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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Lupusreference #TMAs ... Erythematosus #SLE ... #hematology #rheumatology ... #differential #
Proposed Treatment Algorithm for MAS / HLH

#MAS #HLH #Hemophagocytic #Lymphohistiocytosis #Management #Treatment #Algorithm #Hematology
Proposed Treatment ... Algorithm for MAS ... / HLH #MAS # ... Lymphohistiocytosis #Management #Treatment ... #Algorithm #Hematology
Thrombotic Microangiopathy - TMAs
1. Not all MAHAs are due to TMAs but (almost) all TMAs cause
Microangiopathy - TMAs ... MAHAs are due to TMAs ... but (almost) all TMAs ... catastrophic APS, SRC, SLE ... #hematology #diagnosis
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
erythematosus (SLE ... erythematosus (SLE ... erythematosus (SLE ... ANA #patterns #rheumatology ... #diagnosis #differential
Adult-Onset Still's Disease
Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia
+ Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
fraction < 20% Differential ... Complications: MAS ... diagnosis #management #treatment ... #rheumatology
Approach to a Positive (+) Rheumatoid Factor (RF)
(+) RF does not always = RA
Rheumatic disorders:
 •
arthritis • SLE ... Rheumatoid #Factor #RF #rheumatology ... #laboratory #differential
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
dysmotility • MCTD (SLE ... • Specific for SLE ... severe disease Treatment ... Erythematosus #SLE ... Summary #diagnosis #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Activation Syndrome (MAS ... erythematosus [SLE ... Treatment: • Corticosteroids ... Diagnosis #Management #Hematology ... #Rheumatology
Initial triage of the neutropenic adult - Algorithm for the evaluation and treatment of adult patients
evaluation and treatment ... workup #diagnosis #hematology ... #indications #differential
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Erythematosus (SLE ... are evocative of SLE ... life-threatening • Treatment ... management of SLE ... Management #Summary #rheumatology