Collagen Vascular DiseasesCollagen Vascular Diseases The four most common disorders of this group are rheumatoid arthritis, systemic lupus erythematosus,

Collagen Disorders

Michael H. Wilhelm, CRNA, APRN

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.


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.


Peripheral Joints Hands, feet, wrist (most common)

Nonarticular muscular structures Tendons, ligaments and fascia

Systemic Involvement Blood Vessels, heart, lungs, etc.


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


Manifestations continued: Liver : Hepatitis Blood: Anemia, leukopenia


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


Joint Involvement Morning Stiffness – joints of the hand, feet, wrist and knees Nearly every joint is effected

Thoracic, lumbar, sacral spine almost always spared Cervical spine involvement is frequent

Atlantoaxial subluxation (partial or complete dislocation of the 1st and 2nd cervical vertebrae)

Cricoarytenoid arthritus Edema of the arytenoids, upper airway obstruction

Tempromandibular Joint Limitations in mandibular motion

Joints of the Larynx Limitations of the vocal cord movement, edema


Systemic Involvement Vascular

Vasculitis – peripheries Aortitus – dilation of the aortic root resulting in aortic regurgitation

Cardiovascular Pericarditus, endocarditis, LV Failure (CHF), valve fibrosis, arteritis

involving coronary arteries, myocardial infarction Pulmonary

Pleural effusions, fibrosis Costochondral involvement

Decreases lung volumes Decreases vital capacity Leads to V/Q Mismatch decreasing arterial oxygenation


Neuromuscular Loss of strength in muscle adjacent to joints Neuropathy resulting from nerve compression

Hematologic Anemia

Liver/Kidney Rarely problems occur




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.


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


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.


A diagnosis of SLE is likely when patients have three of four manifestations Antinuclear

antibodies Characteristic rash Thrombocytopenia Serositis Nephritis

Common Presenting Features Fever Malaise Joint Pain Myalgias Fatigue


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 Involvement Cardiovascular

Pericarditis, pericardial effusions, friction rub, tachycardia, CHF, LV dysfunction, valve abnormalities (aortic, mitral)

Pulmonary 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


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


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 I

MMUNOLOGY

OMG


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

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 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) 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 Careful airway management Minimize pain/stress Positioning Cardiac Workup Careful Ventilation Careful blood pressure monitoring Intraoperative Medications

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.


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.


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.


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.

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Collagen Vascular DiseasesCollagen Vascular Diseases The four most common disorders of this group are rheumatoid arthritis, systemic lupus erythematosus,