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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 ... #Erythematosus ... #rheumatology # ... differential #diagnosis #causes
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Diseases: • Systemic lupus ... erythematosus ... • HLH • Paroxysmal ... 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 ... Diagnosis and Management ... Positive in 60-80% of cases ... #Systemic #Erythematosus ... #Summary #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus ( ... autoantibodies that will cause ... #Erythematosus ... CNS #neurology #rheumatology ... cerebritis #diagnosis #management
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
erythematosus ... SJIA], systemic lupus ... erythematosus [ ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Aortitis - Differential Diagnosis Framework
Non-Infectious Aortitis:
 • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
(RA), Systemic lupus ... erythematosus ( ... , Sarcoidosis, HLA-B27 ... (SPA) • Other Causes ... Differential #Diagnosis #rheumatology
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
Causes of Splenomegaly ... Inflammatory • Systemic Lupus ... Erythematosus ... Diagnosis #Algorithm #Causes ... #Hematology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... #Systemic #Lupus ... #Erythematosus ... Summary #diagnosis #rheumatology ... #management
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... S - Systemic Lupus ... Erythematosus ( ... #differential #hematology
Causes of Diffuse Lymphadenopathy - Differential Diagnosis Algorithm
Reactive:
 - Systemic Inflammatory
     •
Causes of Diffuse ... • Systemic Lupus ... Erythematosus ... Diagnosis #Algorithm #Causes ... #Hematology #Oncology