Coal workers' pneumoconiosis: an Australian perspective.

Coal workers' pneumoconiosis (CWP) is an untreatable but preventable lung disease arising from chronic inhalation of coal dust. Recent reports of CWP in Queensland, along with international data, suggest that there is a resurgence in pneumoconiosis. The prevalence of CWP varies considerably between countries. In Australia, there is no mandatory reporting system and no national data on the prevalence of CWP. The symptoms and manifestations of CWP vary depending on the composition of the inhaled dust, duration of exposure, stage of disease and host-related factors. CWP may develop into progressive massive fibrosis (PMF), which can be fatal. Radiological assessment should be performed according to evidence-based standards using the ILO (International Labour Office) International Classification of Radiographs of Pneumoconioses. As preventing exposure to coal dust prevents CWP, it is important to implement and enforce appropriate standards limiting exposure. In Australia, these standards currently vary between states and are not in keeping with international understanding of the levels of coal dust that cause disease. Longitudinal screening programs are crucial for monitoring the health of coal workers to identify individuals with early-stage disease and prevent progression from mild disease to PMF. We recommend: standardisation of coal dust exposure limits, with harmonisation to international regulations; implementation of a national screening program for at-risk workers, with use of standardised questionnaires, imaging and lung function testing; development of appropriate training materials to assist general practitioners in identifying pneumoconiosis; and a system of mandatory reporting of CWP to a centralised occupational lung disease register.

Everolimus treatment of abdominal lymphangioleiomyoma in five women with sporadic lymphangioleiomyomatosis.

OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare systemic disease of young women arising from mutations in the tuberous sclerosis complex (TSC) genes, TSC1 or TSC2. This disrupts the mammalian target of rapamycin (mTOR) pathway, affecting cellular proliferation and growth. mTOR inhibitors are a promising novel therapy in LAM. The mTOR inhibitor sirolimus is reported to produce resolution of lymphatic abnormalities in LAM, but the efficiacy of the mTOR inhibitor everolimus has not been assessed. We aimed to examine the efficacy of everolimus on lymphatic abnormalities in LAM. DESIGN, SETTING AND PARTICIPANTS: Open-label treatment of five patients with sporadic LAM (sLAM) and abdominopelvic and lung involvement at the outpatient LAM clinic of a tertiary city teaching hospital. Clinical data were collected during treatment of the women and included regular clinical reviews, everolimus levels, lung function and computed tomography assessment before and after 6 months of everolimus treatment. MAIN OUTCOME MEASURES: Symptoms and level of resolution of lymphangioleiomyomas. RESULTS: All five women experienced significant shrinkage or complete resolution of the lymphangioleiomyomas during treatment. In one woman, cessation of everolimus resulted in recurrence of symptoms. Adverse events were compatible with the known side-effect profile of everolimus, but overall the drug was well tolerated. CONCLUSIONS: This is the first report to suggest that everolimus has efficacy in the treatment of lymphangioleiomyoma and chylous ascites in sLAM.

Investigating cystic lung disease: a respiratory detective approach.

The cystic lung diseases are rare orphan lung disorders that most physicians will see infrequently in their everyday practice. Diagnostic and treatment options have improved over recent decades, with opportunities for slowing rate of progression and improving outcome for patients. This review provides a summary of the clinical approach to these lung disorders, including how to differentiate between different imaging patterns, clinical features, differential diagnosis and characteristics of the commonest presenting disorders. EDUCATIONAL AIMS: To understand the clinical, pathological and radiological features of cystic lung disordersTo explore the differential diagnosis of cystic lung diseaseTo be familiar with the key features (clinical, radiological, physiological and pathological) of the commoner cystic lung diseases, which assist in differentiating between these.

Clinical Utility of Computer-Aided Diagnosis of Vertebral Fractures From Computed Tomography Images.

Osteoporotic vertebral compression fractures (VCFs) are a risk factor for morbidity and mortality, frequently asymptomatic and often present in computed tomography (CT) scans performed for unrelated conditions. Computer-aided diagnosis (CAD) of VCF from such images can potentially improve identification and treatment of osteoporosis. This single-blinded, single tertiary center study compared a CAD (Zebra Medical Vision®) to an adjudicated imaging specialist reevaluation using a retrospective consecutive sample of abdominal and thoracic CT scans (n = 2357) performed as part of routine care. Subjects over 50 years between January 1, 2019 and May 12, 2019 were included. Duplicates and unanalyzable scans were excluded resulting in a total of 1696 CT scans. The sensitivity, specificity, and accuracy were calculated for all VCF and for Genant grades 2 or 3 (ie, height loss of >25%) using imaging specialist as the gold standard. Prestudy VCF reporting by hospital-rostered radiologist was used to calculate the number of scans needed to screen (NNS) to detect one additional VCF using CAD. Prevalence of any VCF was 24% (406/1696) and of Genant 2/3 VCF was 18% (280/1570). The sensitivity and specificity were 54% and 92%, for all fractures, respectively, and 65% and 92% for Genant 2/3 fractures, respectively. Accuracy for any VCF, and for detection of Genant 2/3 VCF, was 83% and 88%, respectively. Of 221 CAD-detected VCFs, 133 (60.2%) were reported prestudy resulting in 88 additional fractures (72 Genant 2/3) being identified by CAD. NNS to detect one additional VCF was 19 scans for all fractures and 23 for Genant 2/3 fractures. Thus, the CAD tested in this study had a high specificity with moderate sensitivity to detect incidental vertebral fractures in CT scans performed for routine care. A low NNS suggests it is an efficient tool to assist radiologists and clinicians to improve detection and reporting of vertebral fractures. © 2020 American Society for Bone and Mineral Research (ASBMR).