Lung transplantation for silicosis and recovery: an Australian case study.

Lung transplantation is a well-established treatment for a variety of end-stage pulmonary diseases. However, the journey of a lung transplant recipient is complex and multifaceted. Silicosis is a rare indication for lung transplantation, but no other treatment is yet available for this disease in its end stages. This Australian case study presents a 52-year-old man with silicosis who received bilateral lung transplantation. The patient was frequently noncompliant with noninvasive ventilation therapy and experienced the complication of type 2 respiratory failure. Patient education and support provided, particularly around medication management following transplantation surgery, are discussed here. The patient's social situation and its implications for both him and his family are also considered.

Transplantation for work-related lung disease in the USA.

OBJECTIVES: Work-related lung diseases (WRLDs) are entirely preventable. To assess the impact of WRLDs on the US transplant system, we identified adult lung transplant recipients with a WRLD diagnosis specified at the time of transplant to describe demographic, payer and clinical characteristics of these patients and to assess post-transplant survival. METHODS: Using US registry data from 1991 to 2018, we identified lung transplant recipients with WRLDs including coal workers' pneumoconiosis, silicosis, asbestosis, metal pneumoconiosis and berylliosis. RESULTS: The frequency of WRLD-associated transplants has increased over time. Among 230 lung transplants for WRLD, a majority were performed since 2009; 79 were for coal workers' pneumoconiosis and 78 were for silicosis. Patients with coal workers' pneumoconiosis were predominantly from West Virginia (n=31), Kentucky (n=23) or Virginia (n=10). States with the highest number of patients with silicosis transplant were Pennsylvania (n=12) and West Virginia (n=8). Patients with metal pneumoconiosis and asbestosis had the lowest and highest mean age at transplant (48.8 and 62.1 years). Median post-transplant survival was 8.2 years for patients with asbestosis, 6.6 years for coal workers' pneumoconiosis and 7.8 years for silicosis. Risk of death among patients with silicosis, coal workers' pneumoconiosis and asbestosis did not differ when compared with patients with idiopathic pulmonary fibrosis. CONCLUSIONS: Lung transplants for WRLDs are increasingly common, indicating a need for primary prevention and surveillance in high-risk occupations. Collection of patient occupational history by the registry could enhance case identification and inform prevention strategies.

EBUS-TBNA is Sufficient for Successful Diagnosis of Silicosis with Mediastinal Lymphadenopathy.

BACKGROUND: Silicosis is an occupational lung disease resulting from inhalation of respirable crystalline silica. Recently, an international silicosis epidemic has been noted among artificial stone workers. OBJECTIVE: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is currently used for patients with unexplained lymphadenopathy. Since silicosis may present with prominent lymphadenopathy, the diagnostic yield of EBUS-TBNA in diagnosing silicosis was evaluated. METHODS: Twenty-eight patients with suspected silicosis referred for outpatient evaluation in three large tertiary hospitals were evaluated. Patients with mediastinal lymphadenopathy underwent EBUS-TBNA, while others underwent TBB and/or video-assisted thoracoscopic surgery (VATS). RESULTS: Eleven patients with mediastinal lymphadenopathy (39%) were evaluated using EBUS-TBNA. The diagnosis was accurate in all cases, demonstrating silica particles under polarized light, with no complications. Among the remaining patients, TBB was only 76% diagnostic, therefore requiring VATS. CONCLUSIONS: EBUS-TBNA is a useful and sufficient tool to diagnose silicosis in patients with mediastinal lymphadenopathy along compatible exposure histories.

What is expected in lung function after lung transplantation due to end-stage pulmonary silicosis?

In this study, we aimed to determine the impact of lung transplantation (LTx) on pulmonary function tests (PFTs) and survival among patients with end-stage silicosis. We included patients with end-stage silicosis on the wait list for LTx, between January 1989 and July 2015 (N = 26). Sixteen of these patients received LTx; 10 were eligible, but did not undergo LTx (non-LTx) during the study period. Retrospective information on PFTs (spirometry [volumes and flows], 6-minute walking test [6MWT], and DLCO) was retrieved from patients' medical charts, including baseline information for all patients and follow-up information for the LTx. At baseline, most patients presented with spirometric and 6MWT values that were suggestive of severe disease (FEV1 /FVC 76.5 ± 29.7; 6MWT 267.4 ± 104.5 m). Significant increases in these values were observed at follow-up in the LTx (P = .036 and .151, respectively). The overall median survival of patients in the LTx and non-LTx was 3.35 years (95% CI: 0.16-14.38) and 0.78 years (95% confidence interval [CI]: 0.12-3.65) (P = 0.002), respectively. For patients with end-stage silicosis, LTx offers significant benefits regarding pulmonary function and survival when compared to non-LTx, and is a reliable tool to help this critical population of patients, whose only treatment option is LTx.

Survival following lung transplantation for artificial stone silicosis relative to idiopathic pulmonary fibrosis.

BACKGROUND: Silicosis is a progressive lung disease resulting from the inhalation of respirable crystalline silica. Lung transplantation is the only treatment for end-stage silicosis. The aim of this study was to analyze the survival experience following lung transplantation among patients with silicosis. METHODS: We reviewed data for all patients who underwent lung transplantation for silicosis and a matched group undergoing lung transplantation for idiopathic pulmonary fibrosis (IPF) at a single medical center between March 2006 and the end of December 2013. Survival was followed through 2015. RESULTS: A total of 17 lung transplantations were performed for silicosis among 342 lung transplantations (4.9%) during the study period. We observed non-statistically significant survival advantage (hazard ratio 0.6; 95%CI 0.24-1.55) for those undergoing lung transplantation for silicosis relative to IPF patients undergoing lung transplantation during the same period. CONCLUSIONS: Within the limits of a small sample, survival in silicosis patients following lung transplantation was not reduced compared to IPF. Am. J. Ind. Med. 60:248-254, 2017. © 2017 Wiley Periodicals, Inc.

Radiological Evaluation of Artificial Stone Silicosis Outbreak: Emphasizing Findings in Lung Transplant Recipients.

OBJECTIVE: The aim of this study was to describe the computed tomography (CT) findings and correlate pulmonary function tests (PFTs) of silicosis patients with emphasis on the findings in lung transplantation (LTX) recipients. METHODS: We studied the chest CT scans from 82 marble workers exposed to artificial stone dust and who had a diagnosis of silicosis, of whom 13 patients underwent LTX. Silicosis-associated findings were graded and correlated to concomitant PFT. RESULTS: A statistically significant inverse relationship was found between chest CT scores and PFT including forced expired volume in the first second (r = -0.54, P < 0.0001), total lung capacity (r = -0.4, P < 0.0001), and diffusion capacity for carbon monoxide single breath % (r = -0.6, P < 0.0001) parameters. Progressive massive fibrosis indicating advanced and complicated silicosis was found in 85% of LTX patients, as compared with 40% in patients with maintained pulmonary function. Ground-glass opacities were seen in some LTX patients with or without signs of progressive massive fibrosis. Two of these patients had silicoproteinosis diagnosed within the resected lung, indicating an acute or accelerated form of silicosis. CONCLUSIONS: This silicosis current outbreak is important because of the worldwide use of this and similar high-silica-content, artificial stone products, which can cause progressive severe forms of silicosis. Along with standard clinical assessment and PFT, CT parameters are indicative measures of the disease severity.

[Lung transplantation for phase â ¢ silicosis: a series of 32 cases].

Objective: To evaluate the effect of lung transplantation for phase Ⅲ silicosis. Methods: From September 2002 to September 2015, 32 patients with end-stage silicosis underwent lung transplantation at Department of Thoracic Surgery, Affiliated Wuxi People's Hospital, Nanjing Medical University. There were 29 male and 3 female patients aged from 24 to 63 years. Thirty-two patients were diagnosed as phase Ⅲ silicosis by the local occupational disease prevention and control center. Fifteen patients were type Ⅰ respiratory failure and 17 patients were type Ⅱ. There were 14 cases accepted bilateral sequential lung transplantation and 18 cases accepted single lung transplantation, including 13 cases with right single lung transplantation and 5 cases with left single lung transplantation. Extracorporeal membrane oxygenation was used in 13 patients. Pulmonary function monitoring was performed at 3 months, 6 months, 1 year, and 2 years after lung transplantation. Clinical characteristics were compared using t-test, χ2 test and Fisher exact test between groups, Kaplan-Meier survival curve and Log-rank test were used to find out the factors affecting survival. Results: All the patients received lung transplantation successfully. One patient died of multiple organ failure, 1 died of sepsis, and 1 succumbed to sudden cardiac death. Twenty-nine patients were discharged from hospital. During follow-up, there were 5 deaths, two patients died of sepsis 7 months postoperatively, 1 died of renal failure 5 months post-transplant, 1 died of sudden cardiac death, and the remaining 1 patient died of bronchiolitis obliterans. Twenty-four patients lived a good quality of life, with survival rates of 90.6% at 3 months, 80.8% at 1 year, 76.7% at 3 years, and 76.7% at 5 years. Significant difference was not observed between single and bilateral lung transplantation about long-term survival rate. During follow-up pulmonary function post-transplant (3 months, 6 months, 1 year, and 2 years) were improved dramatically compared with preoperative level, and patients lived a good quality of life. Conclusion: Lung transplantation is beneficial for patients with phase Ⅲ silicosis, long-term survival is probable.

Long-Term Survival After Lung Transplantation in Patients with Silicosis and Other Occupational Lung Disease.

BACKGROUND: Survival after lung transplantation (LTx) for patients with occupational lung disease (OLD) is not well studied. METHODS: The United Network for Organ Sharing (UNOS) database was queried from 2005 to 2013 to assess survival after LTx in patients with silicosis and non-silicotic OLD compared to non-OLD patients. RESULTS: Of 7273 adult LTx recipients, 7227 (24 with silicosis and 29 with non-silicotic OLD) were included in our univariate and Kaplan-Meier function analysis and 6370 for multivariate Cox models. Univariate Cox models did not identify survival differences in silicosis (HR 0.717; 95 % CI 0.358-1.435; p = 0.347) and non-silicotic OLDs (HR 0.934; 95 % CI 0.486-1.798; p = 0.839). Kaplan-Meier function analysis did not identify a survival disadvantage for either silicosis or non-silicotic OLD (log-rank test: χ (2) 0.93, p = 0.627). Patients with non-silicotic OLD were at risk for worse survival for the first 2.5 years post-transplant; however, at the conclusion of the study, this group had the highest survival rate. Multivariate Cox models confirmed no increased risk for mortality for silicosis (HR 1.264; 95 % CI 0.631-2.534; p = 0.509) and non-silicotic OLD (HR 1.114; 95 % CI 0.578-2.147; p = 0.747). CONCLUSION: Long-term survival for adult patients with silicosis and non-silicotic OLD after LTx is not significantly different compared to the general lung transplant population.

[Optimization of cholecystectomy method in patients with professional diseases of respiratory organs].

Results of cholecystectomy performance were analyzed in 99 patients with a biliary calculous disease, the course of which have become complicated by cholecystitis occurrence, in 49 of them professional diseases of respiratory organs, including silicosis, antracosis, were registered. Estimating results of laparoscopic and open cholecystectomy, we have established, that carboperitoneum in laparoscopic cholecystectomy impacts negatively the course of early postoperative period, what manifests with hemodynamic and respiratory disorders. The level of fibronectin and endotelin--1 in the blood plasm directly depends on the respiratory disorders severity and may be applied as a test for respiratory dysfunction. The method of cholecystectomy in patients, suffering professional diseases of respiratory organs, must be selected, depending on severity of functional disorders of respiratory organs.

Repeated administration of bone marrow-derived cells prevents disease progression in experimental silicosis.

BACKGROUND/AIMS: Bone marrow-derived cells (BMDCs) reduced mechanical and histologic changes in the lung in a murine model of silicosis, but these beneficial effects did not persist in the course of lung injury. We hypothesized that repeated administration of BMDCs may decrease lung inflammation and remodeling thus preventing disease progression. METHODS: One hundred and two C57BL/6 mice were randomly divided into SIL (silica, 20 mg intratracheally [IT]) and control (C) groups (saline, IT). C and SIL groups were further randomized to receive BMDCs (2×10(6) cells) or saline IT 15 and 30 days after the start of the protocol. RESULTS: By day 60, BMDCs had decreased the fractional area of granuloma and the number of polymorphonuclear cells, macrophages (total and M1 phenotype), apoptotic cells, the level of transforming growth factor (TGF)-ß' and types I and III collagen fiber content in the granuloma. In the alveolar septa, BMDCs reduced the amount of collagen and elastic fibers, TGF-ß, and the number of M1 and apoptotic cells. Furthermore, interleukin (IL)-1ß, IL-1R1, caspase-3 mRNA levels decreased and the level of IL-1RN mRNA increased. Lung mechanics improved after BMDC therapy. The presence of male donor cells in lung tissue was not observed using detection of Y chromosome DNA. CONCLUSION: repeated administration of BMDCs reduced inflammation, fibrogenesis, and elastogenesis, thus improving lung mechanics through the release of paracrine factors.

Survival following lung transplantation for silicosis and other occupational lung diseases.

BACKGROUND: Information is scant assessing outcomes in lung transplantation (LT) in advanced occupational lung diseases (OLD). AIMS: To analyse survival after LT for OLD. METHODS: Using data from the US Organ Procurement and Transplantation Network Registry (OPTN-R), we identified subjects aged ≥ 18 years transplanted for OLD from 2005 to 2010. OPTN-R selected referents of corresponding age, sex and body mass index (BMI) who underwent LT for other diagnoses were also identified. Post-LT survival time was estimated with Cox proportional hazard models. Baseline age, BMI, forced expiratory volume in 1 s, creatinine, lung allocation score, donor age, donor lung ischaemic time and transplant type (single versus bilateral) were included as covariates. Time-dependent covariates were used to model differences in relative risk over time. RESULTS: Thirty-seven males underwent LT for silicosis (n = 19) or other OLD (n = 18) during the analytic period (0.5% of all LTs). For non-silicotic OLD, 6-month and 1- and 3-year survival estimates were 66, 55 and 55%, compared with the silicotic group (86, 86 and 76%) and referent group (89, 84 and 67%). During the first year post-transplant, those with OLD (silicosis and others combined) manifested an overall 2-fold increased mortality risk [hazard ratio (HR) 2.3, 95% CI 1.3-4.4; P < 0.05] compared to referents. In stratified analysis, this increased risk of death was restricted to those with non-silicotic OLD (HR 3.1, 95% CI 1.5-6.6; P < 0.01). Poorer survival was limited to the first year post-LT. CONCLUSIONS: Subjects undergoing LT for OLD other than silicosis may be at increased risk of death in the first year post-transplantation.

[Lung transplantation for silicosis: a report of 5 cases].

OBJECTIVE: To investigate the selection of recipients, operative technique, and perioperative management of lung transplantation for silicosis. METHODS: Lung transplantations (LTx) were performed for five end-stage silicosis in our hospital who were diagnosed in accordance with recommendations of the local Prophylactic Therapeutic Institution for Occupational Diseases. The chest roentgenogram and high resolution CT showed somewhat pulmonary interstitial fibrosis, pulmonary emphysema and massive opacities. The mean pulmonary artery pressure (mPAP) was > 30mmHg, NYHA III or IV. Two patients received thoracic surgery prior to LTx, one patient was ventilator-dependent. One patient received bilateral sequence lung transplantation (BSLT) under extracorporeal membrane oxygenation (ECMO) support. Four patients received single lung transplantation (SLT), 3 under ECMO support. RESULTS: Patient five died of multiple organ failure on postoperative day 8, the remaining four patients were discharged from hospital. During follow up, patient three died of severe infection 7 month postoperatively, the remaining three patients were alive for 5 years, 3 years and 2 years respectively, and lived good quality of life, especially with lower mPAP and improved lung function. Although our patients suffered low-grade chronic rejection with the manifestation of bronchiolitis obliterative syndrome (BOS). CONCLUSION: Lung transplantation is a viable option for patients with end-stage silicosis, providing acceptable quality of life and survival. Both SLT and BSLT are satisfactory approach for end-stage silicosis,and long-term survival requires further investigations.

[Investigating the treatment of silicosis with autologous bone marrow-derived mesenchymal stem cells].

OBJECTIVE: To explore the safety and curative effects of autologous bone marrow-derived mesenchymal stem cells (BMSCs) in the treatment of silicosis. METHODS: The protocol was approved by the Ethics Committee of the hospital, and ten patients with silicosis who had given written consent were enrolled in this study. BMSCs isolated from 100 ml of bone marrow for each case were purified and cultured. In each case the 3rd generation of qualified BMSCs (5 × 10(7)) were intravenously administered weekly for 3 weeks. Three cases among 10 patients were treated with BMSCs modified by hepatocyte growth factor (HGF) gene. The clinical symptoms, chest films, chest CT, pulmonary functions, T cells, serum IgG and ceruloplasmin (CP) were observed in 6 or 9 months after treatment. RESULTS: No obvious sub-effect was observed in cases treated with BMSCs, the clinical symptoms (such as cough, sputum and chest tightness) basically disappeared in 9 months after treatment. Pulmonary function tests showed that FVC increased from 71.2% ± 17.0% to 84.0% ± 10.9% (P < 0.01) and FEV1.0 increased from 67.5% ± 17.7% to 80.6% ± 14.9% (P < 0.01). The levels of serum CP and IgG significantly decreased (P < 0.01). Further, the chest films and CT in cases treated with autologous BMSCs modified by HGF gene were improved to different extent. CONCLUSION: Treatment with autologous BMSCs modified by HGF gene exhibit a beneficial effect on silicosis.

Primary cutaneous mucormycosis in a lung transplant recipient: case report and concise review of the literature.

Mucormycosis (zygomycosis) is an invasive, opportunistic fungal infection caused by organisms of the class Zygomycetes. Immunocompromised individuals, including both solid organ and hematopoietic stem cell transplant recipients, are preferentially affected. Among solid organ transplant (SOT) recipients, the sinuses, with or without involvement of the orbits and cerebrum, are the most common sites of disease, although the pulmonary allograft appears to be targeted following lung transplantation. Here, we describe the unique case of a lung transplant recipient who developed multifocal cutaneous mucormycosis without involvement of the pulmonary allograft, and review the published literature regarding incidence, treatment, and prognosis of primary cutaneous mucormycosis following SOT.

[Working as a sandblaster can cause silicosis]. / Arbejde med sandblæsning kan give lungesilikose.

Silicosis is a common occupational disease worldwide. It is caused by the inhalation of crystalline silicon dioxide, i.e. silica. Quartz is a common form of silica and occurs in sandstone and granite. Occupational exposure can occur e.g. in mining, quarrying and sandblasting. The inhaled silica triggers an inflammatory response when phagocytosed which eventually causes fibrosis. We present a 45-year-old male Danish sandblaster who developed silicosis, and due to rapid decline in lung function received a lung transplant with an excellent result.

Burst abdomen: an unusual complication of silicosis in pregnancy.

We present a case of silicosis in a 37-year-old pregnant woman, a second gravida with previous caesarean section. She was referred to our hospital at 42 weeks of gestation with breathlessness and oligohydramnios. She had worked in a glass and talc powder factory for 11 years and was diagnosed as having silicosis 2 years prior; she was on treatment. Following admission, she was evaluated for dyspnoea and underwent emergency Caesarean section for poor cervical dilation. She developed a burst abdomen on the third postoperative day with loops of gangrenous bowel protruding outside the abdomen. Emergency laparotomy with ileal resection and ileostomy was carried out. She was discharged on day 14 and is on follow-up. To the best of our knowledge, this is the first case of silicosis in pregnancy presenting with an unusual complication.

Single lung transplantation for end-stage silicosis: report of a case. Lung Transplant Group.

After successful animal studies since 1986, a single left lung transplantation was performed on a 35-year-old male patient with end-stage silicosis in July 1991. The surgical technique was similar to that used by the Toronto Transplant Group, except for some modification in bronchial anastomosis. The donor lung was preserved by simple surface cooling after the administration of heparin, methylprednisolone and PGE1. The ischemic time for the donor lung was 3.5 hours. A cardiopulmonary bypass through the femoral vessels was applied for a duration of 90 minutes. Immediate postoperative complications included massive bleeding, disseminated intravascular coagulation, adult respiratory distress syndrome and acute graft rejection. Fortunately, we overcame these complications through intensive care. Immunosuppression included antilymphocytic globulin, cyclosporine, azathioprine and corticosteroids. The results of this single lung transplantation were satisfactory. The patient was doing well and was able to satisfactorily breathe room air six weeks after the transplantation. Unfortunately, the patient died of opportunistic systemic aspergillosis six months after the transplantation. In conclusion, lung transplantation is an effective treatment for patients with end-stage lung diseases, and the results of this first single lung transplantation in Taiwan are encouraging and promising.