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

What?
A subset of
nervous system (CNS ... erythematosus [SLE ... Treatment: • Corticosteroids ... #Diagnosis #Management ... #Hematology #Rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
CNS Manifestations ... Demyelinating Syndrome ... Cerebrovascular Disease ... #rheumatology # ... cerebritis #diagnosis #management
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
Oral aphthae : SLE ... • Arthritis: SLE ... Arthritis, AS Treatment ... #diagnosis #management ... signs #symptoms #rheumatology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
block • Sjogren syndrome ... polymyositis overlap syndrome ... Treatment: • ... Summary #diagnosis #rheumatology ... #management
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
: SLE+++, Adult-onset ... Still disease, ... Lymphohistiocytosis #diagnosis #management ... #treatment #summary ... #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
(depending on CNS ... or autoimmune disease ... for refractory disease ... #Treatment #management ... #hematology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... age, however the disease ... • Bicytopenia Treatment ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Sjogren's Syndrome Overview

Epidemiology:
• F > M: 9:1
• 5-6th Decades (can be any age)

Autoimmune exocrinopathy multisystemic disease
overlaps with RA, SLE ... • 8%-27% for CNS ... Adenocarcinoma Treatment ... #Rheumatology # ... Diagnosis #Management
Immune Reconstitution Inflammatory Syndrome - Overview of IRIS

What Is IRIS?
	• A state of hyperinflammatory response that
Reconstitution Inflammatory Syndrome ... worsening of that disease ... pleural effusions, CNS ... meningitis • Guidelines ... Differential #Diagnosis #Management
TAFRO

TAFRO syndrome was first described in 2010, standing for:
- Thrombocytopenia
- Anasarca
- Fever
- Reticulin fibrosis
- Organomegaly

TAFRO syndrome
erythematosus (SLE ... histopathologic changes in LNs ... Hypoalbuminemia Treatment ... TAFRO #diagnosis #management ... #rheumatology #