Antineutrophil Cytoplasmic Autoantibodies (ANCA) in Children with Cystic Fibrosis

Šedivá, Anna; Bartůňková, Jiřina; Kolářová, I.; Hrušák, Ondřej; Vávrová, V.; Maçek, Milan; M-Lockwood, Christopher; Dunn, Austin

doi:10.1006/jaut.1997.0186

Cited by 40 publications

(12 citation statements)

References 10 publications

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“…Such a study would also begin to address any potential confounding role that age might play in autoimmunity. In addition, examining the full repertoire of autoantibodies in CF patients would provide a more complete picture of the immune response, as anti-PR3 autoantibodies have been reported in pediatric CF patients (70).…”

Section: Discussionmentioning

confidence: 99%

The role for neutrophil extracellular traps in cystic fibrosis autoimmunity

Skopelja¹,

Hamilton²,

Jones³

et al. 2016

JCI Insight

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Section: Discussionmentioning

confidence: 99%

The role for neutrophil extracellular traps in cystic fibrosis autoimmunity

Skopelja¹,

Hamilton²,

Jones³

et al. 2016

JCI Insight

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“…[7–13]. Anti-neutrophil cytoplasmic antibodies (ANCA) with BPI specificity have been identified in different diseases associated with Gram-negative bacteria, such as inflammatory bowel diseases (IBD) and primary sclerosing cholangitis [14], and are frequently present in CF patients [11, 15, 16]. …”

Section: Introductionmentioning

confidence: 99%

BPI-ANCA and Long-Term Prognosis among 46 Adult CF Patients: A Prospective 10-Year Follow-Up Study

Lindberg

Carlsson

Löfdahl

et al. 2012

Clinical and Developmental Immunology

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Introduction. Anti-neutrophil cytoplasmic antibodies specific for bactericidal/permeability-increasing protein (BPI-ANCA) are frequent in CF patients and mainly develop in response to infection with Pseudomonas aeruginosa. It is not known to what extent BPI-ANCA correlates to prognosis. Objectives. To evaluate the prognostic value of IgA-BPI-ANCA, measured at the beginning of the study, for transplantation-free survival. Methods. A cohort of 46 adult, nontransplanted CF patients was generated, 1995–1998, and characterized using Leeds criteria, lung function, and IgA-BPI-ANCA levels measured by ELISA. The cohort was followed until December 2009, using the combined endpoint of death or lung transplantation. Results. Lung function and IgA-BPI-ANCA, but not Leeds criteria, were significantly associated with adverse outcome. No patient with normal lung function at baseline reached endpoint. Within 10 years 8/11 with high BPI-ANCA reached an endpoint compared to 3/17 ANCA-negative patients. A similar result was seen within the Leeds I group where 7 out of 9 BPI-ANCA-positive patients reached endpoint, compared to none of the 5 patients without BPI-ANCA. Conclusions. IgA-BPI-ANCA is associated with adverse outcome among Pseudomonas aeruginosa infected CF patients, suggesting that BPI-ANCA is a biomarker of an unfavourable host-pathogen interaction.

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“…Hyper-gammaglobulinemia and the presence of ICs in pwCF may be caused by systemic inflammation, even in the absence of self-directed autoimmune responses (13). Per se, IC formation is a physiological mechanism as it is the result of antibody production (IgG or IgM) and their binding to molecular targets, resulting in antigen neutralization.…”

Section: The Molecular Pathophysiology Of Vasculitis In Cf Immune Commentioning

confidence: 99%