J Korean Rheum Assoc.  2007 Jun;14(2):154-159. 10.4078/jkra.2007.14.2.154.

Two Cases of Adult Onset Still's Disease with Disseminated Intravascular Coagulation and Multiple Organ Involvement Treated with Cyclosporine

• Shim EJ
• Lee YH
• Lee J

• KMID: 1526433
• DOI: http://doi.org/10.4078/jkra.2007.14.2.154

Abstract

Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis. It is characterized by spiking fever, evanescent skin rash, arthralgia or arthritis, hepatosplenomegaly and laboratory abnormalities including neutrophilic leukocytosis, abnormal liver function tests and raised levels of serum ferritin. Coagulation abnormality is a rare presenting feature but it might be life threatening when associated with hepatopathy and hematologic abnormalities. We report two cases of AOSD with disseminated intravascular coagulation and multiple organ involvement, which improved with glucocorticoid and cyclosporine combination therapy.

Keyword

Adult onset Still's disease; Multiple organ failure; Disseminated intravascular coagulation; Cyclosporine

MeSH Terms

Adult*
Arthralgia
Arthritis
Cyclosporine*
Disseminated Intravascular Coagulation*
Exanthema
Ferritins
Fever
Humans
Leukocytosis
Liver Function Tests
Multiple Organ Failure
Neutrophils
Still's Disease, Adult-Onset*
Cyclosporine
Ferritins

Figure

• 

  Fig. 1. Brain MRI (T2-weighted image) shows bilateral asymmetric high signal intensity at pons (A), temporal lobe (B), occipitoparietal subcortical area, and thalamus.

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