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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
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... antiphospholipid syndrome ... S - Systemic Lupus ... #differential #hematology
Monoclonal Gammopathy of Clinical Significance (MGUS) - Differential Diagnosis

Neurologic-Centered:
 (+) Systemic Features: POEMS, AL amyloid, Cryoglobulinemia
capillary leak syndrome ... • Schnitzler syndrome ... immunotactoid GN ... , Type I cryo GN ... Differential #Diagnosis #hematology
Rapidly Progressive Glomerulonephritis (RPGN)
RPGN has three primary pathophysiologic causes differentiated by immunofluorescence

Immune Complex Mediated (granular staining):
pathophysiologic causes ... Complement: Post-strep GN ... , Lupus Nephritis ... diagnosis #algorithm #rheumatology
Paraproteinemias

Entities That Can Feature A Monoclonal Protein/M Component:
• MM
• WM
• MGUS
• MGCS
• MGRS
• Splenic Marginal Zone
Immunotactoid GN ... Cryoglobulinemic GN ... -C3 GN • Cast ... to cold IgG • Lupus ... Paraproteinemias #Hematology
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
Causes of Splenomegaly ... Inflammatory • Systemic Lupus ... Spherocytosis • Acquired causes ... Diagnosis #Algorithm #Causes ... #Hematology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Wiskott-Aldrich syndrome ... Diseases: • Systemic lupus ... Differential #Diagnosis #Hematology
Purpura - Differential Diagnosis Framework

Non-blanchable, dark red to purple, hemorrhagic skin lesions that result from leakage
Kasabach-Merritt syndrome ... Gardner-Diamond syndrome ... Differential #Diagnosis #hematology ... #rheumatology # ... #nonpalpable #dermatology
Mucous Membrane Disorder - Differential Diagnosis Algorithm
Erosions/Ulcers/Blisters
 - Primary Dermatologic Diseases
     •
Stevens-Johnson Syndrome ... • Systemic lupus ... • Behcet's syndrome ... MucousMembranes #dermatology ... Diagnosis #Algorithm #causes
Causes of Diffuse Lymphadenopathy - Differential Diagnosis Algorithm
Reactive:
 - Systemic Inflammatory
     •
Causes of Diffuse ... • Systemic Lupus ... Diagnosis #Algorithm #Causes ... #Hematology #Oncology