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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
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 ... Erythematosus ( ... Demyelinating Syndrome ... autoantibodies that will cause ... #rheumatology #
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
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Non-autoimmune rheumatologic ... Malignancy (e.g. hematologic ... lower extremity edema ... • May involve PNS ... #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
Connective tissue disease-associated interstitial lung diseases

LIP: lymphoid interstitial pneumonia. NSIP: nonspecific interstitial pneumonia. OP: organizing pneumonia.
SSc: systemic sclerosis ... PSS: Sjogren syndrome ... arthritis SLE : systemic ... Differential #Comparison #Table ... #Pulmonary #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 ... Summary #diagnosis #rheumatology
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