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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 ... #rheumatology # ... differential #diagnosis ... #causes
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Arthritis 69% - Malar ... - Hemolytic anemia ... Diagnosis and Lupus ... #rheumatology
Differential Diagnosis for Hemolytic Anemia
Intrinsic Causes:
 • Enzyme deficiencies ex. G6PD, pyruvate kinase 
 • Hemoglobinopathies
Differential Diagnosis ... for Hemolytic Anemia ... Bartonella, Babesia, Malaria ... , spider/snake bites ... Diagnosis #causes
Causes of Diffuse Lymphadenopathy - Differential Diagnosis Algorithm
Reactive:
 - Systemic Inflammatory
     •
Causes of Diffuse ... Diagnosis Algorithm ... Lupus Erythematosus ... • Insect Bites ... #Causes #Hematology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Lupus (SLE) General ... ) Blood (75%): Anemia ... erythematosus #signs ... #symptoms #diagnosis ... #rheumatology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Diagnosis Congenital ... deficiency • Fanconi’s anemia ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... #Diagnosis #Hematology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Diagnostic Framework ... usually IgG) (e.g. lupus ... • Infections: Malaria ... #differential # ... diagnosis #hematology
Systemic Lupus Erythematosus (SLE) - Typical Signs/Symptoms
Alopecia, Fever, Malar rash, Photosensitivity, Adenopathies, Lupus pneumonia, Pleurisy, Pancreatitis,
Systemic Lupus Erythematosus ... SLE) - Typical Signs ... Alopecia, Fever, Malar ... #SLE #Signs #Symptoms ... #diagnosis #rheumatology
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
- Differential ... Diagnosis Algorithm ... Inflammatory • Systemic ... Lupus Erythematosus ... #Causes #Hematology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... (SLE) - Diagnosis ... Manifestations: Malar ... Positive in 60-80% of cases ... #Summary #rheumatology