Diagnosis and management of ANCA associated vasculitis

Berden, Annelies E.; Göçeroğlu, Arda; Jayne, David; Luqmani, Raashid; Rasmussen, Niels; Bruijn, Jan A.; Bajema, Ingeborg M.

doi:10.1136/bmj.e26

Cited by 109 publications

(97 citation statements)

References 62 publications

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“…In MPA, immune deposits on the vessel wall are usually absent, and serum antineutrophil cytoplasmic antibodies (ANCAs) are frequently present. MPA shares these characteristic features with other ANCA-associated vasculitides (AAVs) such as granulomatosis with polyangiitis (Wegener's) (GPA), eosinophilic GPA (Churg-Strauss), and renal limited vasculitis (1,2).…”

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confidence: 99%

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Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

Yamagata

Ikeda

Tsushima

et al. 2016

Arthritis & Rheumatology

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Objective. To determine the prevalence of lung abnormalities on chest computed tomography (CT) in patients with microscopic polyangiitis (MPA), to assess the responsiveness of such abnormalities to initial treatment, and to assess associations between these abnormalities and patient and disease characteristics and mortality.Methods. We retrospectively identified 167 consecutive hospital-based patients with MPA in 3 hospitals in Japan. We longitudinally collected clinical information for 150 of these patients, for whom CT images obtained before treatment were available. We then determined the presence of 22 imaging components of lung abnormalities in these patients.Results. The vast majority of patients (97%) had at least 1 lung abnormality on chest CT images, including interstitial lung lesions (66%), airway lesions (66%), pleural lesions (53%), and emphysematous lesions (37%). In multivariate analyses, ground-glass opacity was associated with the Birmingham Vasculitis Activity Score, whereas 3 of 4 airway lesions were associated with myeloperoxidase-antineutrophil cytoplasmic antibodies. Latent class analysis identified a distinct group of patients with airwaypredominant lung lesions. Airway lesions such as bronchiolitis and bronchovascular bundle thickening were among the components that showed improvement within 3 months of the initial treatment. An idiopathic pulmonary fibrosis pattern was the only chest CT variable that was independently associated with shorter survival.Conclusion. Abnormalities in a wide range of anatomic areas, including the whole airway, can be identified in the lungs of patients with MPA before treatment. The prevalence, clustering patterns, and responsiveness to treatment of individual lung abnormalities provide groundwork for informing future studies to understand the pathophysiology of MPA.Microscopic polyangiitis (MPA) is a necrotizing systemic vasculitis that affects small to medium-sized vessels. In MPA, immune deposits on the vessel wall are usually absent, and serum antineutrophil cytoplasmic antibodies (ANCAs) are frequently present. MPA shares these

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confidence: 99%

“…In patients with MPA, a wide range of organ systems can be affected (1)(2)(3). The lung is one of the major organs involved, and recent studies demonstrated that pulmonary involvement is associated with unfavorable outcomes (4,5).…”

mentioning

confidence: 99%

Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

Yamagata

Ikeda

Tsushima

et al. 2016

Arthritis & Rheumatology

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“…In general, rhinitis is the first sign in most people. 2,4 Our patient also had sinusitis , B/L lung nodulo cavitatory opacities along with proteinuria and digital gangrene of B/L lower limbs. The disease is highly associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinase 3 (PR3) or myeloperoxidase (MPO).…”

Section: Discussionmentioning

confidence: 68%

“…Its causes are unknown, although microbes, such as bacteria and viruses, as well as genetics have been implicated in its pathogenesis. 2,3 Inflammation with granuloma formation against a nonspecific inflammatory background is the classical tissue abnormality in all organs affected by GPA. 4 Initial signs are extremely variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms.…”

Section: Discussionmentioning

confidence: 99%

Digital gangrene a rare early manifestation of Wegener's granulomatosis: a case report

et al. 2016

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“…In this case, the IIF test was positive for cytoplasmic ANCA and the PR3 test was positive (although low) for anti-PR3. All 3 are strongly associated with GPA 4 .…”

Section: To the Editormentioning

confidence: 97%

A False-positive Antineutrophil Cytoplasmic Antibody Proteinase 3 Test in a Patient with Pulmonary-renal Syndrome

Agterhuis

Mulder²,

Roozendaal³

et al. 2014

J Rheumatol

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Diagnosis and management of ANCA associated vasculitis

Cited by 109 publications

References 62 publications

Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

Digital gangrene a rare early manifestation of Wegener's granulomatosis: a case report

A False-positive Antineutrophil Cytoplasmic Antibody Proteinase 3 Test in a Patient with Pulmonary-renal Syndrome

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