15 results
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome
Clinical Syndrome:
 • Common Clinical Features: alveolitis, ear and
enzyme, X-linked, Autoinflammatory ... Features: alveolitis, ear ... myeloid cells NEJM ... #E1 #Xlinked #Autoinflammatory ... #Somatic #rheumatology
SAPHO Syndrome Summary

What?
SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
SAPHO syndrome is ... Etiology unknown: Autoinflammatory ... antibiotics with antiinflammatory ... #Rheumatology # ... diagnosis #management
Susac Syndrome - Clinical Triad
 - Central nervous system dysfunction
 - Branch retinal artery occlusion
 -
Susac Syndrome - ... disease flares - 2-year ... David #Susac #Syndrome ... Triad #Diagnosis #Management ... #Rheumatology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
Behçet's Syndrome ... Giant retinal tears ... #diagnosis #management ... #signs #symptoms ... #rheumatology #
Sarcoidosis - Diagnosis and Management Summary
Epidemiology
1) High incidence in Scandinavian countries (11-24 cases per 100,000 individuals
Diagnosis and Management ... Specific sarcoidosis syndromes ... • Heerfordt syndrome ... Sarcoidosis #Diagnosis #Management ... #Signs #Symptoms
Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
 • Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity
vs Tumor Lysis Syndrome ... hyperviscosity syndrome ... WBC >100k, + lab signs ... TLS #diagnosis #management ... #hematology
Giant cell arteritis (GCA)

Giant cell arteritis (GCA) definition: Most common systemic inflammatory vasculitis in older adults
systemic sx + signs ... in literature (NEJM ... cases (Cornelia NEJM ... them, but urgent rheumatology ... Symptoms #Diagnosis #Management
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... Diagnosis: HLH signs ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
VASCULITIS: • Behçet Syndrome ... • Cogan Syndrome ... well as inner ear ... purpura: strong sign ... differential #diagnosis #rheumatology
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
purpura: Strong sign ... Mucocutaneous lymph node syndrome ... vasculitis • Behcet syndrome ... vasculitis • Cogan syndrome ... Differential #Diagnosis #Rheumatology