Combined pulmonary fibrosis and emphysema associated with microscopic polyangiitis

Τzouvelekis, Αrgyris; Zacharis, George; Oikonomou, Anastasia; Koulelidis, Andreas; Steiropoulos, Paschalis; Froudarakis, Marios; Kriki, Pelagia; Vargemezis, Vassilios; Bouros, Demosthenes

doi:10.1183/09031936.00216311

Cited by 25 publications

(18 citation statements)

References 9 publications

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“…( 3 ) Later on, CPFE was described in patients with other ILDs, such as connective tissue disease (CTD)-associated ILD, ( 4 - 7 ) as well as in patients with microscopic polyangiitis. ( 8 ) …”

Section: Introductionmentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

et al. 2014

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Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

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Section: Introductionmentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

et al. 2014

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“…Even more intriguingly, a previous histopathologic analysis showed that a wide variety of extravascular pulmonary injuries, including airway lesions, can be observed in patients with AAVs (9). In addition, combined pulmonary fibrosis with emphysema (CPFE) was recently reported in patients with connective tissue diseases (10,11) and in patients with MPA or myeloperoxidase (MPO)-ANCA positivity (12,13), suggesting the more complex picture of pulmonary involvement in MPA. However, the prevalence of individual components of pulmonary involvement in consecutive patients with MPA, including those without apparent pulmonary manifestations, has not been reported.…”

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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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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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“…Increased MPO on activated neutrophils promote degranulation and oxidative bursts that could lead to alveolar epithelial damage, fibroblastic proliferation and ultimately the development of fibrosis [8]. Another conceivable mechanism is that at early stages of the disease, ANCA levels remain below the threshold of conventional laboratory measurements and once the levels rise and become detectable by laboratory methods, the disease manifest itself clinically, with features of vasculitis [9]. However the reason for this remains beyond cogitation and may be related to unidentified infectious or noninfectious antigens, autoimmunity, impaired regenerative responses, and the antagonistically pleiotropic action of genes involved in wound healing or development [10,11].…”

Section: Discussionmentioning

confidence: 99%

Idiopathic pulmonary fibrosis and p-ANCA positivity predating manifestations of systemic vasculitis

Khan¹,

Fitzgerald²,

Harrison³

et al. 2015

Pulmonol Respir Res

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The occurrence of perinuclear anti-neutrophilic cytoplasmic antibodies (p-ANCA) in pulmonary fibrosis has been reported in a number of small studies, however, the appearance of p-ANCA in already established idiopathic pulmonary fibrosis (IPF), predating the manifestations of vasculitis has not been widely appreciated. We report 2 cases with radiographic evidence of established IPF where p-ANCA was negative at the time of diagnosis and became positive during the course of the disease, for a variable period of time, prior to the subsequent development of systemic vasculitis. Our cases suggest a possible rationale for serial p-ANCA measurements in patients with pulmonary fibrosis even in the absence of features suggestive of vasculitis. Furthermore, the required frequency of ANCA measurement in the absence of clinical features of vasculitis is unknown. Larger studies with repetitive p-ANCA measurement in tandem with ongoing clinical assessment for vasculitis are required to assess the prevalence of ANCA and to confirm our observation.

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Combined pulmonary fibrosis and emphysema associated with microscopic polyangiitis

Cited by 25 publications

References 9 publications

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

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

Idiopathic pulmonary fibrosis and p-ANCA positivity predating manifestations of systemic vasculitis

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