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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
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Lupus (SLE) General ... Demyelinating syndromes ... ) Blood (75%): Anemia ... #Lupus #SLE #systemic ... #rheumatology
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
(SLE), Sjogren's ... Syndrome (SS), ... (SLE), Systemic ... ANA #patterns #rheumatology ... #diagnosis #differential
SLICC SLE Diagnostic Criteria - Systemic Lupus Erythematosus
Clinical and Immunologic Criteria

CLINICAL CRITERIA
 - ACUTE CUTANEOUS LUPUS
SLICC SLE Diagnostic ... Criteria - Systemic ... (<1000/MM3) - THROMBOCYTOPENIA ... #Diagnosis #Rheumatology ... #SLE #Lupus #Erythematosus
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Neurological 12% - Thrombocytopenia ... - Hemolytic anemia ... #Systemic #Lupus ... #differential #rheumatology
Causes of Thrombocytopenia - Differential Diagnosis Algorithm
 
Decreased Megakaryopoiesis
 • Aplastic Anemia
 • Toxic Damage (e.g.
Causes of Thrombocytopenia ... Megakaryopoiesis • Aplastic Anemia ... Autoimmune • ITP • SLE ... Non-lmmune: • HELLP Syndrome ... Algorithm #Causes #Hematology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
deficiency • Fanconi’s anemia ... Wiskott-Aldrich syndrome ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... #Hematology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
[SLE], AOSD) • ... with leukopenia, anemia ... , and thrombocytopenia ... #Diagnosis #Management ... #Hematology #Rheumatology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... block • Sjogren syndrome ... polymyositis overlap syndrome ... #Lupus #Erythematosus ... diagnosis #rheumatology
Causes of Purpura or Easy Bruising in Children

Platelet count reduced, i.e. thrombocytopenia
 - Increased platelet destruction
) - SLE ( ... systemic lupus erythematosus ... - Fanconi anaemia ... Henoch—Schönlein purpura, SLE ... peds #pediatrics #diagnosis