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Vasculitides
Rheumatology
Question | Answer |
---|---|
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. |