Autoimmune Diseases Flashcards
Autoimmune Diseases
Organ-Specific Diseases Mediated by Antibodies
Autoimmune hemolytic anemia Autoimmune thrombocytopenia Autoimmune atrophic gastritis or perniculous anemia Myasthenia gravis Grave's disease Goodpasture syndrome
Autoimmune Diseases
Systemic Diseases Mediated by Antibodies
Systemic lupus erythematosus
Autoimmune Diseases
Organ-Specific Diseases Mediated by T Cells
Type 1 diabetes
Multiple sclerosis
Autoimmune Diseases
Systemic Diseases Mediated by T Cells
Rheumatoid arthritis
Systemic sclerosis (scleroderma)
Sjogren syndrome
Autoimmune Diseases
Organ-Specific Diseases Postulated to be Autoimmune
Inflammatory bowel diseases (Crohn’s disease or ulcerative colitis)
Primary biliary cholangitis
Autoimmune (chronic active) hepatitis
Autoimmune Diseases
Systemic Diseases Postulated to be Autoimmune
Polyarteritis nodosa
Inflammatory myopathies
Lupus
Systemic disease mediated by antibodies
Complex multi-systemic autoimmune disease characterized by presence autoreactive B & T cells & production broad heterogenous group auto-antibodies
Lupus Pathogenesis
Women 15-44yo
African American, Asian Americans, Hispanic/Latinos, Native Americans, or Pacific Islanders
50 genes associated w/ SLE
UV light, infection, virus, stress
Auto-antibodies production w/o regulation
Immune activation T & B cells hyperactive & innate immune systems programmed towards inflammation eventually leading to tissue damage & clinical disease manifestations
Lupus Diagnosis
CBC Antibody tests - ANA - Anti dsDNA antibody Complement test Blood clotting tests Urine test Biopsies
Lupus Classification
Malar rash Photosensitivity Oral or nasopharyngeal ulcers Discoid rash Renal disorders Serositis (pleurisy, pericarditis) Neurologic disorders Hematologic disorders Immunologic disorders Non-erosive arthritis at least 2 peripheral joints Presence antinuclear antibody (ANA)
Lupus Types
Systemic lupus erythematosus
Drug-induced lupus erythematosus (hydralazine, procainamide, isoniazid)
Cutaneous lupus erythematosus - caused by maternal antibodies, born w/ skin rash, liver problems, & low blood counts, congenital heart block
Neonatal lupus
SLE S/S
CNS - vasculitis, anxiety, depression, psychosis, seizures, stroke
CV - pericarditis, pericardial effusions, CHF, HTN, mitral/aortic regurgitation, conduction defects, thrombocytopenia, anemia, leukopenia, antiphospholipid syndrome (acquired hypercoagulability - embolism)
Pulmonary - pleural effusions, restrictive disease, atelectasis
Airway - mucosal ulceration, cricoarytenoid arthritis, recurrent laryngeal nerve palsy
Renal - glomerular nephritis, proteinuria, hypoalbuminemia, hematuria, renal failure
Musculoskeletal - arthritis, avascular necrosis d/t steroid use
Lupus Treatment
Tylenol NSAIDs Immunosuppressants Corticosteroid Antimalarial Anticoagulants Monoclonal antibodies Repository corticotropin injections
Lupus Anesthetic Implications
Pre-anesthesia evaluation Cricoarytenoid arthritis (airway implications) Recurrent laryngeal nerve palsy Preop testing - PFTs, echo, EKG, renal function, labs Corticosteroids stress dosing Discontinue anticoagulants Consult w/ rheumatologist Potential postop obstruction
Rheumatoid Arthritis
Onset 25-55yo
2-3x more prevalent in women
Environmental
Heredity (genetic factors 50%)
Viral/bacterial infection
Osteoarthritis
Degenerative disease Morning stiffness lasting <30min Heberden's nodes Asymmetrical Cartilage loss
Rheumatoid Arthritis S/S
Autoimmune disease Morning stiffness lasting >30min Extra-articular involvement Symmetrical - vary in severity (S/S come & go) Inflamed synovium
Smaller joints involved 1st (hands, feet, wrists) & progresses to ankles, hips, shoulders
Inflamed synovial joint membrane
Rapid division & cell growth w/in joint
Pro-inflammatory cytokines
Release osteolytic enzymes, collagenases, proteases
Nerve entrapment - carpal tunnel syndrome
TMJ - synovitis 45-75% involvement
Atlantoaxial Instability
C1-C2 (atlas-axis) involvement
Atlanto-odontoid separation → neurologic damage
Atlantoaxial subluxation → impairs vertebral artery blood flow
Cricoarytenoid Joint
26-86% patients w/ severe RA
Vocal cord nodules or polyps
Present w/o clinical symptoms
Hoarseness
Pain w/ swallowing
Stridor
Dyspnea
RA Treatment
Disease modifying anti-rheumatic drugs DMARDS Non-biologic Anti-metabolite - Methotrexate (Rheumatrex) - Sulfasalazine - Azathioprine Biologic TNF inhibitors - Etanercept (Enbrel) - Adalimumab (Humira) - Infliximab (Remicade) Interleukin-1 receptor agonists - Leflunomide (Arava) Anti-CD20 monoclonal antibody - Rituximab (Rituxan)
RA Anesthetic Considerations
Medications - NSAIDs & corticosteroids
Airway - TMJ, cervical spine, cricoarytenoid joint
Positioning - padding to prevent further joint damage
Spinal anesthesia - sensory & ↓CSF
Scleroderma
“Hard skin”
Inflammation
Vascular sclerosis
Skin & viscera fibrosis
Injury to vascular endothelium results in protein leakage into interstitial space
Collagen production not slowed down & deposited throughout body
Scleroderma Causes
Unknown Collagen vascular disease & autoimmune characteristics Onset 20-40yo Women Accelerated by pregnancy CREST syndrome - Calcinoses Ca2+ deposits - Raynaud's phenomenon - Esophageal hypomotility - Sclerodactyly (thickened/tight skin) - Telangiectasia (dilated capillaries)
Scleroderma S/S
CNS - peripheral & cranial neuropathies d/t nerve compression by thickened connective tissue, trigeminal neuralgia, dry eyes
Musculoskeletal - thickened skin, diffuse edema, contractures, skeletal muscle myopathy, arthritis & limited joint mobility, avascular necrosis
CV - coronary arteries & conduction system sclerosis, replace cardiac tissues w/ fibrous tissue, systemic & pulmonary HTN, pericarditis & pericardial effusion, intermittent vasospasm, Raynaud’s
Respiratory - diffuse interstitial pulmonary fibrosis 80% patients, arterial hypoxemia 2° ↓diffusion capacity, ↓pulmonary compliance
GI - dry oral mucosa, progressive GI tract fibrosis, dysphagia, hypomotility, ↓LES tone → aspiration risk, malabsorption vitamin K deficiency (coagulopathies)
Renal - ↓renal blood flow → systemic HTN, renal artery stenosis d/t arteriolar intimal proliferation
Scleroderma Anesthetic Considerations
Fibrosis potential to lead to limited mouth opening & difficult intubation Dermal thickening → difficult IV access Pulmonary HTN ↓pulmonary compliance ↓O2 diffusion Chronic system HTN LES hypotonia → aspiration risk Sensitive to respiratory depressants Regional anesthesia challenging d/t contractures & ↓joint mobility Protect eyes from corneal abrasion Renal dysfunction & drug elimination
