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

What?
A subset of
• Systemic lupus ... nervous system (CNS ... • HLH Labs: ... #Diagnosis #Management ... #Hematology #Rheumatology
Connective tissue disease-associated interstitial lung diseases

LIP: lymphoid interstitial pneumonia. NSIP: nonspecific interstitial pneumonia. OP: organizing pneumonia.
interstitial lung diseases ... PSS: Sjogren syndrome ... SLE : systemic lupus ... Differential #Comparison #Table ... #Pulmonary #Rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Demyelinating Syndrome ... Cerebrovascular Disease ... autoantibodies that will cause ... thrombosis and/or blood-brain ... #neurology #rheumatology
Aortitis - Differential Diagnosis Framework
Non-Infectious Aortitis:
 • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
arteritis, Cogan's Syndrome ... : IgG4-Related Disease ... (RA), Systemic lupus ... , Sarcoidosis, HLA-B27 ... Differential #Diagnosis #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Phagocytosis of blood ... • Autoimmune diseases ... Adult-onset Still disease ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Non-autoimmune rheumatologic ... lower extremity edema ... proteinuria, dependent edema ... • May involve PNS ... Erythematosus #Diagnosis #Rheumatology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... ) - HELLP syndrome ... hemoglobinuria (PNH ... usually IgG) (e.g. lupus ... differential #diagnosis #hematology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
(depending on CNS ... or autoimmune disease ... for refractory disease ... #Treatment #management ... #hematology
Drug Induced Lupus vs SLE
Drug Induced Lupus (DIL):
 • Epidemiology: -10% of all lupus cases, drug-dependent,
Usual therapeutic management ... Evolution: Chronic disease ... sle #comparison #table ... #rheumatology # ... diagnosis #management
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
common in Asia • HLA-B51 ... basal ganglia, CNS ... meningitis), MCC CNS ... #diagnosis #management ... signs #symptoms #rheumatology