Transcript Collagen Disorders

Collagen Vascular Diseases
 The four most common disorders of this group are
rheumatoid arthritis, systemic lupus
erythematosus, scleroderma, and polymyositis.
 The etiology of the collagen vascular diseases is
unknown, however the immune system has been
implicated.
 All of these diseases effect the joints, each has
systemic effects as well.
Rheumatoid Arthritis
 Pathophysiology
 Rheumatoid arthritis begins with cellular
hyperplasia of the synovium, followed by
invasion of the synovium by lymphocytes,
plasma cells, and fibroblasts. Ultimately, the
cartilage and articular surfaces are destroyed.
 The cause of Rheuatoid Arthritis is unknown,
but rheumatoid factor (an antiimmunoglobulin
antibody) is present in 90% of patients
 The hands and wrist are the first to be effected,
especially the metacarpophalangeal and
proximal interphalangeal joints.
Rheumatoid Arthritis
 Compression of lower extremity peripheral nerves can
produce paresis and sensory loss over the leg.
 Spinal cord compression does not correlate with
patient’s symptoms, and asymptomatic patients may
have a high degree of cord compression.
 Rheumatoid arthritis affects the joints of the larynx,
cervical spine, and temporomandibular joint.
Rheumatoid Arthritis
 Peripheral Joints
 Hands, feet, wrist (most common)
 Nonarticular muscular structures
 Tendons, ligaments and fascia
 Systemic Involvement
 Blood Vessels, heart, lungs, etc.
Rheumatoid Arthritis
 Manifestations involving other systems:
 Skin : Raynaud’s, Digital necrosis
 Eyes : Scleritis, corneal ulceration
 Lung : Pleural effusion, pulmonary effusion
 Heart : Pericarditis, cardiac tamponade,
coronary arteritis, aortic insufficiency
 Kidney : Interstitial fibrosis,
glomerulonephritis, amyloid deposition
 PNS : Compression syndromes,
mononeuritis
Rheumatoid Arthritis
 Manifestations continued:
 Liver : Hepatitis
 Blood: Anemia, leukopenia
Rheumatoid Arthritis
 About 6 million American have Rheumatoid Arthritis
 75% of them are women
 Can occur at any age
 Women: between ages 35-50
 Men: somewhat later
Rheumatoid Arthritis
 Joint Involvement
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Morning Stiffness – joints of the hand, feet, wrist and knees
Nearly every joint is effected
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Cervical spine involvement is frequent
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Edema of the arytenoids, upper airway obstruction
Tempromandibular Joint
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Atlantoaxial subluxation (partial or complete dislocation of the 1st and
2nd cervical vertebrae)
Cricoarytenoid arthritus
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Thoracic, lumbar, sacral spine almost always spared
Limitations in mandibular motion
Joints of the Larynx
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Limitations of the vocal cord movement, edema
Rheumatoid Arthritis
 Systemic Involvement
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Vascular
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Cardiovascular
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Vasculitis – peripheries
Aortitus – dilation of the aortic root resulting in aortic regurgitation
Pericarditus, endocarditis, LV Failure (CHF), valve fibrosis, arteritis
involving coronary arteries, myocardial infarction
Pulmonary
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Pleural effusions, fibrosis
Costochondral involvement
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Decreases lung volumes
Decreases vital capacity
Leads to V/Q Mismatch decreasing arterial oxygenation
Rheumatoid Arthritis
 Neuromuscular
 Loss of strength in muscle adjacent to joints
 Neuropathy resulting from nerve compression
 Hematologic
 Anemia
 Liver/Kidney
 Rarely problems occur
Rheumatoid Arthritis
Rheumatoid Arthritis
Rheumatoid Arthritis
 Treatment:
 Analgesics, NSAIDS,Methotrexate, COX-2
inhibitors, and corticosteroids.
 Many of these dugs cause anemia,
thrombocytopenia, and hepatitis.
 Steroids are reserved for those patients who
fail to respond to first line drugs such as
Methotrexate due to the long term effects of
taking steroids.
 Surgical procedures such as synovectomy,
tenolysis, and joint replacement.
Rheumatoid Arthritis
 Management of anesthesia:
 Baseline ABGs, PFTs, clotting times,
CBC, ECHO/Stress Test
 Assess cervical spine ROM and airway
 Assess steroid use
 Possible awake FOI, glidescope
 Proper positioning
 Careful airway management/ventilation
Rheumatoid Arthritis
 Management of anesthesia:
 Intubation: cricoarytenois arthritis may
be recognized by erythema and edema of
vocal cords which may decrease glottic
opening. A smaller ETT may be needed.
Exaggerated edema and stridor may
occur postextubation.
 Corticosteroids may be needed
perioperatively.
 Careful positioning.
Systemic Lupus Erythematosus
 An autoimmune disease in which patients produce
autoantibodies to DNA and also to RNA
polymerase, cardiolipin, and ribosomal
phosphoproteins.
 Clinical manifestations may be due to the
production of and autoantibody highly specific for
a single protein within an organ.
 Common manifestations are polyarthritis and
dermatitis.
Systemic Lupus Erythematosus
 A diagnosis of SLE is
likely when patients have
three of four
manifestations
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Antinuclear antibodies
Characteristic rash
Thrombocytopenia
Serositis
Nephritis
 Common Presenting
Features
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Fever
Malaise
Joint Pain
Myalgias
Fatigue
Systemic Lupus Erythematosus
 About 1.5 Million Americans have SLE
 Affects women 9 times more likely than men
 Can occur at any age
 Higher incident in African-American women
Systemic Lupus Erythematosus
 Systemic Involvement
 Cardiovascular
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Pericarditis, pericardial effusions, friction rub, tachycardia, CHF, LV
dysfunction, valve abnormalities (aortic, mitral)
 Pulmonary
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Pleural effusions, pneumonia, dry cough, dyspnea, pulmonary
HTN
PFTs show restrictive lung disease
Recurrent atelectasis can result in “shrinking lung syndrome”
Involvement of the larynx and the trachea is rare, but may include
true vocal fold thickening or paralysis, cricoarytenois arthritis, and
subglottic stenosis
Systemic Lupus Erythematosus
 CNS
 Cognitive dysfunction occurs in approximately 1/3 of the
patients
 Mood Disturbances
 Deterioration of intellectual capacity
 Atypical migraine headaches followed by visual disturbances
 Cutaneous
 Mala or “butterfly” rash is presenting sign in 50% of patients
 Rash on trunk (red scaly patches), alopecia
 Photosensitivity
Systemic Lupus Erythematosus
 Neuromuscular
 Loss of strength in muscle adjacent to joints
 Neuropathy resulting from nerve compression
 Hematologic
 Anemia, Thrombocytopenia, leukopenia, Prolonged PT and
PTT
 Liver
 Autoimmune Hepatitis in severe cases
 Kidney
 Glomerulonephritis, proteinuria, hypoalbuminemia, renal
failure
Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
 Management of Anesthesia:
 Careful pre-operative assessment including CXR, echo,
renal function, liver function, and PFT’s.
 Airway Assessment
 Proper Positions/Peripheral Neuropathy
 Careful muscle relaxation titration
 Arthritic involvement is rare in the cervical spine.
 If postextubation laryngeal edema or stridor occur IV
administration of corticosteroids is effective.
 Patients receiving corticosteroids may require
intraoperative steroids.
Scleroderma
 A chronic autoimmune disease of the connective tissue
 Characterized by the formation of scar tissue (fibrosis) in
the skin and organs of the body and presence of
autoantibodies
 The cause of Scleroderma is unknown, but the disease
process has the characteristics of both a collagen disease
and an autoimmune process
 Progressive fibrosis, resulting form increases collagen
deposits in the interstitium and intima of small arteries and
connective tissue of involved organs is the pathologic hall
mark of the disease
Scleroderma
 Affects approximately 300,000 people in the US
 Affects women 4 times more likely than men
 Juvenile scleroderma affects approximately 7000 children in
the US
 Choctaw Native Americans have the highest reported
prevalence
 Men and African-American women have the worse
prognosis
Scleroderma
 Limited form of Scleroderma tends to be confined to the skin,
fingers and face
 Often referred to as “CREST” Syndrome
 C – Calcinosis – Calcium deposits in the skin
 R – Raynaud’s Phenomenon – Spasms of tiny blood vessels in
response to cold or stress
 E – Esophagus Dysfunction – Acid reflux and decreased motility
of the esophagus
 S – Sclerodactyly – Thickening and tightening of the skin on the
fingers and hands
 T – Telangiectasis – Dilation of capillaries causing red marks on
surface of the skin
Scleroderma
 Systemic Involvement
 Skin/Musculoskeletal
 Inflammation and taut skin leading to decreased ROM of
the fingers, toes and jaw
 Skeletal muscle myopathy leading to muscle weakness
 Cardiovascular
 Sclerosis of coronary arteries, fibrous tissue replaces
cardiac muscle, systemic and pulmonary HTN
 Dysrhythmias, cardiac conduction abnormalities, CHF,
pericarditis, pericardial effusion
Scleroderma
 Pulmonary
 Interstitial fibrosis, pulmonary HTN, decreases
inspiratory capacity, increases residual volume, chest wall
restriction
 GI
 GI fibrosis, hypomotility of the esophagus and small
intestine, decreased lower esophageal sphincter tone,
reflux esophagitis
 Renal
 Renal artery obstruction, decreased renal blood flow,
systemic HTN, renal failure
Scleroderma
Scleroderma
 Management of anesthesia:
 Baseline ABGs, PFTs, CXR, EKG, Room
Air Sat
 Full stomach precautions
 Managing BP/Fluid Mangement
 Avoid hypothermia
 Careful airway management/ventilation
 Avoid hypoxemia and respiratory acidosis
 Possible awake FOI, glidescope
Scleroderma
 Regional Anesthesia
 Advantages
 Post operative analgesia
 Peripheral vasodilation
 Decreased risk of post operative ventilation support
 Disadvantages
 Regional Anesthesia may be technically difficult due to
the taut skin and joint changes
Scleroderma
 Management of Anesthesia:
 Ventilation with increased FiO2 is required
 Invasive cardiac monitoring due to
exaggerated responses to inhaled
anesthetics.
 Difficult venous access.
 Muscle involvement may increase sensitivity
to muscle relaxants (use short acting).
 Regional anesthetics may be prolonged.
 Avoid stellate ganglion block.
Marfan’s Syndrome
 Autosomal dominant, multisystem, fibrous connective
tissue disorder
 Affects blood vessel walls, tendons, ligaments,
cartilage, heart walls/valves, aorta, and other
structures
 Characterized by disproportionately long limbs, long
thin fingers, a typically tall stature and a predisposition
to cardiovascular abnormalities
Marfan’s Syndrome
 Approximately 200,000 people in the US
 Each parent with the condition has a 50% chance of
passing onto offspring
 Men and women are equally likely to have disease
 Caused by a mutation of a gene on Chromosome 15
Marfan’s Syndrome
 Systemic Involvement
 Musculoskeletal
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Grows to about average height
Arachnodactyly (long slender limbs, fingers and toes)
Scoliosis, thoracic lordosis
Pectus excavatum or pectus cairnatum
High palates and jaws
 Cardiovascular
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Dilated aorta, risk of ruptured aortic aneurysm
Prolapse of the mitral or aortic valves
Palpitations, tachydysrhythmia
Raynaud’s Phenomenon
Marfan’s Syndrome
 Pulmonary
 Spontaneous Pneumothorax
 Sleep Apnea
 Obstructive Lung Disease
 CNS
 Dural ectasia (weaking of the connective tissue of the dural
sac)
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Lower back pain, leg pain, abdominal pain, neuropathy,
headache
 Eye
 Retinal detachment, glaucoma, lens discoloration, myopia,
corneal flatness
Marfan’s Syndrome
Marfan’s Syndrome
 Anesthesia Management
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Careful airway management
Minimize pain/stress
Positioning
Cardiac Workup
Careful Ventilation
Careful blood pressure monitoring
Intraoperative Medications
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Beta Blockers
ACE Inhibitors
ARBs
Antibiotics
Epidermolysis Bullosa
 A rare skin disease which can be inherited or
acquired. The acquired forms are autoimmune.
 The end result is loss or absence of normal
intercellular bridges and separation of skin layers.
The separation of skin layers results in intradermal
fluid accumulation and bullae formation.
 Minor skin trauma produces skin blisters.
Epidermolysis Bullosa
 Involves fingers and toes mostly.
 Esophageal involvement is common, resulting in
dysphasia and esophageal strictures
 Anemia and hypoalbuminemia lead to increased
infection.
 Glomerulonephritis secondary to strep infection.
 Patients rarely survive over 30 years.
Epidermolysis Bullosa
 Therapy is rather unsuccessful and steroids
tend not to work.
 Management of Anesthesia:
 Avoid trauma to the skin and mucous membranes
 Trauma from tape, BP cuffs, tourniquets, and EKG pads
can cause bullae.
 Pad BP cuffs
 Lubrication of face mask
 Avoid upper airways.
Epidermolysis Bullosa
 Management of anesthesia:
 Lubricate laryngoscope to reduce friction.
 Scarring of the oral cavity can produce immobility
of the tongue, therefore consider awake fiberoptic.
 Avoid esophageal stethoscopes.
 Ketamine is a good choice due to most surgeries
are superficial and to the extremities.
 Regional has been successful.
Pemphigus
 An autoimmune disease where auto antibodies are
highly specific and result in the excessive
production of proteolytic enzymes that disrupt cell
adhesion, leading to separation of epithelial layers.
 Pemphigus vulgaris is most common and most
clinically important to anesthesiologists because of
the occurrence of oral lesions.
 Lesions of the pharynx, larynx, esophagus,
conjunctiva, urethra, cervix, and anus also
develop.
Pemphigus
 Treatment with corticosteroids and
immunosupressants is highly effective.
 Management of Anesthesia:
 Corticosteroid supplementation.
 Upper airway techniques are similar to epidermolysis
bullosa.
 Ketamine and regional are also successful for these
patients.
 Look for side effects of treatment drugs with anesthesia.
Such as cyclophosphamide may prolong SCH and
mivacurium by inhibiting cholinesterase activity.
Questions