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Connect-Tissue-200002 - Connective Tissue Diseases Edward D...

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Connective Tissue Diseases Edward D. Buckingham, MD Byron J. Bailey, MD
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Introduction collagen vascular diseases, autoimmune diseases knowledge of immune system difficult to diagnose nonspecific symptoms tend to overlap common histiologic feature inflammatory damage CT and blood vessels fibrinoid material deposition
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Immune System lymphoid stem cell B-cell T-cell macrophage or monocyte NK cells
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Connective Tissue Diseases abnormal interaction three theories sequestered antigen exogenous antigen altered antigen
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Systemic Lupus Erythematosus General autoimmune multisystem disease prevalence 1 in 2,000 9 to 1; female to male (1 in 700) peak age 15-25 immune complex deposition photosensitive skin eruptions, serositis, pneumonitis, myocarditis, nephritis, CNS involvement
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Systemic Lupus Erythematosus specific labs - native(Double stranded) DNA, SM antigen lupus like reaction(procainamid e, hydralazine, ect) LE cells
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SLE - Diagnostic Criteria
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SLE Head and Neck Manifestations Malar rash first sign in 50% Erythematous maculopapular eruption after sun exposure Oral ulceration
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SLE
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SLE Head and Neck Manifestations 3-5% perforated nasal septum larynx and trachea involvement rare TVC thickening, paralysis, cricoarytenoid arthritis, subglottic stenosis acute parotid enlargement 10%
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SLE Head and Neck Manifestations xerostomia occas neuropathy 15% discoid lupus
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SLE Treatment avoid sun exposure NSAIDS topical and systemic steroids antimalarials low-dose methotrexate azothioprine, cyclophosphamide restricted symptomatic treatment saliva substitutes, Klack’s solution postprandial rinses with H2O2 and H2O
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Rheumatoid Arthritis General synovial tissue involvement symmetric peripheral joints (hands, feet, wrists)
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RA General nonarticular muscular structures tendon, ligament, fascia systemic disease occas. vasculitis, pulmonary fibrosis pathogenosis inflammatory cell infiltrates synovial proliferation HLA Dw4
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RA General prevalence 1% of population 2-3 times F>M 4th and 5th decade signs and symptoms morning stiffness lasting greater than 30 min sub-Q rheumatoid nodules synovial fluid inflammation 2K - 75K 50% PMN’s
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RA General diagnosis based on clinical grounds labs RF pos. in 12 months 90% RA associated nuclear antigen (RANA) anti-RA-33
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RA Head and Neck Manifestations TM joint 55% symptomatic 70% incidence on X- ray juvenile RA - micrognathia
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RA Head and Neck Manifestations cricoarytenoid joint most common cause of arthritis 30% patients hoarse 86% pathologic involvement exertional dyspnea, ear pain, globus hoarseness rheumatoid nodules, recurrent nerve involvement stridor local/systemic steroids poss. Tracheotomy
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RA Head and Neck Manifestations CHL
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