Charles Sharp

Rituximab in autoimmune connective tissue disease–associated interstitial lung disease

OBJECTIVEnCTD-associated interstitial lung disease (ILD) often fails to respond to conventional immunomodulatory agents. There is now considerable interest in the use of rituximab in systemic autoimmune CTD in patients refractory to standard treatments. The aim of this study was to review the experience of North Bristol NHS Trust managing patients with CTD-associated ILD with rituximab and explore possible associations with treatment response.nnnMETHODSnWe conducted a retrospective analysis of all patients who received rituximab under the Bristol CTD-ILD service, having failed to respond to other immunomodulatory treatments. Results were collated for pulmonary function and radiological outcomes before and after treatment.nnnRESULTSnTwenty-four patients were treated with rituximab. Their physiological parameters had failed to improve despite other immunomodulatory agents, with a mean change in forced vital capacity (FVC) prior to therapy of - 3.3% (95% CI - 5.6, -1.1) and mean change in diffusing capacity of carbon monoxide of - 4.3% (95% CI - 7.7, -0.9). After rituximab, radiology remained stable or improved for 11 patients, while worsening was observed in 9 patients. The decline in FVC was halted following treatment, with a mean change of + 4.1% (95% CI 0.9, 7.2), while diffusing capacity of carbon monoxide was stable [mean change +2.1% (95% CI - 1.0, 5.2)]. Patients with myositis overlap or antisynthetase syndrome appeared to respond well to treatment, with four patients showing clinically significant improvement in FVC >10%.nnnCONCLUSIONnRituximab is a therapeutic option in treatment-refractory CTD-associated ILD. Some disease subgroups may respond better than others, however, more work is needed to define its role in managing these patients.

Use of transbronchial cryobiopsy in the diagnosis of interstitial lung disease-a systematic review and cost analysis.

BackgroundnHistological diagnosis by surgical lung biopsy for interstitial lung disease (ILD) is currently limited. Transbronchial cryobiopsy via flexible bronchoscope may this for more patients. The relative costs, diagnostic yields and safety of this approach and more traditional approaches have not been determined.nnnObjectivesnTo perform a systematic review and meta-analysis of transbronchial cryobiopsy, forceps transbronchial biopsy and video assisted (VATS) surgical lung biopsy assessing their relative diagnostic yields and safety. To perform a cost analysis to demonstrate any savings through change to the newer technique.nnnMethodsnWe performed a systematic review of the literature using MEDLINE and EMBASE for all original articles on the diagnostic yield and safety of transbronchial cryobiopsy, forceps transbronchial biopsy and VATS-biopsy in ILD up to February 2016. Data were extracted on yield and complication rates, in addition to study characteristics. Theoretical cost analysis was performed from local institution financial data, 2015-16 reimbursement tariffs and results of the systematic review.nnnResultsnA meta-analysis of 11 investigations for transbronchial cryobiopsy, 11 for forceps transbronchial biopsy and 24 for VATS-biopsy revealed diagnostic yields of 84.4% (75.9-91.4%), 64.3% (52.6-75.1%) and 91.1% (84.9-95.7%), respectively. Pneumothorax occurred in 10% (5.4-16.1%) of transbronchial cryobiopsy procedures, moderate bleeding in 20.99% (5.6-42.8%), with three deaths reported. Surgical mortality was 2.3% (1.3-3.6%). Cost analysis demonstrated potential savings of £210 per patient in the first year and £647 in subsequent years.nnnConclusionsnTransbronchial cryobiopsy represents a potentially cost-saving approach to improve histological diagnosis in ILD, however is accompanied by a significant risk of moderate bleeding.

Duration of benefit following completion of pulmonary rehabilitation in interstitial lung disease—an observational study

Background: It remains unclear for how long the benefits of pulmonary rehabilitation (PR) last in interstitial lung disease (ILD). An increasing number of ILD patients complete PR and it is vital they be offered the most beneficial approaches. Methods: This is a retrospective, observational study of a cohort with ILD who had completed PR. Incremental shuttle walk (ISWT) and chronic respiratory disease questionnaire (CRDQ) were compared before PR, at course completion, and 6/12 months follow-up. Focus group discussions with ILD participants who had completed PR and their carers established qualitative views on existing and potential future PR provision. Results: 79 participants with ILD were identified at course completion, with 39 followed to 12 months. 11 participants died during follow-up. Initial benefits from PR were not sustained at 6 months (ISWT change 0.0m (95% CI-23.2 to 23.2 m), CRDQ change 2.5 (95% CI–2.4 to 7.4)) and 12 months (ISWT change–0.7 m (95% CI–37.3 to 35.9 m), CRDQ change 4.0 (95% CI–2.2 to 10.2)). Continued home exercise gave longer lasting benefit in exercise capacity. Focus group discussions highlighted the value attached to PR and suggested areas for improvement. Conclusions: Standard PR gives initial benefits in participants with ILD who complete the course, however these are not sustained. Tailored approaches to this group would be appreciated by this group and should be explored.

A comparison of published multidimensional indices to predict outcome in idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) has an unpredictable course and prognostic factors are incompletely understood. We aimed to identify prognostic factors, including multidimensional indices from a significant IPF cohort at the Bristol Interstitial Lung Disease Centre in the UK. Patients diagnosed with IPF between 2007 and 2014 were identified. Longitudinal pulmonary physiology and exercise testing results were collated, with all-cause mortality used as the primary outcome. Factors influencing overall, 12- and 24-month survival were identified using Cox proportional hazards modelling and receiver operating characteristic curve analysis. We found in this real-world cohort of 167 patients, diffusing capacity for carbon monoxide (DLCO) and initiation of long-term oxygen were independent markers of poor prognosis. Exercise testing results predicted 12-month mortality as well as DLCO, but did not perform as well for overall survival. The Composite Physiological Index was the best performing multidimensional index, but did not outperform DLCO. Our data confirmed that patients who experienced a fall in forced vital capacity (FVC) >10% had significantly worse survival after that point (p=0.024). Our data from longitudinal follow-up in IPF show that DLCO is the best individual prognostic marker, outperforming FVC. Exercise testing is important in predicting early poor outcome. Regular and complete review should be conducted to ensure appropriate care is delivered in a timely fashion. DLCO is a powerful prognostic marker in IPF http://ow.ly/EaEr307VTRN

UK trainee experience in interstitial lung disease: results from a British Thoracic Society survey

Interstitial lung disease (ILD) is a growing field of respiratory medicine in which novel therapies are emerging. It is important that trainees gain competence and confidence in this area. To explore the training experiences of specialty trainees, we conducted a survey of their practical experience and confidence in diagnosing and managing ILD.

Remote, Depth-Based Lung Function Assessment

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Toward Respiratory Assessment Using Depth Measurements from a Time-of-Flight Sensor

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Depth-Based Whole Body Photoplethysmography in Remote Pulmonary Function Testing

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P274 Cognitive function in idiopathic pulmonary fibrosis

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S20 Kbild scores have similar power to predict survival as pulmonary physiology in interstitial lung disease

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