Ceruloplasmin levels in antineutrophil cytoplasmic antibody-positive patients.
Anti-myeloperoxidase (MPO) antibodies are associated with the development of anti-neutrophil cytoplasmic antibody (ANCA)-related vasculitis. The imbalance between the protease-antiprotease activity in the neutrophils has been implicated in the pathogenesis of ANCA-related vasculitis. Ceruloplasmin is an acute-phase protein that has antiproteinase and antioxidant properties and inhibits MPO activity. We attempted to study the association between serum ceruloplasmin and ANCA in childhood vasculitis. Forty-five ANCA-related diseases were included in the study. The age range was 4-16 years. Patients were divided into two groups based on indirect immunofluorescence and/or ELISA specificity (MPO). Twenty-six patients had p-ANCA- and 19 patients had c-ANCA-positive disease. Nine patients with Henoch-Schönlein purpura were studied as an ANCA-negative control group. Serum ceruloplasmin levels in p-ANCA-, c-ANCA-positive patients, and controls were 125.85+/-93.48 mg/dl, 59.79+/-17.60 mg/dl, and 64.34+/-18.77 mg/dl, respectively, and were significantly higher in patients with p-ANCA ( P<0.05). Ceruloplasmin levels were significantly decreased in remission ( P<0.05). Median MPO level in p-ANCA-positive patients was 15.2 (5-250) and was negative in all c-ANCA-positive patients. There was a significant positive correlation between MPO and ceruloplasmin levels ( r=0.70, P<0.05). Of 26 patients (53.8%) in the p-ANCA-positive group, 14 had renal involvement. The patients with renal disease had significantly higher ceruloplasmin levels than others (151.17+/-92.14 and 134.64+/-95.16 mg/dl respectively, P<0.05). In conclusion, the increase in ceruloplasmin levels during the acute phase suggests that this might be an activation criterion or a response to neutrophil-mediated tissue injury. Increased ceruloplasmin levels together with p-ANCA positivity may be predictive for renal involvement and a serious clinical course. The correlation between ceruloplasmin and MPO levels supports their association. Further studies are necessary to elucidate whether genetic and/or functional alterations in ceruloplasmin are effective in the pathogenesis of vasculitis.
Baskin
Esra
E
Department of Pediatric Nephrology, Hacettepe University, Ankara, Turkey. esrabaskin@hotmail.com
Bakkaloglu
Aysin
A
Besbas
Nesrin
N
Hascelik
Gulsen
G
Saatci
Umit
U
Gök
Faysal
F
Ozen
Seza
S
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000235:genetics; D001799:Blood Sedimentation; D002570:Ceruloplasmin / Q000235:genetics / Q000378:metabolism*; D002648:Child; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D011695:IgA Vasculitis / Q000097:blood; D008297:Male; D009195:Peroxidase / Q000097:blood; D014657:Vasculitis / Q000097:blood* / Q000235:genetics
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase; D002570:Ceruloplasmin