Exam 2 Musculoskeletal Diseases Flashcards
What is scleroderma?
Inflammation and autoimmune disease that causes vascular injury and vascular obliteration. Chronic hardening and tightening of the skin and connective tissues.
Tissue fibrosis and organ sclerosis.
What are the 3 forms of scleroderma?
- Localized scleroderma- skin, face, distal limb
- Limited cutaneous systems sclerosis - CREST syndrome, has prominent skin manifestations
- Diffuse cutaneous system sclerosis- generalized skin involvement and cardiac complications.
The limited symptoms of scleroderma are referred to as CREST. What does CREST stand for?
S/S of Scleroderma
Skin:
MS:
Nervous System:
CV:
Skin: Taut skin
MS: Limited mobility/contractures, skeletal muscle myopathy
Nervous System: Nerve compression
CV: Systemic and pulmonary HTN, dysrhythmias, vasospasm in small arteries of fingers, CHF
S/S of Scleroderma
Pulmonary:
Renal:
GI:
Pulmonary: Diffuse interstitial pulmonary fibrosis, decreased pulmonary compliance (stiff lungs)
Renal: Decreased renal blood flow and systemic HTN
GI: Xerostomia (dry mouth), poor dentition, fibrosis of GI tract (no Reglan, will not stimulate fibrotic GI tract), reflux
Scleroderma Treatment
Alleviating symptoms…
ACE inhibitors for renal protection - only treatment that has been proven to alter the course of scleroderma
SclerodermaAnesthesia Management and Considerations
Airway:
IV:
Pulmonary:
GI:
Eyes:
Other:
Airway: Mandibular motion, small mouth opening, neck ROM (do not manipulate neck while asleep), oral bleeding
CV: IV/arterial line access may be difficult, use US.
Pulmonary: Decreased pulmonary compliance and reserve, avoid increasing PVR (do not make them hypoxic)
GI: Aspiration caution, increase gastric pH by using PPI.
Eyes: tape eyes, ointment, gauze, goggles
Other: Regional anesthesia may be best for these patients, keep patient warm, VTE prophylaxis, Positioning with care, Pulse ox difficulties (ear probe)
What is Pseudohypertrophy Muscular Dystrophy/ Duchenne Muscular Dystrophy (DMD)
Mutation in the dystrophin gene (x-linked recessive) causes fatty infiltration leading to pseudohypertrophic muscles.
Common in 2-5 y/o boys
By age 8-10 Wheelchair bound
Usually live until ages 20-25 years (CHF, pneumonia)
Initial symptoms of Duchenne Muscular Dystrophy
Initial symptoms: waddling gait, frequent falling, difficulty climbing stairs, tiptoes.
Duchenne Muscular DystrophyS/S
CNS:
MS:
CV:
Pulmonary:
GI:
CNS: Intellectual disability
MS: Kyphoscoliosis, skeletal muscle atrophy, serum CK 20-100x normal d/t increase muscle permeability
CV: Sinus tachycardia, cardiomyopathy, EKG abnormalities (tall R-wave in V1)
Pulmonary: OSA, weakened respiratory muscles, and cough
GI: Hypomotility, gastroparesis
Duchenne Muscular DystrophyAnesthesia Management
Airway:
Pulmonary:
CV:
GI:
Airway: weak laryngeal reflexes and cough (You want these patients “singing” on the tube during emergence.)
Pulmonary: weakened muscles
CV: Pre-op EKG and/or echo based on the severity
GI: Delayed gastric emptying, aspiration risk
Other anesthesia management for Duchenne Muscular DystrophyAnesthesia:
What drug do you want to avoid?
When do you want to extubate these patients?
What type of incidence are higher in these patients?
What type of anesthesia may be best?
Avoid succinylcholine can cause hyperkalemia and rhabdomyolysis. Use NDMR (Rocuronium).
Pharyngeal and respiratory muscle weakness, secure the airway. Make sure the patient is fully awake.
MH – increased incidence. Have Dantrolene ready. Use TIVA as an alternative
Regional Anesthesia may be best than GA.
What is Myasthenia Gravis?
A chronic autoimmune disorder involving the NMJ. There will be decreased functional post-synaptic AChreceptors.
Muscle weakness w/ rapid exhaustion of voluntary muscles. Partial recovery with rest.
ACh receptor-bindingantibodies are linked to thymus abnormalities.
Myasthenia GravisS/S
Eyes:
Oral:
Pulmonary:
Skeletal:
Heart:
Endocrine:
Ptosis (droopy eyelid), diplopia (double vision)
Dysarthria (slurred speech), dysphagia, and difficulty handling saliva
Isolated respiratory failure (rare)
Arm, leg, or trunk muscle weakness
Myocarditis
Autoimmune diseases associated
RA, SLE, pernicious anemia, hyperthyroidism
Differentiate between Myasthenia GravisMyasthenic Crisis vs Cholinergic Crisis.
What is the effect of the Edrophonium/Tensilon Test in each condition?
Myasthenic crisis:
D/t drug resistance or insufficient drug therapy
S/S: severe muscle weakness and respiratory failure
Cholinergic crisis (SLUDGE):
D/t excessive anticholinesterase treatment
S/S: muscarinic side effects – profound muscle weakness, salivation, miosis, bradycardia, diarrhea, abdominal pain
Edrophonium/Tensilon Test:
1-2 mg IVPof edrophonium will improve the myasthenic crisis and makes the cholinergic crisis worse.
Myasthenia GravisTreatment
Anticholinesterases (first-line treatment):
Thymectomy (when will you see results):
Immunosuppression:
Immunotherapy:
Anticholinesterases:
The first line of treatment
Pyridostigmine last longer than neostigmine
Thymectomy:
Induces remission
Reduced use of immunosuppressives
Reduces ACh receptor antibody levels
Full benefit delayed (2-6 months)
Immunosuppression
Corticosteroids, azathioprine, cyclosporine, mycophenolate
Immunotherapy (short-term effect)
Plasmapheresis will remove antibodies from circulation.
Immunoglobulin will have a temporary effect.
Myasthenia GravisAnesthesia Management
Weakened ________ effort
Marked sensitivity to ______
Resistance to ____________
Weakened pulmonary effort (Aspiration risk)
Marked sensitivity to nondepolarizing muscle relaxants (decrease amount). Use nerve stimulator and titrate to nerve stimulator
Resistance to succinylcholine (might need a higher dose for effect, but this can lead patients into MG Crisis, just avoid using Sux)
What is Osteoarthritis?
This is the most common joint disease in the ____?
OA is characterized by _________ inflammation.
Does stiffness fade throughout the day?
Degenerative process affecting articular cartilage, the most common joint disease in the elderly.
Characterized by minimal inflammation usually a result of chronic joint trauma (sports), biomechanical stresses, joint injury, abnormal joint loading, neuropathy, ligament injury, muscle atrophy, and obesity.
Pain present with motion,relieved by rest. Morning stiffness fades throughout the day. (T-rex)
What joints will osteoarthritis affect?
Weight-bearing and distal interphalangeal joints.
Heberden nodes- Distal interphalangeal joints, first knuckle (pictured)
Protrusion of the nucleus pulposus (herniated disc) can lead to compression of nerve roots.
Degenerative disease – vertebral bodies and intervertebral disks. OA will affect the breakdown of the cartilage of the joints and discs in the neck and lower back. Middle to lower C-spine and L-spine.
OsteoarthritisTreatment
PT and exercise
Maintaining muscle function
Pain relief - heat, simple analgesics, anti-inflammatory drugs, transcutaneous nerve stimulator, acupuncture. OA patients are typically NOT on steroids
Joint replacement surgery - necessary when pain is debilitating
OsteoarthritisAnesthesia Management
Airway
Be aware of limited ROM, and keep them comfortable.
Document the patient’s position on the OR table.
Don’t lean on the patient.
What is Rheumatoid Arthritis?
An autoimmune-mediated, systemic inflammatory disease that usually affects the proximal interphalangeal and metacarpophalangeal joints (2nd & 3rd knuckle).
Rheumatoid nodules at pressure points (elbows)
Rheumatoid factor (IG antibody present in 95% of patients with RA)
Characteristics of Rheumatoid Arthritis
Single or multiple joints
Painful synovial inflammation, swelling, and increased fluid
Morning stiffness like OA, but remains stiff throughout the entire day.
Symmetrical distribution of several joints.
Fusiform swelling - Joints become enlarged and the fingers crooked (pictured)
Synovitis of the temporomandibular joint (decrease mouth opening)
Affects nearly all joints (except the t-spine and lumbosacral spine)
Rheumatoid Arthritis S/S
Atlantoaxial subluxation:
Cricoarytenoid arthritis:
Osteoporosis
NM
Atlantoaxial subluxation instability (C1 and C2): Odontoid process (dens) protrudes into the foramen magnum. The instability can place pressure on the transverse ligament and spinal cord. Instability can also impair vertebral artery blood flow.
Cricoarytenoid arthritis:
Acute – hoarseness, dyspnea, and stridor w/ tenderness over the larynx; swelling and redness of arytenoids
Chronic – asymptomatic or variable degrees of hoarseness, dyspnea, and upper airway obstruction
Osteoporosis: More common in women than men
NM: Weakened skeletal muscles (peripheral neuropathies)
Rheumatoid ArthritisS/S
CV:
Pulmonary:
Hematology:
Dryness:
CV: Pericarditis, accelerated coronary atherosclerosis
Pulmonary: Restrictive lung changes, decrease Vt
Hematology: anemia, neutropenia, elevated platelets
Dryness: Keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth)
Rheumatoid ArthritisTreatment
NSAIDS
Corticosteroids
DMARDs May take 2-6 months to see effects
Tumor necrosis factor (TNF-alpha) inhibitors and interleukin(IL-1) inhibitors.
TNF-alpha inhibitors work faster than DMARDs.
IL-1 inhibitors – slower onset and less effective.
Surgery is usually held off until the patient is in intractable pain, has impairment ofjoint function, and is in need of jointstabilization. Usually a total replacementsurgery.
Rheumatoid ArthritisAnesthesia Management
Airway- Atlantoaxial subluxation ,TMJ limitation, Cricoarytenoid joints. (handle w/care)
Severe rheumatoid lung disease
Protect eyes - Keratoconjunctivitis sicca (dry eyes)
Stress dose - give before surgery, may have a decrease amount of endogenous cortisol.
What is Systemic Lupus Erythematosus?
Typical Manifestation:
Multisystem chronic inflammation characterized by antinuclear antibody production.
Typical manifestations (Usually will have 3 out 5 of the following):
1. Antinuclear antibodies
2. Malar rash (butterfly rash)
3. Thrombocytopenia
3. Serositis- inflammation of a serous membrane around the organ
5. Nephritis
Systemic Lupus ErythematosusS/S
Join/Spine:
CNS:
CV:
Pulmonary:
Renal:
Joint/Spine: Polyarthritis and dermatitis, symmetrical arthritis (most common sx), no spinal involvement, avascular necrosis of the femoral head or condyle
CNS: Cognitive dysfunction, psychological changes
CV: Pericarditis, coronary atherosclerosis, Raynaud’s
Pulmonary: Lupus pneumonia, restrictive lung disease, vanishing lung syndrome
Renal: Glomerulonephritis, decreased GFR, risk of renal failure
Systemic Lupus ErythematosusS/S
GI/Liver:
NM:
Hematology:
Skin:
GI/Liver: ABD pain, pancreatitis, elevated liver enzymes
NM: Skeletal muscle weakness
Hematology: Thromboembolism, thrombocytopenia, hemolytic anemia,
Skin: Butterfly-shaped malar rash, discoid lesions, alopecia
Systemic Lupus ErythematosusTreatment
NSAIDs or ASA - will treat arthritis and serositis
Anti-malarial (Hydroxychloroquine and quinacrine) - will treat arthritis and dermatological manifestations
Corticosteroids- By reducing inflammation, corticosteroids can help to improve the production and survival of platelets in the bone marrow.
Immunosuppressants will be used if steroids are not effective (Methotrexate, azathioprine)
Systemic Lupus ErythematosusAnesthesia Management
Based upon manifestations and organ dysfunction
Airway: recurrent laryngeal nerve palsy (common), cricoarytenoid arthritis
Stress dose of corticosteroids
What is recurrent laryngeal nerve palsy?
How does lupus cause recurrent laryngeal nerve palsy?
A condition where the nerve that controls the muscles of the larynx (voice box) is damaged, which can lead to problems with speaking, breathing, and swallowing.
In lupus, the immune system attacks healthy tissues, including the nerves. This can lead to inflammation of the laryngeal nerve or blood vessels, which can cause them to become compressed or damaged.