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diagnosis erythematosus hematology hemophagocytic lupus lymphohistiocytosis summary systemic activation antinxp2 behcet behcets capillary cerebritis clarksons cns dermatology dermatomyositis leak macrophage
Systemic Lupus Erythematosus - Summary Antinuclear (ANA) - 95% - Initial screening test Anti-dsDNA - 50% • Associated
block • Sjogren syndrome ... polymyositis overlap syndrome ... Treatment: • ... Summary #diagnosis #rheumatology ... #management
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
: SLE+++, Adult-onset ... Still disease, ... Lymphohistiocytosis #diagnosis #management ... #treatment #summary ... #rheumatology
Behcet's Syndrome - Treatment Ulcers: • Treatment: Topical steroids • Prevention: Colchicine • Azathioprine for refractory disease Arthritis:
Behcet's Syndrome ... : • Treatment: ... #Syndrome #Treatment ... #management #pharmacology ... #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... age, however the disease ... • Bicytopenia Treatment ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
lymphohistiocytosis (HLH ... erythematosus [SLE ... Treatment: • Corticosteroids ... #Diagnosis #Management ... #Hematology #Rheumatology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
association • ... Oral aphthae : SLE ... Arthritis, AS Treatment ... #diagnosis #management ... signs #symptoms #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE) Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus (SLE ... Demyelinating Syndrome ... Cerebrovascular Disease ... CNS #neurology #rheumatology ... cerebritis #diagnosis #management
Clarkson’s Disease - Capillary leak syndrome Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
Capillary leak syndrome ... tissues: Myalgia, paresthesia ... edema, compartment syndrome ... #diagnosis #management ... #rheumatology
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
Sjogren's Syndrome ... overlaps with RA, SLE ... Adenocarcinoma Treatment ... #Rheumatology # ... Diagnosis #Management
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
had more severe disease ... positive patients Treatment ... three days #AntiNXP2DM ... #diagnosis #management ... #rheumatology #
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