Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Download Collagen Disorders
Document related concepts
Transcript
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 Morning Stiffness – joints of the hand, feet, wrist and knees Nearly every joint is effected Cervical spine involvement is frequent Edema of the arytenoids, upper airway obstruction Tempromandibular Joint Atlantoaxial subluxation (partial or complete dislocation of the 1st and 2nd cervical vertebrae) Cricoarytenoid arthritus Thoracic, lumbar, sacral spine almost always spared Limitations in mandibular motion Joints of the Larynx Limitations of the vocal cord movement, edema Rheumatoid Arthritis Systemic Involvement Vascular Cardiovascular 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 Pleural effusions, fibrosis Costochondral involvement 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 Antinuclear antibodies Characteristic rash Thrombocytopenia Serositis Nephritis Common Presenting Features 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 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 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 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. 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
