title: Relationship among antineutrophil cytoplasmic antibody, blood urea nitrogen and complement in patients with eosinophilic granulomatosis polyangiitis (Churg-Strauss syndrome).
abstract: Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by a history of asthma, hypereosinophilia. The prevalence of ANCA in EGPA is less common than in other ANCA-associated vasculitis. Increasing evidence of complement activation in the pathogenesis of ANCA-associated vasculitis has been provided by studies in animal models. We examined EGPA patients with cutaneous manifestations as an initial sign and investigated the correlations among clinical, serological and histopathological findings. We focused on differences among ANCA, blood urea nitrogen and complement levels such as complement 3 (C3), C4 and total complement hemolytic activity (CH50). We retrospectively investigated the records of 22 patients (11 male and 11 female) with EGPA admitted to our hospital from 1997-2012. Ten of the 22 patients (46%) were positive for serum myeloperoxidase (MPO)-ANCA. In contrast, all the patients were negative for serum proteinase 3 ANCA. There was a significantly positive correlation between serum CH50 and C4 levels in patients with EGPA. Serum blood urea nitrogen (BUN) levels differed significantly between MPO-ANCA-positive and -negative patients. Serum CH50 levels were higher in MPO-ANCA-positive patients compared to negative patients. Serum BUN levels were higher in elevated CH50 patients compared to normal and low CH50-negative patients. We propose that positive findings for MPO-ANCA with CH50 high activity may be a risk factor for developing renal insufficiency. Assuming there are correlations between the presence of ANCA and complements, earlier diagnosis based on initial efficacious treatment for EGPA.
authors.affiliation: Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan. tami@marianna-u.ac.jp
authors.forename: Tamihiro
authors.initials: T
authors.lastname: Kawakami
authors.forename: Satoko
authors.initials: S
authors.lastname: Kimura
authors.forename: Sora
authors.initials: S
authors.lastname: Takeuchi
authors.forename: Yoshinao
authors.initials: Y
authors.lastname: Soma
mesh_terms: D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001806:Blood Urea Nitrogen; D015267:Churg-Strauss Syndrome / Q000097:blood* / Q000473:pathology; D003165:Complement System Proteins / Q000378:metabolism*; D005260:Female; D006801:Humans; D007866:Leg / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D003165:Complement System Proteins
keywords: Churg-Strauss syndrome; antineutrophil cytoplasmic antibody; blood urea nitrogen, complement; eosinophilic granulomatosis with polyangiitis