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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 ... #Erythematosus ... #rheumatology # ... differential #diagnosis #causes
SLICC SLE Diagnostic Criteria - Systemic Lupus Erythematosus
Clinical and Immunologic Criteria

CLINICAL CRITERIA
 - ACUTE CUTANEOUS LUPUS
Erythematosus Clinical ... CRITERIA - ANA level ... Anti-dsDNA antibody level ... #Diagnosis #Rheumatology ... #SLE #Lupus #Erythematosus
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Malar rash 40% - Fever ... - Hemolytic anemia ... #Erythematosus ... #differential #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus ( ... autoantibodies that will cause ... Puncture, EEG Treatment ... #Erythematosus ... CNS #neurology #rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... Positive in 60-80% of cases ... life-threatening • Treatment ... #Systemic #Erythematosus ... Management #Summary #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
erythematosus ... erythematosus [ ... with leukopenia, anemia ... Treatment: • Corticosteroids ... #Rheumatology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Features of Systemic Lupus ... (SLE) General: Fever ... ) Blood (75%): Anemia ... SLE #systemic #erythematosus ... symptoms #diagnosis #rheumatology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
deficiency • Fanconi’s anemia ... Diseases: • Systemic lupus ... erythematosus ... Differential #Diagnosis #Hematology
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
weight loss, and fever ... Non-autoimmune rheumatologic ... lymphopenia, low PLT • Anemia ... to: Arthritis, Fever ... #Diagnosis #Rheumatology
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
Criteria for Systemic Lupus ... Erythematosus ... Constitutional domain: Fever ... #Erythematosus ... #diagnosis #rheumatology