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Kawasaki Disease (KD) - Diagnosis and Management Summary

Kawasaki Disease (KD) is the most common vasculitis of
Presentation: • Fever ... Diagnostic Criteria - Fever ... KD #vasculitis #rheumatology ... #pediatrics #diagnossi ... #management #treatment
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
• Periodic fever ... multiorgan failure • Fever ... - Nonremitting fever ... ALT ↑ bilirubin level ... #Hematology #Rheumatology
Adult-Onset Still's Disease
Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia
+ Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Adult-Onset Still's Disease ... Clinical triad: Fever ... autoinflammatory diseases ... #diagnosis #management ... #treatment #rheumatology
Extra-articular and Other Conditions Associated with Joint Pain

Eyes
  • Iritis or uveitis - Spondyloarthropathies, Sarcoidosis,
Hyperthyroidism Fever ... • Rheumatic fever ... SLE, Rheumatic fever ... • New murmur, fever ... differential #diagnosis #rheumatology
Purpura - Differential Diagnosis Framework

Non-blanchable, dark red to purple, hemorrhagic skin lesions that result from leakage
Kasabach-Merritt syndrome ... Gardner-Diamond syndrome ... mountain spotted fever ... Differential #Diagnosis #hematology ... #rheumatology #
Adult-Onset Still's Disease (AOSD)
Rare inflammatory disorder of unknown etiology, difficult to diagnose because of heterogenous presentation.
Adult-Onset Still's Disease ... : High spiking fever ... Systemic AOSD: high fever ... #AOSD #rheumatology ... #diagnosis #management
Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
 • Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity
, dyspnea, +/- CXR ... - 80% have fever ... organ damage • Treatment ... TLS #diagnosis #management ... #hematology
Skin Conditions Associated with Joint Pain

Rash
	• Human parvovirus B19 infection

Malar rash
	• SLE
	• Human parvovirus B19 infection
	•
infection • Lyme disease ... migrans • Lyme disease ... • Rheumatic fever ... Antiphospholipid-antibody syndrome ... differential #diagnosis #rheumatology
Adult Onset Still's Disease
Systemic inflammation with urticarial rash.
Rash: 
 • Nonpruritic, evanescent (correlates with time of
correlates with time of fever ... Daily high spiking fever ... - Fever spikes ... Fever of at least ... AOSD #diagnosis #rheumatology
Behcet's Syndrome - Treatment
Ulcers:
 • Treatment: Topical steroids
 • Prevention: Colchicine
 • Azathioprine for refractory disease
Arthritis:
Behcet's Syndrome ... : • Treatment: ... #Syndrome #Treatment ... #management #pharmacology ... #rheumatology