Pulmonary fibrosis—an uncommon manifestation of anti-myeloperoxidase-positive systemic vasculitis?

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Abstract

Small vessel vasculitides such as microscopic polyangiitis and Wegener’s granulomatosis commonly involve the kidney and lung, with alveolar haemorrhage being the commonest manifestation of pulmonary involvement. Here we describe a patient who developed acute renal failure and pulmonary haemorrhage with positive autoantibodies against myeloperoxidase 1 year after a diagnosis of usual interstitial pneumonia had been made and we discuss the uncommon association of pulmonary fibrosis and anti-myeloperoxidase positive vasculitis.

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Most cited references 22

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Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro.

R J Falk, R Terrell, L Charles … (1990)

Anti-neutrophil cytoplasmic autoantibodies (ANCA) are in the circulation of most patients with pauci-immune necrotizing vasculitis and pauci-immune crescentic glomerulonephritis. The current study demonstrates an effect of these autoantibodies on neutrophil function in vitro. ANCA cause normal human neutrophils to undergo an oxidative burst and degranulate. Both ANCA phenotypes (i.e., cytoplasmic-pattern ANCA and myeloperoxidase-specific ANCA) induce neutrophil activation. ANCA sera and purified immunoglobulins significantly increase the release of reactive oxygen species when compared with controls. ANCA, in a dose-dependent manner, induce the release of primary granule contents. These effects are markedly enhanced by priming neutrophils with tumor necrosis factor. Flow cytometry studies demonstrate the presence of myeloperoxidase on the surface of neutrophils after cytokine priming, indicating that primed neutrophils have ANCA antigens at their surfaces to interact with ANCA. These observations suggest an in vivo pathogenetic role for ANCA. We propose that, in patients with necrotizing vasculitis, ANCA-induced release of toxic oxygen radicals and noxious granule enzymes from cytokine-primed neutrophils could be mediating vascular inflammation.

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Characteristics of Chinese patients with Wegener's granulomatosis with anti-myeloperoxidase autoantibodies.

Ming-Hui Zhao, Zhong Zou, Hai-rong Wang … (2005)

Cytoplasmic antineutrophil cytoplasmic autoantibodies (cANCA)/proteinase-3(PR3)-ANCA was considered the serologic diagnostic marker for Wegener's granulomatosis (WG). However, Chinese patients with MPO-ANCA positive WG were frequently diagnosed. We now analyze the characteristics of patients with MPO-ANCA positive WG and investigate the difference between patients with MPO-ANCA and PR3-ANCA. Patients with WG were selected according to both Chapel Hill Consensus Conference definition and American College of Rheumatology (ACR) classification criteria in 500 Chinese patients with ANCA-associated systemic vasculitides. The clinical manifestions were compared between patients with MPO-ANCA and with PR3-ANCA. Eight-nine patients fulfilled the diagnostic criteria of WG: 54/89(60.7%) were MPO-ANCA positive, 34/89(38.2%) were PR3-ANCA positive. Patients with MPO-ANCA were predominantly female compared with patients with PR3-ANCA. Patients with MPO-ANCA also had multisystem involvement. However, the prevalences of arthagia, skin rash, ophthalmic and ear involvement were significantly lower in patients with MPO-ANCA than those in patients with PR3-ANCA (46.3% vs. 70.6%, P < 0.05; 20.4% vs. 44.1%, P < 0.05; 27.8% vs. 58.8%, P < 0.01; 40.7% vs. 67.6%, P < 0.05, respectively). The prevalence of elevated initial serum creatinine was significantly higher in patients with MPO-ANCA than that in patients with PR3-ANCA (81.5% vs. 61.8%, chi(2) = 4.20, P < 0.05). Patients with MPO-ANCA positive WG were not rare in Chinese.

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Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature.

C Agard, L Guillevin, B Hervier … (2009)

To describe the clinical presentation of the association between pulmonary fibrosis (PF) and systemic vasculitis related to antineutrophil cytoplasmic antibodies (ANCA-V). 12 patients (three female, mean age 70.7 years) with ANCA-V associated with "idiopathic" PF were studied retrospectively. ANCA-V and PF were diagnosed simultaneously in eight cases; PF occurred earlier in three cases and during ANCA-V follow-up in one. No patient had intra-alveolar haemorrhage (IAH). ANCA were myeloperoxidase (MPO)-ANCA in all cases. Seven patients had blood eosinophilia at diagnosis. Two patients died during ANCA-V induction therapy. The respiratory status of five patients worsened and three of them died from exacerbation of end-stage respiratory failure. The five remaining patients had a stable respiratory status. The association of PF and ANCA-V does not seem to be fortuitous, even though their clinical evolutions are clearly not related. PF was the major cause of death.

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Journal

Journal ID (nlm-ta): NDT Plus

Journal ID (publisher-id): ndtplus

Journal ID (hwp): ndtplus

Title: NDT Plus

Publisher: Oxford University Press

ISSN (Print): 1753-0784

ISSN (Electronic): 1753-0792

Publication date (Print): August 2010

Publication date (Electronic): 14 April 2010

Publication date PMC-release: 14 April 2010

Volume: 3

Issue: 4

Pages: 351-353

Affiliations

Department of Renal Medicine, simpleNorfolk and Norwich University Hospital , Norwich, UK

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Mahzuz Karim; E-mail: mkarim@ 123456doctors.org.uk

Article

Publisher ID: sfq050

DOI: 10.1093/ndtplus/sfq050

PMC ID: 2904803

PubMed ID: 20640181

SO-VID: ad23596b-14db-40c5-8dd2-ceb9ac0389c9

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This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/2.5), which permits unrestricted non-commercial use, distribution and reproduction in any medium, provided the original work is properly cited.

Pulmonary fibrosis—an uncommon manifestation of anti-myeloperoxidase-positive systemic vasculitis?

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Most cited references 22

Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro.

Characteristics of Chinese patients with Wegener's granulomatosis with anti-myeloperoxidase autoantibodies.

Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature.

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