Rheumatology
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GCA Epi | White; >50 yo; F:M = 2:1
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GCA Labs | very high ESR/CRP, anemia, low albumin
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GCA Complications: | sudden, irreversible blindness if left untreated
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PMR sx: | pain & stiffness pelvic / shoulder girdles; NOT weakness; Severe AM stiffness (obstructs function); ESR / CRP high (not so high as in GCA)
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PMR tx | Mod or LD pred; taper slowly (1-3 yr); PMR response to pred is diagnostic / dramatic
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There is an association between PMR and: | GCA (20%)
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PMR sx: | pain & stiffness pelvic / shoulder girdles; NOT weakness; Severe A.M. stiffness (obstructs function); ESR / CRP high (not so high as in GCA)
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PMR & giant cell arteritis comorbid rate | 20%
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Small vessel vasculitis: palpable purpura | genl diagnostic of vasculitis; skin does not blanch when pressed; usu found below waist
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Small Vessel Vasculitis DDx | Wegener; HSP; Cryoglobulinemia (Hep C); Drug reaction; Meningococcemia
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Wegener: most often affects: | upper airway (saddle nose deformity)(suspect in refractory sinus prob); lungs (nodules/ hemorrhage); kidneys (acute glomnephritis)
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Wegener: dx | bx involved tissue (gold std)
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Wegener: Labs: | c-ANCA (anti-neutrophil cytoplasmic antibody) (AKA PR3-ANCA, reacts w/ proteinase-3); inflammation: high ESR, CRP, anemia
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Wegner: biopsy: | of most available tissue (esp lung or kidney)
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Wegener: Tx: | corticosteroids (HD initially); cyclophosphamide
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Giant cell arteritis Sx: | HA (new / different); Scalp tenderness (esp temporal art); Jaw claudication; Systemic illness (wt loss, fevers, malaise)
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polyarteritis nodosa S/S | Fever, wt loss, arthralgia, polyneurop, palpable purpura, liv reticularis; ARF?
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Giant cell arteritis: Tx | Tx whenever suspected; HD prednisone, then taper; temp art bx (ENT or Eye)
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Anti-Phospholipid Syndrome (APS) definition | syndrome of antibody-mediated hypercoagulability
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APS sx | recurrent thrombosis (art and/or venous); PG loss (early or late); lupus-like sx but fail to meet SLE dx criteria
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APS: primary = | APS alone
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APS: Secondary = | APS in addition to other autoimmune dz, usually lupus
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APS dx / testing: | Anti-cardiolipin Ab; Lupus anticoag; False pos RPR
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APS Tx: if thrombosis: | life-long anticoagulation: warfarin plus ASA 81mg qd
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APS Tx: if PG loss: | heparin & ASA 81mg during future PG
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APS diagnostic mgmt. must include: | Echocardiography to rule out vegetations
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HS purpura triggered by | GAS infxn or URI in kids (4-10 yo)
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Henoch Schonlein purpura presentation | Acute. Circular purpuric 2-3 cm lesions on LEs. Nonpitting edema on dorsal hands & feet. Colicky abd pain, melena, N/V. Arthritis. Self-limiting.
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HSP labs | Mild platelet elevation. Hemogram & coags normal.
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