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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 ... Microangiopathies #Systemic #Lupus ... Erythematosus #SLE ... #hematology #rheumatology ... differential #diagnosis #causes
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... and Management ... , oral ulcers, Lupus ... Positive in 60-80% of cases ... #Summary #rheumatology
Drug Induced Lupus vs SLE
Drug Induced Lupus (DIL):
 • Epidemiology: -10% of all lupus cases, drug-dependent,
vs SLE Drug Induced ... Discontinuation of causal ... Usual therapeutic management ... #table #rheumatology ... #diagnosis #management
Hydroxychloroquine Serum Measurement in SLE
 • Terminal elimination half-life = 40 days ([HCQI reflects long-term intake)
Measurement in SLE ... #Measurement #SLE ... #rheumatology # ... pharmacology #levels #lupus ... #management
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus (SLE ... autoantibodies that will cause ... Erythematosus #SLE ... CNS #neurology #rheumatology ... cerebritis #diagnosis #management
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Lymphohistiocytosis (HLH ... Autoimmune diseases: SLE ... Drugs, Unknown cause ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
Aortitis - Differential Diagnosis Framework
Non-Infectious Aortitis:
 • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
(RA), Systemic lupus ... erythematosus (SLE ... , Sarcoidosis, HLA-B27 ... (SPA) • Other Causes ... Differential #Diagnosis #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lymphohistiocytosis (HLH ... SJIA], systemic lupus ... erythematosus [SLE ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... • Specific for SLE ... #Systemic #Lupus ... Summary #diagnosis #rheumatology ... #management
Causes of Thrombocytopenia - Differential Diagnosis Algorithm
 
Decreased Megakaryopoiesis
 • Aplastic Anemia
 • Toxic Damage (e.g.
Causes of Thrombocytopenia ... Autoimmune • ITP • SLE ... Alloimmune • anti-HLA ... Diagnosis #Algorithm #Causes ... #Hematology