Microscopic polyangiitis associated with pulmonary fibrosis

Casares, Marcelo Fernández; González, Alejandra; Fielli, Mariano; Caputo, Flavia; Bottinelli, Yanina; Zamboni, Marcelo

doi:10.1007/s10067-014-2676-1

Cited by 47 publications

(78 citation statements)

References 20 publications

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“…PF has been reported in 23% of GPA cases [52] and in 2.7–45% of MPA patients. In these cases, interstitial disease was usually identified at disease onset [7,11,17–20,48,53,54]. Importantly, there is a significant predominance of MPO-ANCA in published series, i.e., 46–71% of all cases compared to 0–29% for PR3-ANCA [13,27,51,52,54–56].…”

Section: Epidemiologymentioning

confidence: 99%

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Interstital lung disease in ANCA vasculitis

Alba

Flores‐Suárez

Henderson

et al. 2017

Autoimmunity Reviews

127

135

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Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are immune-mediated disorders that primarily affect small blood vessels of the airway and kidneys. Lung involvement, one of the hallmarks of microscopic polyangiitis and granulomatosis with polyangiitis, is associated with increased mortality and morbidity. In recent years, several retrospective series and case reports have described the association of interstitial lung disease (ILD) and ANCA vasculitis, particularly those positive for ANCA specific for myeloperoxidase. In the majority of these patients pulmonary fibrosis occurs concurrently or predates the diagnosis of ANCA vasculitis. More importantly, these studies have shown that ILD has an adverse impact on the long-term prognosis of ANCA vasculitis. This review focuses on the main clinical and radiologic features of pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibodies. Major histopathology features, prognosis and therapeutic options are summarized.

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Section: Epidemiologymentioning

confidence: 99%

“…Of relevance, frequency of ILD in ANCA vasculitis seems to be higher in Japan than in Western populations [17,18,20,54,55,60]. Reasons for this particular association may include a higher prevalence of MPO-ANCA positivity and increased frequency of lung involvement and diffuse alveolar hemorrhage [11,61,62].…”

Section: Epidemiologymentioning

confidence: 99%

Interstital lung disease in ANCA vasculitis

Alba

Flores‐Suárez

Henderson

et al. 2017

Autoimmunity Reviews

127

135

View full text Add to dashboard Cite

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“…Pulmonary fibrosis may precede other disease manifestations by a range of 5-108 months, but would not occur after the diagnosis of MPA [65,67]. The prognosis of MPA with pulmonary fibrosis is worse than that of MPA without pulmonary fibrosis and seems to be better than that of patients with idiopathic pulmonary fibrosis [65,67,68].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

“…In a long-term retrospective study over a 15-year period in 28 patients with MPA, pulmonary fibrosis was identified in 9 patients (32%) and DAH was present in 11 cases (39%). A definite or possible usual interstitial pneumonia pattern was present in 8 cases (88%) and was associated with poor prognosis [65]. In another study of interstitial pneumonia with anti-myeloperoxidase-ANCA, 8 patients had a usual interstitial pneumonia pattern accompanied by areas of nonspecific interstitial pneumonia pattern and 1 patient had diffuse alveolar damage [66].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

“…In another study of interstitial pneumonia with anti-myeloperoxidase-ANCA, 8 patients had a usual interstitial pneumonia pattern accompanied by areas of nonspecific interstitial pneumonia pattern and 1 patient had diffuse alveolar damage [66]. Pulmonary fibrosis may precede other disease manifestations by a range of 5-108 months, but would not occur after the diagnosis of MPA [65,67]. The prognosis of MPA with pulmonary fibrosis is worse than that of MPA without pulmonary fibrosis and seems to be better than that of patients with idiopathic pulmonary fibrosis [65,67,68].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

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The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.

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Reduced survival in microscopic polyangiitis patients with pulmonary fibrosis in a respiratory referral centre

et al. 2015

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We read with interest the article by Fernández-Casares et al. [1]. Pulmonary fibrosis (PF) seems to be a growing complication arising during the long-term evolution of microscopic polyangiitis (MPA). It seems, though, as when compared to idiopathic pulmonary fibrosis, it holds still a better prognosis than the latter [2].We want to share our observations regarding survival and differences in patients with MPA who developed PF or not. As in the case of the above-cited report, our patients attend a respiratory centre. Since November 2013 and until December 2014, 40 patients (mean age 54.2 years) have been diagnosed with MPA (according to the 2012 Chapel Hill Nomenclature Consensus) [3] at our institution. Ninety percent were MPO-ANCA, 5 % PR3-ANCA and 2.5 % double positive by indirect immunofluorescence. At the time of last follow-up since first symptoms of disease (median 43 months, range 11-213), 17 (42.5 %, nine males and eight females) have developed PF (two were histologically proven, and the rest was clinically and tomographically compatible with usual interstitial pneumonia [4]), with 14 developing PF prior to other MPA manifestations. Main clinical differences were as follows: gender (18 females without PF and 8 with PF; p=0.04), a higher frequency of myalgias (9 vs 3; p=0.008), nasal crusting and peripheral neuropathy (3 vs 0 for both findings; p=0.02), a lower frequency of diffuse alveolar haemorrhage (6 vs 15; p= 0.04), acute respiratory failure (1 vs 8; p=0.02), haematuria (6 vs 13; p=0.003) and lymphopaenia (0 vs 5; p=0.01) in those with PF. The latter also had higher Hb at presentation (mean 12.1 vs 10.6 g/dL; p=0.047), lower ESR (mean 18.8 vs 30.8; p=0.02) and higher damage as measured by the Vasculitis Damage Index [5] (mean 4.6 vs 2.6; p=0.008). At diagnosis, activity (BVAS score) [6] did not differ.By means of Kaplan-Meier analysis, we found that survival is decreased in those who develop PF by almost half. As shown in the figure, considering either the time of first MPA symptom (Fig. 1a) or the time from diagnostic confirmation (Fig. 1b), those with PF have shorter life expectancy than those without. Median survival of the whole group was 98 months, 104 for those with PF and 212 for those without. There were nine deaths, seven with PF and two without PF.These observations are in accordance with previous reports from different parts of the world, including the recent one from Latin America [1,7,8], in which it has also been observed that an important percentage of patients may present with PF before other disease characteristics appear [7]. The influence of ethnics on such outcome seems minimal, although a larger number of patients and longer follow-up is needed to conclusively state this. It is interesting that those without PF were predominantly females. Gender was independent from others not found to be distinct (laboural, tobaccoism) which may predispose males to develop PF more frequently. In trying to establish factors leading to MPA development in PF patients, eosinophils in BAL and low att...

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Microscopic polyangiitis associated with pulmonary fibrosis

Cited by 47 publications

References 20 publications

Interstital lung disease in ANCA vasculitis

Interstital lung disease in ANCA vasculitis

The Respiratory System in Autoimmune Vascular Diseases

Reduced survival in microscopic polyangiitis patients with pulmonary fibrosis in a respiratory referral centre

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