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Vasculitides

Rheumatology

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

 



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