Autoimmune, Musculoskeletal (2) Flashcards

1
Q

Examples of Autoimmune Disorders: Rheumatic

A

Rheumatic arthritis
Scleroderma
Sjögren’s syndrome
Systemic lupus erythematosus

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2
Q

Examples of Autoimmune Disorders: Gastrointestinal

A

Chronic active hepatitis
Crohn’s disease
Primary biliary cirrhosis
Ulcerative colitis

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3
Q

Examples of Autoimmune Disorders: Endocrine

A

Graves’ disease
Hashimoto’s thyroiditis
Type 1 Diabetes Mellitus

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4
Q

Examples of Autoimmune Disorders: Neurologic

A

Multiple Sclerosis
Myasthenia gravis

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5
Q

Examples of Autoimmune Disorders: Hematologic

A

Autoimmune hemolytic anemia
Idiopathic thrombocytopenia purpura
Pernicious anemia

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6
Q

Examples of Autoimmune Disorders: Renal

A

Goodpasture syndrome

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7
Q

Examples of Autoimmune Disorders: Multi-organ system

A

Ankylosing spondylitis
Polymyositis
Psoriasis
Sarcoidosis
Vasculitis

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8
Q

What is one of the major genes involved in autoimmune disorders

A

Human Leukocyte Antigens (HLA) - found on the surface of most cells
~ Primary Function: To serve as recognition molecules in the initiation of an immune response

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9
Q

On what Chromosome is HLA found?

A

Chromosome 6

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10
Q

Rheumatoid Arthritis: What is it?

A

Symmetrical polyarthropathy that affects weight bearing joints and proximal interphalangeal and metacarpophalangela joints.
~ Significan systemic involvement
~ Approximately 70% of cases are associated with HLA-DR4 subtype

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11
Q

Rheumatoid Arthritis: Anesthetic concerns

A

~ Cervical spine: Atlantoaxial instability
~ Airway
~ Involvement of other organs
~ Positioning - skeletal deformities
~ Effects of pharmacotherapy

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12
Q

Rheumatoid Arthritis: Atlantoaxial subluxation concerns

A

Head and neck manipulation during laryngoscopy and movement during transfer may cause neurological injury to patients with subluxation

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13
Q

Rheumatoid Arthritis: Atlantoaxial subluxation

A

Most common subtype - worsened by C1/C2 flexion - therefor direct laryngoscope should be tolerated

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14
Q

Rheumatoid Arthritis: Atlantoaxial subluxation measurments

A

Subluxation exists when the distance between the atlas and the odontoid peg (AADI) exceeds:
~ 3mm in adults
~ 5mm in younger patients
~ Anterior Atlanta-axial subluxation is worsened by neck flexion

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15
Q

Rheumatoid Arthritis: Atlantoaxial subluxation Classification and percentage of incidence

A

Anterior 80%
Posterior <5%
Vertical 10-20%
Lateral/rotatory 5-10%
Subaxial (below C2) Rare

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16
Q

Rheumatoid Arthritis: Cricoarytenoid arthritis description and symptoms

A

Glottic rheumatoid arthritis. The arytenoids and are epiglottic folds are swollen and the airway is narrow
~ Hoarseness
~ Pain on swallowing
~ Dyspnea
~ Stridor

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17
Q

Rheumatoid Arthritis: Airway - TMJ

A

Can cause mandibular deviation and irregular erosion of the TMJ joint

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18
Q

Rheumatoid Arthritis: Sjögren’s syndrome what is it?

A

Infiltration of the lachrymal, salivary and parotid glands with lymphocytes
~ Strong association with HLA
~ Acetylcholine activation does not lead to eccrine secretion

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19
Q

Rheumatoid Arthritis: Sjögren’s syndrome effects:

A

~ Lack of lacrimation
~ Chronic dry mouth
~ Poor dentition
~ Mouth opening

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20
Q

Rheumatoid Arthritis, other organ involvement: Cardiovascular

A

Pericardial effusions/tamponade, pericarditis/endocarditis, LV failure, Valvopathy (aortic insufficiency), CAD, Peripheral vasculitis/Raynaud’s phenomenon

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21
Q

Rheumatoid Arthritis, other organ involvement: Respiratory

A

Restrictive defect (fibrosis alveoli’s), Reduced chest wall compliance (costochondral disease), pleural effusions

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22
Q

Rheumatoid Arthritis, other organ involvement: Hematological

A

Normocytic normochromic anemia, iron deficiency anemia, (peptic ulceration and bleeding), Bone marrow depression

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23
Q

Rheumatoid Arthritis, other organ involvement: Hepatic and renal

A

Chronic renal failure from drug treatment (=25%), hepatomegaly, splenomegaly, decreased serum albumin

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24
Q

Rheumatoid Arthritis, other organ involvement: Neurological and ocular

A

Peripheral neuropathy, autonomic dysfunction, keratoconjunctivitis

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25
Rheumatoid Arthritis, pharmacotherapy: Corticosteroid
Administration during procedure: based on prehospital prescription and severity of surgical procedure ~ Adverse effects: Han, infection, electrolyte imbalance, weight gain, glucose intolerance, adrenal insufficiency, fragile skin
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Rheumatoid Arthritis, pharmacotherapy, Conventional DMARDs: Methotrexate
Administration during procedure: Continue ~ Adverse effects: Pulmonary toxicity, bone marrow suppression, hepatotoxicity, infection
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Rheumatoid Arthritis, pharmacotherapy, Conventional DMARDs: Hydroxychloroquine
Administration during procedure: Continue ~ Adverse effects: Retinal toxicity, cardiotoxicity, blood dyscrasia
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Rheumatoid Arthritis, pharmacotherapy, Conventional DMARDs: Sulfasalazine
Administration during procedure: Continue ~ Adverse effects: Central nervous system symptoms, granulocytopenia, hemolytic anemia
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Rheumatoid Arthritis, pharmacotherapy, Conventional DMARDs: Leflunomide
Administration during procedure: Continue ~ Adverse effects: Hepatotoxicity, myleosuppression infection, pulmonary toxicity
30
Rheumatoid Arthritis: testing, assessment, pre-procedure. Airway: cervical instability, narrow glottis, limited TMJ
~ Preop ROM assessment ~ Consider cervical collar placement, VL or awake FOB ~ Consider flex/ex/odontoid imaging
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Rheumatoid Arthritis: testing, assessment, pre-procedure. Cardiac: pericarditis, tamponade, conduction abnormalities, AI is common
~ ECG, CXR / ECHO
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Rheumatoid Arthritis: testing, assessment, pre-procedure. Pulmonary: fibrosis, restrictive lung disease (thoracic restriction)
PFTs: Spirometry and Flow-Volume loops
33
Rheumatoid Arthritis: testing, assessment, pre-procedure. Renal: chronic NSAID use (insufficiency)
~ U/A: Elevated Protein and Protein:Creatinine ratio. ~ CBC: Hypoalbuminemia, Pitting edema
34
Rheumatoid Arthritis: testing, assessment, pre-procedure. GI: chronic NSAID use (bleeding)
CBC: Hypoalbuminemia, H&H, Coags
35
Rheumatoid Arthritis: testing, assessment, pre-procedure. Immune: higher risk if on DMARDs
Continue conventional (non-biologics) D/C Biologics 7-10 days
36
Rheumatoid Arthritis: testing, assessment, pre-procedure. Intraop positioning
Document deformities / neuro deficits
37
Rheumatoid Arthritis: Regional anesthesia?
~ Central regional techniques when no significant thoracic, lumbar, and sacral spine involvement exists and coagulation studies WNL ~ Peripheral blocks: ok as long as coags WLN, consider ROM limitations ~ Chosen regional technique must outlast procedure!
38
Systemic Sclerosis characterized by:
Inflammation, vascular sclerosis, skin and visceral fibrosis ~ Vascular injury can cause protein leakage into the interstitial space ~ Disease can evolve into CREST syndrome
39
The fiver components of CREST Syndrome are:
C = Calcinosis: calcium salt deposits under the skin R = Raynaud's phenomenon: spasm of blood vessels of the fingers and toes when triggered by factors, such as cold, stress or illness E = Esophageal dysmotility: esophageal muscles fail to contract, and the esophagus does not properly deliver food and liquids into your stomach S = Sclerodactyliy: thickening of the skin of the digits, hands and feet T = Telangiectases: lesions formed by collections of dilated blood vessels. occur on the face, upper trunk, hands, mucosal surfaces (eg, lips) and through the GI tract
40
Systemic Sclerosis: testing and concerns
~ Airway: Microstomia. Cervical extension. STOP-BANG ~ Pulmonary hypertension / ILD: Cor-pulmonale cardiomyopathy (TTE), conduction abnormalities (ECG), DLCO, 6-minute walk ~Esophageal dysfunction: GERD - prophylaxis ~VTE risk: A-fib, CHG - prophylaxis
41
Systemic Sclerosis: Anesthetic concerns
~ Decreased mouth opening to be anticipated ~ Trauma during intubation may cause excessive bleeding due to telangiectasis ~ IV access may be difficult due to dermal thickening ~ Increased risk of aspiration - esophageal hypomotility
42
Systemic Lupus Erythematosus: What is it?
Multi system chronic inflammatory disorder characterized by antinuclear antibody production
43
Systemic Lupus Erythematosus: Occurrence and variation
~ Usually occurs in young women ~ Exacerbated by stress (infection, pregnancy, surgery) ~ Onset can be drug induced (most commonly by procainamide, hydralazine, isoniazid, D-penicillamine, and methyldopa ~ Drug induced forms most common in people characterized as slow acetylators (slower to metabolize these drugs) ~ Presence of nephritis and hypertension is associated with worse prognosis
44
Systemic Lupus Erythematosus: Cutaneous symptoms
~ Classic Butterfly molar rash often exacerbated by sunlight ~ Discoid lesions on the face, scalp, and upper trunk ~ Alopecia is common
45
Systemic Lupus Erythematosus: Anesthetic concerns / management
Identifying specific organ dysfunctions and the clinical picture will determine the appropriate anesthetic plan
46
Antiphospholipid Syndrome: what is it?
~ Acquired autoimmune disorder ~ Production of antibodies agains phospholipid binding proteins ~ Recurrent venous or arterial thrombosis and/or fetal loss ~ Prevalence of APS in SLE = 30-35%
47
Antiphospholipid Syndrome: Thrombotic events
Antiphospholipid syndrom destroys protein S and protein C which inactivate factor Va. Since factor Va is no longer being inhibited clotting continues (almost like DIC)
48
Ankylosing Spondylitis: what is it?
Chronic inflammatory disease of th axial spine ~ Hallmark feature is chronic back pain and progressive spine stiffness ~ Extraarticular manifestations of AS include inflammatory bowel disease, psoriasis, and uveitis
49
Ankylosing Spondylitis, Anesthetic concerns / management: Cardiovascular
Those with <4 METS are at greater cardiac risk
50
Ankylosing Spondylitis: Pulmonary Concerns:
Significant spinal deformity can result in restrictive respiratory physiology. Pleural thickening on CXR. An alternative diagnosis is pulmonary tuberculosis in the setting of immunosuppressant therapy
51
Ankylosing Spondylitis: Neuromuscular concerns
Thorough ROM physical examination to document any preoperative sensory or motor deficits, especially in patients undergoing spinal surgery. Notation of any motor weakness may affect the plan for intraoperative neruomonitoring and the anesthesia
52
Ankylosing Spondylitis: Renal Concerns
Long-term NSAID use. Interstitial nephritis, or chronic kidney dysfunction due to amyloidosis, IgA-nephropaty, and tubulointerstitial nephritis
53
Ankylosing Spondylitis: eye inflammation
40% - uveitis/ conjunctivitis causing pain, visual disturbance, and photophobia
54
Ankylosing Spondylitis: Cardiac lesions
Predominantly aortic valve insufficiency (40%), thoracic aortic involvement, and RBBB
55
Ankylosing Spondylitis: cricoarytenoid
Hoarseness from cricoarytenoid joint disease does occur however is rarely seen
56
Ankylosing Spondylitis: Enthesitis
inflammation of extra-axial tendons and ligaments (40%); most commonly tendoachilles
57
Ankylosing Spondylitis: Pulmonary features
Decreased compliance due to thoracic joint (spine and costochondral) involvement and fusion with restrictive physiology on pulmonary function tests (PFTs)
58
Ankylosing Spondylitis: Hip involvement
Hip involvement (33%) peripheral arthritis; usually monoarticular
59
Ankylosing Spondylitis: Dactylitis
Dactylitis (8%) - painful swelling of the fingers and toes heterotrophic ossification after joint surgeries is more common than in the general population
60
Ankylosing Spondylitis: Specific conditions of the spine
~ Kyphosis: accentuated posterior curvature of the spine ~ Scoliosis: lateral curvature of the spine - Cobb angle more than 10 degrees ~ Kyphoscoliosis: concomitant kyphosis and scoliosis resulting in a lateral bending and rotation of the vertebral column
61
Ankylosing Spondylitis: Cobb method
The Cobb method of measuring scoliosis curves - The most tilted vertebrae above and below the curve are identified. Horizontal lines are drawn parallel with the plane of each vertebrae. Perpendicular lines intersecting those horizontal lines interest at the angle of curvature. (Greater than 50 degrees = Surgery)
62
Ankylosing Spondylitis: Kyphoscolisosis classification - Idiopathic scoliosis
~ Idiopathic scoliosis: Diagnosis of exclusion. 80% of all cases
63
Ankylosing Spondylitis: Kyphoscolisosis classification - Congenital scoliosis
~ Congenital scoliosis: Embryological malformation of one or more vertebrae. The abnormalities cause curvature and other deformities of the spine because on area of the spinal column lengthens at a slower rate than the rest. Because these abnormalities are present at birth, it is usually detected at a younger age and surgical treatment is avoided
64
Ankylosing Spondylitis: Kyphoscolisosis classification - Neuromuscular scoliosis
~ Neuromuscular scoliosis: Scoliosis secondary to neurological muscular diseases. (cerebral palsy, poliomyelitis, meningomyelocele, syringomyelia spinal cord trauma, muscular dystrophy, spinal muscular atrophy, and spina bifida). Progresses more rapidly than idiopathic scoliosis and often requires surgical treatment
65
Kyphoscoliosis - anesthetic concerns
*** Curvature > 60 degrees (severe). Cardiac and respiratory likely
66
Ankylosing Spondylitis: Kyphoscolisosis
Anticipate substantial blood loss with spinal procedures: From surgical field, but occasionally from laceration of the great vessels. Large bore PIVs, fluid infusion devices and have banked PRBCs immediately available.
67
Ehlers-Danlos Syndrome: What is it?
Inherited connective-tissue (collagen) disorder that can affect the skin, joints, and blood vessels ~ Characterized by joint hyper mobility, cutaneous fragility, and hyperextensibility
68
Ehlers-Danlos Syndrome: cause
Due to abnormal production of pro collagen and collagen: there are 9 types but only one form (Type IV) is associated with an increased risk of death due to. the vascular component of the disease.
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Ehlers-Danlos Syndrome: Type IV most common issue
Most common issues with type IV is rupture of large blood vessels or disruption of the bowel
70
Ehlers-Danlos Syndrome: Signs and symptoms (all forms)
~ Joint hypermobility ~ Skin fragility or hyperplasticity ~ Spontaneous rupture of bowel, uterus, or major arteries ~ Premature labor and/or excessive bleeding with child delivery ~ Will often require tracheal dilation ~ Increased risk of pneumothorax ~ Occasionally MR and cardiac conduction abnormalities are seen
71
Ehlers-Danlos Syndrome: major diagnostic criteria of type IV
Thin translucent skin, arterial/intestinal/uterine fragility or rupture, extensive bruising, characteristic facial appearance. Sudden death of a close relative(s).
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Ehlers-Danlos Syndrome: type IV most concerning factors
Only form associated with increased risk of death due to vascular syndrome ~ GI tract, uterus, and vasculature are particularly well endowed with type IV collagen, which accounts for complications such as spontaneous rupture of the bowel, uterus, or major arteries
73
Ehlers-Danlos Syndrome: anesthesia mgmt
~ Increased Cardiac conduction abnormalities ~ Increased bleeding (minimize DL trauma) ~ Avoid instrumentation of nose and esophagus and IM injections ~ Arterial or central venous lines may lead to extensive hematomas ~ IV infiltration may be difficult to recognize due to skin laxity ~ Regional Anestesia is relatively contraindicated
74
Marfans Syndrome: what is it?
Hereditary progressive genetic disease affecting connective tissues ~ Those with syndrome have long arms, legs, fingers and toes
75
Marfans Syndrome: what does it affect?
affects eyes, blood vessels, and skeleton
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Marfans Syndrome: What causes it?
Mutation or change in fibrillar-1 gene causes marfans syndrome ~ Fibrillin 1 a critical growth factor affecting growth and proliferation of cells
77
Marfans Syndrome: Relevant anesthetic considerations
~ Existence of thoracic aneurysm? ~ Hyperextensible joints (possible positioning implications) ~ High arched palate (airway implications) ~ Kyphoscoliosis (difficulties with neuraxial anesthesia)