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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
Dyspnea - Differential Diagnosis Framework

Cardiovascular Causes of Dyspnea:
 • Myocardium:
	- Heart failure
	- CAD/ACS
	- Valvulopathy
 • Electrical:
	-
- Water - Pus ... Hepatopulmonary syndrome ... Barre - ALS - Transverse ... myelitis • Hematology ... : - Anemia
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
lupus erythematosus ... , Drug-induced Lupus ... lupus erythematosus ... SLE), Sjogren's Syndrome ... ANA #patterns #rheumatology
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 ... Differential #Diagnosis #Hematology
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 ... RheumOnePagers) #Systemic ... #rheumatology #
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 ... Thrombocytopenia #Lupus ... symptoms #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 ... with leukopenia, anemia ... Diagnosis #Management #Hematology ... #Rheumatology
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 ... lupus. ... Table #Pulmonary #Rheumatology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... hemoglobinuria (PNH ... usually IgG) (e.g. lupus ... differential #diagnosis #hematology ... #anemia
Nail Disorders secondary to Systemic Diseases
Nail Fold Abnormality
 • SLE
 • Scleroderma
 • Dermatomyositis
Koilonychia - Spoon-shaped
Iron deficiency anemia ... Muehrcke's Lines - Transverse ... • Nephrotic syndrome ... #Secondary #dermatology ... Diagnosis #Algorithm #causes