Asbestos-related pleural and lung fibrosis in patients with retroperitoneal fibrosis

Orphanet Journal of Rare Diseases, Nov 2008

Background Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disease that leads to hydronephrosis and renal failure. In a case-control study, we have recently shown that asbestos exposure was the most important risk factor for RPF in the Finnish population. The aim of this study was to evaluate the relation of asbestos exposure to radiologically confirmed lung and pleural fibrosis among patients with RPF. Methods Chest high-resolution computed tomography (HRCT) was performed on 16 unexposed and 22 asbestos-exposed RPF patients and 18 asbestos-exposed controls. Parietal pleural plaques (PPP), diffuse pleural thickening (DPT) and parenchymal fibrosis were scored separately. Results Most of the asbestos-exposed RPF patients and half of the asbestos-exposed controls had bilateral PPP, but only a few had lung fibrosis. Minor bilateral plaques were detected in two of the unexposed RPF patients, and none had lung fibrosis. DPT was most frequent and thickest in the asbestos-exposed RPF-patients. In three asbestos-exposed patients with RPF we observed exceptionally large pleural masses that were located anteriorly in the pleural space and continued into the anterior mediastinum. Asbestos exposure was associated with DPT in comparisons between RPF patients and controls (case-control analysis) as well as among RPF patients (case-case analysis). Conclusion The most distinctive feature of the asbestos-exposed RPF patients was a thick DPT. An asbestos-related pleural finding was common in the asbestos-exposed RPF patients, but only a few of these patients had parenchymal lung fibrosis. RPF without asbestos exposure was not associated with pleural or lung fibrosis. The findings suggest a shared etiology for RPF and pleural fibrosis and furthermore possibly a similar pathogenetic mechanisms.

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Asbestos-related pleural and lung fibrosis in patients with retroperitoneal fibrosis

Toomas Uibu 2 Ritva Jrvenp 2 Jari Hakomki 2 Anssi Auvinen 1 Eero Honkanen 0 Kaj Metsrinne 5 Pekka Roto 4 Heikki Saha 2 Jukka Uitti 3 Panu Oksa 3 0 Department of Medicine, Division of Nephrology, Helsinki University Central Hospital , Helsinki , Finland 1 Tampere School of Public Health, University of Tampere , Tampere , Finland 2 Departments of Respiratory Medicine, Radiology, and Internal Medicine, Tampere University Hospital , Tampere , Finland 3 Clinic of Occupational Medicine, Tampere University Hospital and Finnish Institute of Occupational Health Tampere , Finland 4 Finnish Health Centers LTD , Tampere , Finland 5 Department of Internal Medicine, Turku University Hospital , Turku , Finland Background: Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disease that leads to hydronephrosis and renal failure. In a case-control study, we have recently shown that asbestos exposure was the most important risk factor for RPF in the Finnish population. The aim of this study was to evaluate the relation of asbestos exposure to radiologically confirmed lung and pleural fibrosis among patients with RPF. Methods: Chest high-resolution computed tomography (HRCT) was performed on 16 unexposed and 22 asbestos-exposed RPF patients and 18 asbestos-exposed controls. Parietal pleural plaques (PPP), diffuse pleural thickening (DPT) and parenchymal fibrosis were scored separately. Results: Most of the asbestos-exposed RPF patients and half of the asbestos-exposed controls had bilateral PPP, but only a few had lung fibrosis. Minor bilateral plaques were detected in two of the unexposed RPF patients, and none had lung fibrosis. DPT was most frequent and thickest in the asbestos-exposed RPF-patients. In three asbestos-exposed patients with RPF we observed exceptionally large pleural masses that were located anteriorly in the pleural space and continued into the anterior mediastinum. Asbestos exposure was associated with DPT in comparisons between RPF patients and controls (case-control analysis) as well as among RPF patients (case-case analysis). Conclusion: The most distinctive feature of the asbestos-exposed RPF patients was a thick DPT. An asbestos-related pleural finding was common in the asbestos-exposed RPF patients, but only a few of these patients had parenchymal lung fibrosis. RPF without asbestos exposure was not associated with pleural or lung fibrosis. The findings suggest a shared etiology for RPF and pleural fibrosis and furthermore possibly a similar pathogenetic mechanisms. - Background Retroperitoneal fibrosis (RPF), or Ormond's disease, is a rare condition with fibrosis covering the abdominal aorta and the ureters. The etiology of RPF is generally unknown. It has been proposed that approximately one-third of RPF cases develop secondarily to aortic aneurysm, abdominal infections or surgery and as a side effect of several drugs, especially methysergide and other ergot derivates [1-3]. Asbestos is known to cause diffuse pleural thickening (DPT) and parietal pleural plaques [4]. High-level asbestos exposure may lead to the development of clinically detectable lung fibrosis (asbestosis) [5]. We have recently shown that asbestos exposure is one of the most important single risk factors for RPF, accounting for approximately 20% of all RPF cases in the Finnish population [6,7]. The aim of this study was to determine whether RPF patients have pleural or lung fibrosis and to assess the relations between asbestos exposure and intrathoracic fibrotic changes in RPF patients. Furthermore we evaluated the susceptibility for pleural and lung fibrosis among asbestos-exposed RPF patients and asbestos-exposed controls. Subjects and methods Study population This material was part of our case-control study including 43 persons with RPF and 179 randomly assigned controls matched for year of birth, gender and central hospital district in Finland [6]. The diagnosis of RPF required the presence of the typical clinical conditionfibrosing mass covering the abdominal aorta and other retroperitoneal structuresand either histological confirmation (35 of 43 persons) or a follow-up of at least 1 year in order to rule out retroperitoneal malignancies (8 of 43 persons). All of the participants were interviewed for medical history and asbestos exposure. The cumulative exposure to asbestos dust was estimated using fiber-years (40-hour shift per week at an average dust level of 1 fiber/ml for 1 year) and graded as follows: no significant asbestos exposure; slight exposure (asbestos exposure <10 fiber-years) and moderate-to-high exposure (asbestos exposure 10 fiber-years). Exposure was assessed by an occupational health physician with special expertise in the evaluation of asbestos exposure, the physician was blinded in terms of the case-control status of the participants. The details of the data collection have been given in our previous report [6]. We asked all of the unexposed and exposed patients with RPF and the con (...truncated)


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Toomas Uibu, Ritva Järvenpää, Jari Hakomäki, Anssi Auvinen, Eero Honkanen, Kaj Metsärinne, Pekka Roto, Heikki Saha, Jukka Uitti, Panu Oksa. Asbestos-related pleural and lung fibrosis in patients with retroperitoneal fibrosis, Orphanet Journal of Rare Diseases, 2008, pp. 29, 3, DOI: 10.1186/1750-1172-3-29