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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Microangiopathies In Systemic ... Lupus Erythematosus ... #Lupus #Erythematosus ... #SLE #hematology ... #rheumatology #
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... Initial screening test ... block • Sjogren syndrome ... severe disease Treatment ... Summary #diagnosis #rheumatology
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
lupus erythematosus ... , Drug-induced Lupus ... lupus erythematosus ... SLE), Sjogren's Syndrome ... ANA #patterns #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lupus erythematosus ... Liver function tests ... Treatment: • Corticosteroids ... Diagnosis #Management #Hematology ... #Rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... • Immunologic Workup ... life-threatening • Treatment ... #Systemic #Erythematosus ... Management #Summary #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Manifestations of Systemic ... Demyelinating Syndrome ... Puncture, EEG Treatment ... RheumOnePagers) #Systemic ... CNS #neurology #rheumatology
Pancytopenia - Differential Diagnosis

Congenital Diseases:
• GATA2 deficiency
• Fanconi’s anemia
• Wiskott-Aldrich syndrome
• Ribosomopathy or telomeropathy

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Wiskott-Aldrich syndrome ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... Differential #Diagnosis #Hematology
Mucous Membrane Disorder - Differential Diagnosis Algorithm
Erosions/Ulcers/Blisters
 - Primary Dermatologic Diseases
     •
Stevens-Johnson Syndrome ... necrolysis - Systemic ... Disease • Systemic ... lupus erythematosus ... • Behcet's syndrome
BEE Syndromes - Immune-mediated conditions affecting the brain, eye, and ear

Visual or auditory symptoms in conjunction
BEE Syndromes - ... central nervous system ... diagnosis and treatment ... • Sjögren syndrome ... • Systemic lupus
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Clinical Features of Systemic ... Lupus (SLE) General ... Demyelinating syndromes ... Thrombocytopenia #Lupus ... symptoms #diagnosis #rheumatology