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diagnosis management hematology activation behcet common cryofibrinogenemia cutaneous cvid differential hemophagocytic hlh hypogammaglobulinemia immunodeficiency immunology lymphohistiocytosis macrophage mas sapho signs
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
association • M > F Clinical ... ulceration • Ocular ... white matter, spinal ... (Clinical Dx). ... signs #symptoms #rheumatology
Vasculitis Involving the Skin - Differential Diagnosis Framework Primary: 50% Cutaneous Vasculitis: Leukocytoclastic Vasculitis Secondary: CTD: SLE, RA
GPA Cutaneous Single ... Vasculitis: • Behcet Disease ... • Cogan syndrome ... Vasculitis #skin #dermatology ... #rheumatology #
Cryofibrinogenemia Summary Cryofibrinogenemia Epidemiology: • 40-70 years with a modest female predominance Cryofibrinogenemia: • The precipitation of a
pulmonary emboli, and ocular ... Diagnosis: • + Clinical ... Antiphospholipid syndrome ... Cryofibrinogenemia #diagnosis #rheumatology ... #hematology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
to 8 years Clinical ... • Liver diseases ... : • Nephrotic syndrome ... (Epstein-Barr syndrome ... hypogammaglobulinemia #immunology #hematology
SAPHO Syndrome Summary What? SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
Rheumatic pain Clinical ... skeleton-sacroiliac joint and spine ... osteoarticular and skin disease ... #Rheumatology # ... diagnosis #management #Dermatology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Clinical Presentation ... Fever is the main clinical ... thrombocytopenia • Liver ... Diagnosis #Management #Hematology ... #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
: Overwhelming clinical ... syndrome associated ... age, however the disease ... lymph node, or liver ... management #treatment #hematology
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
Sjogren's Syndrome ... gland) biopsy • Ocular ... salivary ducts Clinical ... vasculitis: 10%-16% Liver ... #Rheumatology #
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