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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 ... #SLE #hematology ... #rheumatology # ... differential #diagnosis
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
(SLE), Sjogren's ... Syndrome (SS), ... (SLE), Systemic ... ANA #patterns #rheumatology ... #diagnosis #differential
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Lupus (SLE) General ... Demyelinating syndromes ... ) Blood (75%): Anemia ... #SLE #systemic ... #rheumatology
Lupus Classification
Spontaneous Lupus
 - Cutaneous Lupus Erythematosus (CLE)
 - Systemic Lupus Erythematosus 
Drug-Induced Lupus
 - Drug-Induced
Lupus Classification ... ) - Systemic Lupus ... #Classification ... #rheumatology # ... diagnosis #differential
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
deficiency • Fanconi’s anemia ... Wiskott-Aldrich syndrome ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... #Hematology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... block • Sjogren syndrome ... polymyositis overlap syndrome ... #Lupus #Erythematosus ... diagnosis #rheumatology
SLICC SLE Diagnostic Criteria - Systemic Lupus Erythematosus
Clinical and Immunologic Criteria

CLINICAL CRITERIA
 - ACUTE CUTANEOUS LUPUS
SLICC SLE Diagnostic ... Criteria - Systemic ... NEUROLOGIC - HEMOLYTIC ANEMIA ... #Diagnosis #Rheumatology ... #SLE #Lupus #Erythematosus
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
EULAR/ACR Classification ... #Criteria #SLE ... #Systemic #Lupus ... Erythematosus #diagnosis ... #rheumatology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... - Hemolytic anemia ... Lupusreference #SLE ... #Systemic #Lupus ... #differential #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lupus erythematosus ... [SLE], AOSD) • ... with leukopenia, anemia ... #Diagnosis #Management ... #Hematology #Rheumatology