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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Lupus Erythematosus ... #SLE #hematology ... #rheumatology # ... differential #diagnosis ... #causes
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
ANA Patterns and ... (SLE), Sjogren's ... (SLE), Systemic ... #patterns #rheumatology ... #diagnosis #differential
Hemolysis - Diagnostic Algorithm and Causes

Increased red cell production: 
 • Increased Reticulocytosis/polychromasia 
 • Erythroid
Hemolysis - Diagnostic ... Algorithm and Causes ... #Algorithm #Differential ... #Causes #Workup ... #Hematology #Diagnosis
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... hemoglobinuria (PNH ... usually IgG) (e.g. lupus ... #differential # ... diagnosis #hematology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... • Immunologic Workup ... Positive in 60-80% of cases ... #lupus #Systemic ... Management #Summary #rheumatology
Causes of Extravascular and Intravascular Hemolysis - Differential Diagnosis Algorithm
Extravascular - Spleen and RES-mediated hemolysis
 -
Causes of Extravascular ... Hemolysis - Differential ... • PCH • PNH ... Extravascular #Differential ... #Hematology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
of Systemic Lupus ... Erythematosus (SLE ... autoantibodies that will cause ... #SLE #CNS #neurology ... #rheumatology #
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
- Differential ... Inflammatory • Systemic ... Lupus Erythematosus ... Serum Sickness Hemolytic ... #Hematology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Lymphadenopathies 7% - Hemolytic ... Diagnosis and Lupus ... #Systemic #Lupus ... #rheumatology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Inflammatory and ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... Antibody-mediated hemolysis ... #Diagnosis #Hematology