrome
- caused by antibodies to the ACh receptors
- results in a decrease in the number of receptors
Myasthenia gravis: symptoms and patient population
what muscles do the weakness effect, how does it vary and population
- weakness of eyes, mouth, throat, and limbs
- symptoms variable within a single day
- more apparent when the person is tired
- may also affect the respiratory muscles
- common in young adult women and older men
Myasathenia gravis
treatment
- removal of thymus gland (if assoicated with thymoma)
- plasmapheresis
- IVIg
- steriods
- acetylcholinesterase inhibitors
Myasthenia gravis
PT exam and intervention
- outcome measures: functional endurance
- strength exam
- functional mobilty
- aerobic capactiy training
