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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Erythematosus ... #Erythematosus ... #SLE #hematology ... #rheumatology # ... differential #diagnosis #causes
Hemolysis - Diagnostic Algorithm and Causes

Increased red cell production: 
 • Increased Reticulocytosis/polychromasia 
 • Erythroid
Algorithm and Causes ... #Hemolytic #Anemia ... #Algorithm #Differential ... #Causes #Workup ... #Hematology #Diagnosis
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
erythematosus ( ... erythematosus ( ... SLE), Sjogren's ... #Antinuclear #Antibodies ... ANA #patterns #rheumatology
Causes of Anemia with Normal Mean Corpuscular Volume (MCV) - Differential Diagnosis Algorithm
Decreased WBCs
 - Decreased/NormaI
Causes of Anemia ... Differential Diagnosis Algorithm ... RA, SLE, PRV, Chronic ... Bleed • Hemolysis ... #Causes #Hematology
SLICC SLE Diagnostic Criteria - Systemic Lupus Erythematosus
Clinical and Immunologic Criteria

CLINICAL CRITERIA
 - ACUTE CUTANEOUS LUPUS
SLICC SLE Diagnostic ... Erythematosus Clinical ... ANEMIA - LEUKOPENIA ... #Rheumatology # ... SLE #Lupus #Erythematosus
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Lymphadenopathies 7% - Hemolytic ... anemia 4% - Myositis ... Auto-Antibodies ... #differential #rheumatology
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
pericarditis • Hematologic ... Thrombocytopenia, Autoimmune hemolysis ... Highly specific antibodies ... Classification #Criteria #SLE ... #diagnosis #rheumatology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... anemia): ... usually IgG) (e.g. lupus ... differential #diagnosis #hematology ... #anemia
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
Differential Diagnosis Algorithm ... Inflammatory • Systemic Lupus ... Erythematosus ... Serum Sickness Hemolytic ... #Causes #Hematology
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 ... Antibody-mediated hemolysis ... Differential #Diagnosis #Hematology