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hemophagocytic hlh treatment behcet cryofibrinogenemia differential erythematosus lupus macrophage mas signs sjogrens summary symptoms systemic vasculitides vasculitis
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Macrophage Activation ... erythematosus [SLE ... #Syndrome #Diagnosis ... #Management #Hematology ... #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... excessive macrophage activation ... age, however the disease ... #diagnosis #management ... #treatment #hematology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
→Activation of CD8 ... : SLE+++, Adult-onset ... Still disease, ... #diagnosis #management ... treatment #summary #rheumatology
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Classification: ... Differential Diagnosis ... Non-autoimmune rheumatologic ... Chronic B-cell activation ... #Rheumatology
Cryofibrinogenemia Summary Cryofibrinogenemia Epidemiology: • 40-70 years with a modest female predominance Cryofibrinogenemia: • The precipitation of a
thrombophlebitis, pulmonary ... Cryofibrinogenemia Diagnosis ... Antiphospholipid syndrome ... #rheumatology # ... hematology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
matter, spinal cord ... Oral aphthae : SLE ... • Arthritis: SLE ... #diagnosis #management ... signs #symptoms #rheumatology
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
- Differential Diagnosis ... • Cogan Syndrome ... vasculitis, and SLE ... #rheumatology # ... classification
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
overlaps with RA, SLE ... : • 10%-15% Pulmonary ... Nephrogenic DI Hematologic ... #Rheumatology # ... Diagnosis #Management
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