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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 ... #SLE #hematology ... #rheumatology # ... differential #diagnosis #causes
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 ... Puncture, EEG Treatment ... #rheumatology #
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 ... life-threatening • Treatment ... Management #Summary #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
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lupus erythematosus ... (CNS) dysfunction ... Treatment: • Corticosteroids ... Diagnosis #Management #Hematology ... #Rheumatology
Mucous Membrane Disorder - Differential Diagnosis Algorithm
Erosions/Ulcers/Blisters
 - Primary Dermatologic Diseases
     •
Stevens-Johnson Syndrome ... necrolysis - Systemic ... lupus erythematosus ... MucousMembranes #dermatology ... Diagnosis #Algorithm #causes
Approach to Thrombocytosis - Elevated Platelet Count - Differential Diagnosis Algorithm

Peripheral Smear concerning for malignancy?
→ Refer to hematology ... for workup, including ... Rebound effect from treatment ... Kawasaki, Nephrotic syndrome ... Diagnosis #Algorithm #hematology
BEE Syndromes - Immune-mediated conditions affecting the brain, eye, and ear

Visual or auditory symptoms in conjunction
central nervous system ... (CNS) involvement ... diagnosis and treatment ... Inflammatory causes ... • Systemic lupus
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
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
Causes of Splenomegaly ... Inflammatory • Systemic ... Lupus Erythematosus ... Diagnosis #Algorithm #Causes ... #Hematology