Korean J Fam Med.  2010 Apr;31(4):308-313. 10.4082/kjfm.2010.31.4.308.

A Case Report of Anti-Neutrophil Cytoplasmic Antibodies Negative Churg-Strauss Syndrome

• Lee SB
• Kang HC
• Choi JJ

• KMID: 2234218
• DOI: http://doi.org/10.4082/kjfm.2010.31.4.308

Abstract

Churg-Strauss syndrome is a rare primary vasculitis called allergic granulomatosis angiitis. There is a substantial overlap of symptoms or signs between different vasculitides, so that a diagnosis of any vasculitis can be determined by a combination of clinical symptoms and laboratory tests. We report a case of a 54-year-old woman who had bronchial asthma, eosinophilia, peripheral polyneuropathy, pulmonary infiltrate, maxillary sinusitis, and extravascular eosinophilic infiltration on skin biopsy. So she was diagnosed as having Churg-Strauss syndrome, but she was antineutrophil cytoplasmic antibodies-negative on immunologic study. She had fever and showed cardiac involvement that was presented as cardiomegaly and pulmonary edema at chest radiography and left ventricular hypertrophy on electrocardiography. After steroid therapy, general condition and skin manifestation were rapidly improved.

Keyword

Churg-Strauss Syndrome; Vasculitis; ANCA

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Asthma
Biopsy
Cardiomegaly
Churg-Strauss Syndrome
Cytoplasm
Electrocardiography
Eosinophilia
Eosinophils
Female
Fever
Humans
Hypertrophy, Left Ventricular
Maxillary Sinus
Maxillary Sinusitis
Middle Aged
Polyneuropathies
Pulmonary Edema
Skin
Skin Manifestations
Thorax
Vasculitis
Antibodies, Antineutrophil Cytoplasmic
Churg-Strauss Syndrome