A case of periodic-fever-syndrome-like disorder with lipodystrophy, myositis, and autoimmune abnormalities

被引：11

作者：

Kasagi, Shimpei ^{[1
]}

Kawano, Seiji ^{[1
]}

Nakazawa, Takashi ^{[1
]}

Sugino, Hirotoshi ^{[2
]}

Koshiba, Masahiro ^{[3
]}

Ichinose, Kunihiro ^{[4
]}

Ida, Hiroaki ^{[4
]}

Eguchi, Katsumi ^{[4
]}

Kumagai, Shunichi ^{[1
]}

机构：

[1] Kobe Univ, Dept Clin Pathol & Immunol, Grad Sch Med, Chuo Ku, Kobe, Hyogo 6500017, Japan

[2] Sugino Syonika Clin, Asakita Ku, Hiroshima 7310231, Japan

[3] Hyogo Coll Med, Dept Clin Lab, Nishinomiya, Hyogo 6638501, Japan

[4] Nagasaki Univ, Dept Internal Med 1, Nagasaki Univ Hosp Med & Dent, Grad Sch Biomed Sci, Nagasaki 8528501, Japan

来源：

MODERN RHEUMATOLOGY | 2008年 / 18卷 / 02期

关键词：

Periodic fever syndrome; Myositis; Lipodystrophy; TNF-alpha;

D O I：

10.1007/s10165-008-0033-4

中图分类号：

R5 [内科学];

学科分类号：

1002 [临床医学]; 100201 [内科学];

摘要：

A 24-year-old Japanese woman had been suffering from a periodic fever since 10 months of age. She developed deformities in her fingers, with severe atrophy of subcutaneous adipose tissue, myositis, and frostbitten hands. She showed elevated C-reactive protein, creatine kinase, and gamma-globulin. She was also positive for antinuclear, anti-DNA, anti-SS-B, and anti-U1RNP antibodies. Her myositis was similar to amyopathic dermatomyositis rather than juvenile dermatomyositis. Although consanguineous marriage of her parents and early onset of disease suggested her disease as a hereditary disorder with periodic fever, her clinical feature and laboratory tests were unlike any known periodic fever syndromes. Her disease was regarded as a unique type of periodic-fever-syndrome-like disorder with autoimmune abnormalities.

引用

页码：203 / 207

页数：5

共 19 条

[1]

ASSOCIATION OF PARTIAL LIPODYSTROPHY AND SJOGRENS SYNDROME [J].