Korean J Med.  2015 Oct;89(4):482-486. 10.3904/kjm.2015.89.4.482.

Adult-Onset Still's Disease with Thrombotic Thrombocytopenic Purpura

• Lee TH
• Lee JM
• Lee SY
• Cho NH
• Son CN
• Kim JM
• Kim SH

• KMID: 2268987
• DOI: http://doi.org/10.3904/kjm.2015.89.4.482

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder that affects multiple organs and is fatal in some cases. Thrombotic thrombocytopenic purpura (TTP), another rare disease, is characterized by multiple organ failure and microangiopathic hemolytic anemia. TTP is an extremely rare complication of AOSD. We report a 59-year-old woman who presented with TTP that manifested after she was diagnosed with AOSD. Initially, her clinical manifestations improved with glucocorticoid therapy. However, her disease worsened and was accompanied by acute kidney injury, thrombocytopenia, hemolytic anemia, petechiae, and impaired consciousness. These clinical findings led to a diagnosis of TTP. This is the first report of AOSD complicated by TTP in Korea. Awareness of the possible development of TTP as a complication of AOSD is important for early diagnosis and treatment.

Keyword

Still's disease, Adult-onset; Purpura, Thrombotic thrombocytopenic

MeSH Terms

Acute Kidney Injury
Anemia, Hemolytic
Consciousness
Diagnosis
Early Diagnosis
Female
Glycogen Storage Disease Type VI
Humans
Korea
Middle Aged
Multiple Organ Failure
Purpura
Purpura, Thrombotic Thrombocytopenic*
Rare Diseases
Still's Disease, Adult-Onset*
Thrombocytopenia