Anti-neutrophil cytoplasmic antibodies (ANCA) in Kawasaki disease

Autor: Earl D. Silverman, Nina Birdi, Ronald M. Laxer
Rok vydání: 1992
Předmět:
Zdroj: Progress in Pediatric Cardiology. 1:88
ISSN: 1058-9813
DOI: 10.1016/s1058-9813(06)80091-3
Popis: ANCA have been described in a number of vasculitic conditions, such as Wegener's Granulomatosis, Polyarteritis Nodosa, ChurgStrauss vasculitis and crescentic glomerulonephritis, and may be a marker of disease activity. We have studied 55 children with Kawasaki Disease (KD) aged 3 months to 8 years for the presence of ANCA by indirect immunofluorescence (IF) of ethanol-fixed neutrophils and by using a commercially available ELISA kit (Biocarb Diagnostics). Forty-nine patients fuEilled 5 or 6 criteria for the diagnosis of KD, and 6 had 4 criteria. All patients were ueated with high dose ASA and intravenous gammaglobulin (IVGG) followed by low dose ASA as per standard protocol. Thirty-seven samples were tested in the acute (0-2wks), 54 in the subacute (2-10 wks) and 22 in the recovery phase (>!0 wks). A positive ANCA was found in 39/55 patients (70%) by IF or ELISA. All IF positive scra were cytoplasmic in pattern. The majority of samples were positive in the subacute phase (33/54). Sixteen of 19 patients (84%) with coronary artery lesions (CAL = dilatation/ectasia/ ancurysm) were ANCA positive, compared to 23/36 (64%) of patients without CAL. Six of 8 patients with coronary aneurysms and all 5 patients with persistent aneurysms were ANCA positive in the acute or suhacute phase. Three of 4 patients with ventriculomegaly on echocardiogram but no CAL and 4/8 patients with isolated pericardial effusion were ANCA positive. All 5 patients who were retreated with IVGG for failure to respond or recurrence of KD were ANCA positive, and 3/5 had CAL (2 with persistent aneurysms). ANCA was detected in 2/33 or 6% of control patients. The high incidence of positive ANCA in this series of patients with KD likely reflects the presence of systemic vasculitis. The majority of samples were positive in the subacute phase, suggesting that ANCA occurs as part of a primary immune response. ANCA appears to be sensitive for CAL in KD but is not specific. As every patient with KD is treated with IVGG, the natural history of the disease is altered and may influence the development of anti-nuclear cytoplasmic antibodies. P24
Databáze: OpenAIRE