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diagnosis treatment hematology differential erythematosus hemophagocytic hlh lupus lymphohistiocytosis sle summary systemic vasculitis acquired activation algorithm anemia anticoagulation autoinflammatory behcet
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome Clinical Syndrome: • Common Clinical Features: alveolitis, ear and
Clinical Syndrome ... Features: alveolitis, ear ... thromboembolic disease ... myeloid cells NEJM ... Autoinflammatory #Somatic #rheumatology
Systemic Lupus Erythematosus - Summary Antinuclear (ANA) - 95% - Initial screening test Anti-dsDNA - 50% • Associated
with more severe disease ... block • Sjogren syndrome ... polymyositis overlap syndrome ... Summary #diagnosis #rheumatology ... #management
Behcet's Syndrome - Treatment Ulcers: • Treatment: Topical steroids • Prevention: Colchicine • Azathioprine for refractory disease Arthritis:
Behcet's Syndrome ... for refractory disease ... #Syndrome #Treatment ... #management #pharmacology ... #rheumatology
Susac Syndrome - Clinical Triad - Central nervous system dysfunction - Branch retinal artery occlusion -
Susac Syndrome - ... - Recurrent disease ... flares - 2-year ... Triad #Diagnosis #Management ... #Rheumatology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
Behçet's Syndrome ... tests in Behcet syndrome ... Giant retinal tears ... #diagnosis #management ... signs #symptoms #rheumatology
Acquired von Willebrand Syndrome - Diagnosis and Management • Autoantibodies to vWF: Lymphoproliferative disorders (e.g. lymphoma,
von Willebrand Syndrome ... Diagnosis and Management ... thrombocythemia), Autoimmune disease ... vonWillebrand #Syndrome ... #treatment #hematology
Extra-articular and Other Conditions Associated with Joint Pain Eyes • Iritis or uveitis - Spondyloarthropathies, Sarcoidosis,
GI - Felty syndrome ... • Felty's syndrome ... Shrinking lung syndrome ... Inflammation of ear ... differential #diagnosis #rheumatology
Iron Deficiency Anemia - Differential Diagnosis Algorithm Labs: Ferritin, iron, transferrin, TIBC, TSAT, sTfR • Blood loss:
Gastrectomy, WHDA syndrome ... Structural: Celiac disease ... diet, Alcoholism, Tea-toast ... Algorithm #ferritin #hematology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
and M panel Management ... : • Nephrotic syndrome ... (Epstein-Barr syndrome ... hypogammaglobulinemia #immunology #hematology ... #diagnosis #management
Cryofibrinogenemia Summary Cryofibrinogenemia Epidemiology: • 40-70 years with a modest female predominance Cryofibrinogenemia: • The precipitation of a
of 37°C to the near-freezing ... with autoimmune diseases ... Antiphospholipid syndrome ... Cryofibrinogenemia #diagnosis #rheumatology ... #hematology
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