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diagnosis treatment differential lupus sle erythematosus dermatology hemophagocytic lymphohistiocytosis vasculitis comparison activation antinxp2 antinxp2dm aortitis associations autoimmune behcet behcets blood
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
lymphohistiocytosis (HLH ... (CNS) dysfunction ... • HLH Labs: ... #Diagnosis #Management ... #Hematology #Rheumatology
Connective tissue disease-associated interstitial lung diseases LIP: lymphoid interstitial pneumonia. NSIP: nonspecific interstitial pneumonia. OP: organizing pneumonia.
SSc: systemic sclerosis ... PSS: Sjogren syndrome ... arthritis SLE : systemic ... Differential #Comparison #Table ... #Pulmonary #Rheumatology
Systemic Lupus Erythematosus - Summary Antinuclear (ANA) - 95% - Initial screening test Anti-dsDNA - 50% • Associated
- Summary Antinuclear ... block • Sjogren syndrome ... polymyositis overlap syndrome ... #diagnosis #rheumatology ... #management
Aortitis - Differential Diagnosis Framework Non-Infectious Aortitis: • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
arteritis, Cogan's Syndrome ... : IgG4-Related Disease ... Arthritis (RA), Systemic ... , Sarcoidosis, HLA-B27 ... Differential #Diagnosis #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
• Autoimmune diseases ... 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 ... : • Systemic steroids ... #Syndrome #Treatment ... #management #pharmacology ... #rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... Summary • Epidemiology ... Positive in 60-80% of cases ... Evolution: Chronic disease ... #Summary #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE) Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Demyelinating Syndrome ... Cerebrovascular Disease ... autoantibodies that will cause ... #neurology #rheumatology ... cerebritis #diagnosis #management
Hemophagocytic Lymphohistiocytosis (HLH) Treatment Primary HLH: - HLH-94 Protocol • Dexamethasone
(depending on CNS ... or autoimmune disease ... for refractory disease ... #Treatment #management ... #hematology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
Systemic disease ... common in Asia • HLA-B51 ... basal ganglia, CNS ... #diagnosis #management ... signs #symptoms #rheumatology
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