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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
Clinical Manifestations of Systemic Lupus Erythematosus (SLE)

#Systemic #Lupus #Erythematosus #SLE #Manifestations #Signs #Symptoms #Rheumatology #Diagnosis
Lupus Erythematosus ... (SLE) #Systemic ... Manifestations #Signs ... #Symptoms #Rheumatology ... #Diagnosis
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
lupus erythematosus ... lupus erythematosus ... SLE), Sjogren's Syndrome ... ANA #patterns #rheumatology ... #diagnosis #differential
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... block • Sjogren syndrome ... #SLE #Summary #diagnosis ... #rheumatology # ... management
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lupus erythematosus ... lupus erythematosus ... 5000 to 10,000 ng/mL ... #Diagnosis #Management ... #Hematology #Rheumatology
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... antiphospholipid syndrome ... Lupus Erythematosus ... CHAMPS #Mnemonic #diagnosis ... #differential #hematology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Lupus Erythematosus ... Demyelinating Syndrome ... autoantibodies that will cause ... CNS #neurology #rheumatology ... #management #treatment
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Clinical Features of Systemic ... Demyelinating syndromes ... #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
- Differential Diagnosis ... Wiskott-Aldrich syndrome ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... #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 ... #Erythematosus ... #Diagnosis #Management ... #Summary #rheumatology