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Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
disease • Periodic fever ... multiorgan failure • Fever ... - Nonremitting fever ... erythematosus [SLE ... #Hematology #Rheumatology
Skin Conditions Associated with Joint Pain

Rash
	• Human parvovirus B19 infection

Malar rash
	• SLE
	• Human parvovirus B19 infection
	•
• Rheumatic fever ... Antiphospholipid-antibody syndrome ... lesions • Discoid lupus ... #skin #rashes #differential ... #diagnosis #rheumatology
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
erythematosus (SLE ... erythematosus (SLE ... ), Sjogren's Syndrome ... ANA #patterns #rheumatology ... #diagnosis #differential
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus (SLE ... Demyelinating Syndrome ... #CNS #neurology ... #rheumatology # ... cerebritis #diagnosis #management
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... severe disease Treatment ... #Systemic #Lupus ... Summary #diagnosis #rheumatology ... #management
Extra-articular and Other Conditions Associated with Joint Pain

Eyes
  • Iritis or uveitis - Spondyloarthropathies, Sarcoidosis,
Hyperthyroidism Fever ... • Rheumatic fever ... fever • Mononeuritis ... • New murmur, fever ... #diagnosis #rheumatology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Malar rash 40% - Fever ... involvement 3% SLE-Related ... (aPL) 30-45% Differential ... Lupusreference #SLE ... #rheumatology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Features of Systemic Lupus ... (SLE) General: ... Fever (50%), Depression ... Demyelinating syndromes ... symptoms #diagnosis #rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... and Management ... , oral ulcers, Lupus ... life-threatening • Treatment ... #Summary #rheumatology
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
Criteria for Systemic Lupus ... Constitutional domain: Fever ... pericarditis • Hematologic ... Classification #Criteria #SLE ... Erythematosus #diagnosis #rheumatology