Pulmonary Interstitial Fibrosis as a Presenting Manifestation in Perinuclear Antineutrophilic Cytoplasmic Antibody Microscopic Polyangiitis

Eschun, Gregg; Mink, Steven N.; Sharma, Sat

doi:10.1378/chest.123.1.297

Cited by 93 publications

(56 citation statements)

References 13 publications

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“…Interstitial lung disease is an underappreciated manifestation of MPA (3)(4)(5)(6). Two American Thoracic Society consensus state- ments on the diagnosis of idiopathic interstitial pneumonias have emphasized the importance of excluding "collagen vascular disease" through clinical and laboratory features (8,11), but neither these consensus statements nor several review articles explicitly list MPA or any other form of vasculitis in the differential diagnosis of idiopathic interstitial pneumonia (12)(13)(14).…”

Section: Discussionmentioning

confidence: 99%

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Microscopic polyangiitis presenting as a “pulmonary‐muscle” syndrome: Is subclinical alveolar hemorrhage the mechanism of pulmonary fibrosis?

Birnbaum¹,

Danoff²,

Askin³

et al. 2007

Arthritis & Rheumatism

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Microscopic polyangiitis (MPA) may present with a syndrome that resembles idiopathic pulmonary fibrosis (IPF). We describe an MPA patient with the clinical presentation of a “pulmonary‐muscle” syndrome in which interstitial lung disease antedated the onset of myopathy. Identification of vasculitis on muscle biopsy was instrumental in recognizing clinical, radiographic, and histopathologic features that were more characteristic of MPA than of IPF. Institution of glucocorticoid and cyclophosphamide therapy led to the induction of a complete remission. The histologic findings in this case implicate subclinical episodes of alveolar hemorrhage as the mechanism of interstitial lung disease in MPA.

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Section: Discussionmentioning

confidence: 99%

“…The classic form of pulmonary involvement is alveolar hemorrhage caused by pulmonary capillaritis. Interstitial lung disease may be more common in MPA than generally appreciated (3)(4)(5)(6), but its pathophysiology remains poorly understood.…”

Section: Is Subclinical Alveolar Hemorrhage the Mechanism Of Pulmonarmentioning

confidence: 99%

Microscopic polyangiitis presenting as a “pulmonary‐muscle” syndrome: Is subclinical alveolar hemorrhage the mechanism of pulmonary fibrosis?

Birnbaum¹,

Danoff²,

Askin³

et al. 2007

Arthritis & Rheumatism

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show abstract

“…While cutaneous, gastrointestinal, musculoskeletal and neurological manifestations can be observed in MPA, the most typical manifestations include rapidly progressive glomerulonephritis and pulmonary involvement. Classical pulmonary involvement consists of alveolar haemorrhage secondary to pulmonary capillaritis [1,2]. Pulmonary fibrosis is also a potentially severe manifestation of MPA, but mild pulmonary fibrosis is significantly associated with an increased rate of mortality [2][3][4].…”

mentioning

confidence: 99%

The oxidation induced by antimyeloperoxidase antibodies triggers fibrosis in microscopic polyangiitis

Guilpain¹,

Chéreau²,

Goulvestre³

et al. 2010

European Respiratory Journal

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Lung fibrosis is considered a severe manifestation of microscopic polyangiitis (MPA). Antimyeloperoxidase (anti-MPO) antibodies in MPA patients' sera can activate MPO and lead to the production of reactive oxygen species (ROS). While high levels of ROS are cytotoxic, low levels can induce fibroblast proliferation. Therefore, we hypothesised that the oxidative stress induced by anti-MPO antibodies could contribute to lung fibrosis.24 MPA patients (45 sera) were enrolled in the study, including nine patients (22 sera) with lung fibrosis. Serum advanced oxidation protein products (AOPP), MPO-induced hypochlorous acid (HOCl) and serum-induced fibroblast proliferation were assayed.AOPP levels, MPO-induced HOCl production and serum-induced fibroblast proliferation were higher in patients than in healthy controls (p,0.0001, p50.0001 and p50.0005, respectively). Increased HOCl production was associated with active disease (p50.002). Serum AOPP levels and serum-induced fibroblast proliferation were higher in patients with active MPA and lung fibrosis (p,0.0001). A significant linear relationship between fibroblast proliferation, AOPP levels and HOCl production was observed only in patients with lung fibrosis.Oxidative stress, in particular the production of HOCl through the interaction of MPO with anti-MPO antibodies, could trigger the fibrotic process observed in MPA.

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“…Of the 59 wellidentified cases in the literature with pulmonary fibrosis and MPA, PF had been manifested prior to the development of MPA in 23, MPA preceded the presence of PF in 1 and both conditions were identified concurrently in 35 [6,[11][12][13][14][15][16]. Nevertheless, it is possible that, in patients in whom both diseases were diagnosed at the same time, PF might have been present earlier but not clinically apparent.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary fibrosis presenting as an early manifestation of microscopic polyangiitis

Eleftheriou

Katsenos

Zorbas

et al. 2015

Monaldi Arch Chest Dis

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Microscopic polyangiitis (MPA) is a systemic small vessel vasculitis that is included in the pulmonary-renal syndromes. Although glomerulonephritis represents the major clinical feature of MPA indicative of renal involvement, diffuse alveolar haemorrhage is the classic manifestation of pulmonary involvement. However, pulmonary fibrosis is a less frequently reported pulmonary manifestation. Herein we describe a patient who was diagnosed with MPA presenting with radiographic evidence of pulmonary interstitial fibrosis as an early clinical manifestation accompanied by constitutional symptoms such as fever and weight loss. We also include a short literature review focusing on the association between pulmonary fibrosis and MPA.

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Pulmonary Interstitial Fibrosis as a Presenting Manifestation in Perinuclear Antineutrophilic Cytoplasmic Antibody Microscopic Polyangiitis

Cited by 93 publications

References 13 publications

Microscopic polyangiitis presenting as a “pulmonary‐muscle” syndrome: Is subclinical alveolar hemorrhage the mechanism of pulmonary fibrosis?

Microscopic polyangiitis presenting as a “pulmonary‐muscle” syndrome: Is subclinical alveolar hemorrhage the mechanism of pulmonary fibrosis?

The oxidation induced by antimyeloperoxidase antibodies triggers fibrosis in microscopic polyangiitis

Pulmonary fibrosis presenting as an early manifestation of microscopic polyangiitis

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