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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

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

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
of Systemic Lupus ... Demyelinating Syndrome ... autoantibodies that will cause ... #Lupus #Erythematosus ... #rheumatology #
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... block • Sjogren syndrome ... #Lupus #Erythematosus ... Summary #diagnosis #rheumatology ... #management
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... Diagnosis and Management ... Positive in 60-80% of cases ... #Systemic #Erythematosus ... #Summary #rheumatology
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
SLE (Systemic Lupus ... lupus erythematosus ... • May involve PNS ... #Lupus #Erythematosus ... #Diagnosis #Rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lupus erythematosus ... (CNS) dysfunction ... lupus erythematosus ... #Diagnosis #Management ... #Hematology #Rheumatology
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... antiphospholipid syndrome ... glomerulonephritis (>90%) S - Systemic ... Lupus Erythematosus ... #differential #hematology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Wiskott-Aldrich syndrome ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... Differential #Diagnosis #Hematology
BEE Syndromes - Immune-mediated conditions affecting the brain, eye, and ear

Visual or auditory symptoms in conjunction
central nervous system ... (CNS) involvement ... Inflammatory causes ... • Systemic lupus ... erythematosus