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differential treatment hlh lymphohistiocytosis vasculitis autoimmune capillary clarksons classification common cryofibrinogenemia cvid hypogammaglobulinemia immunodeficiency immunology leak myopathy myositis sjogrens summary
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... B-LNH), solid cancers ... • Autoimmune diseases ... #management #treatment ... #summary #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 ... : HLH signs and ... #management #treatment ... #hematology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
Lymphoma or other cancers ... gastric and breast cancer ... DDX - Other causes ... hypogammaglobulinemia #immunology #hematology ... #diagnosis #management
Cryofibrinogenemia Summary Cryofibrinogenemia Epidemiology: • 40-70 years with a modest female predominance Cryofibrinogenemia: • The precipitation of a
thrombophlebitis, pulmonary ... Biopsy: Typical pathologic ... Antiphospholipid syndrome ... #rheumatology # ... hematology
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
- Differential Diagnosis ... tract, and often causes ... • Cogan Syndrome ... purpura: strong sign ... #rheumatology #
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
: • 10%-15% Pulmonary ... , INC risk PE, Pulmonary ... Nephrogenic DI Hematologic ... #Rheumatology # ... Diagnosis #Management
Vasculitis - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions • Mononeuritis multiplex • Hematuria, proteinuria,
- Differential Diagnosis ... Vasculitis Differential Diagnosis ... purpura: Strong sign ... tract, and often causes ... #Rheumatology
Clarkson’s Disease - Capillary leak syndrome Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
Capillary leak syndrome ... 150 published cases ... filling +++ (risk of pulmonary ... #diagnosis #management ... timeline #treatment #rheumatology
Autoimmune Myositis - Differential Diagnosis Framework Inflammatory Myopathies: Commonly symmetric proximal muscle weakness, no substantial muscle pain
• Anti-TIF1 (cancer-associated ... papules and Gottron sign ... ) • Shawl sign ... statins - probably caused ... Myositis #Myopathy #rheumatology
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