27 results
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Macrophage Activation Syndrome ... acquired forms of haemophagocytic ... A subset of hemophagocytic ... #Diagnosis #Management ... #Hematology #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... Nicolas Taar ... #HLH #Hemophagocytic ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome
Clinical Syndrome:
 • Common Clinical Features: alveolitis, ear and
Clinical Syndrome ... Features: alveolitis, ear ... thromboembolic disease ... myeloid cells NEJM ... Autoinflammatory #Somatic #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... Overwhelming clinical syndrome ... age, however the disease ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
with more severe disease ... block • Sjogren syndrome ... polymyositis overlap syndrome ... Summary #diagnosis #rheumatology ... #management
Susac Syndrome - Clinical Triad
 - Central nervous system dysfunction
 - Branch retinal artery occlusion
 -
Susac Syndrome - ... - Recurrent disease ... flares - 2-year ... Triad #Diagnosis #Management ... #Rheumatology
Behcet's Syndrome - Treatment
Ulcers:
 • Treatment: Topical steroids
 • Prevention: Colchicine
 • Azathioprine for refractory disease
Arthritis:
Behcet's Syndrome ... for refractory disease ... #Syndrome #Treatment ... #management #pharmacology ... #rheumatology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
Behçet's Syndrome ... tests in Behcet syndrome ... Giant retinal tears ... #diagnosis #management ... signs #symptoms #rheumatology
Acquired von Willebrand Syndrome - Diagnosis and Management
 • Autoantibodies to vWF: Lymphoproliferative disorders (e.g. lymphoma,
von Willebrand Syndrome ... Diagnosis and Management ... thrombocythemia), Autoimmune disease ... vonWillebrand #Syndrome ... #treatment #hematology
Extra-articular and Other Conditions Associated with Joint Pain

Eyes
  • Iritis or uveitis - Spondyloarthropathies, Sarcoidosis,
GI - Felty syndrome ... • Felty's syndrome ... Shrinking lung syndrome ... Inflammation of ear ... differential #diagnosis #rheumatology