Imaging of Lung Disease Associated with Connective Tissue Disease.

There is a well-known association between the connective tissue disorders (CTDs) and lung disease. In addition to interstitial lung disease, the CTDs may affect the air spaces and pulmonary vasculature. Imaging tests are important not only in diagnosis but also in management of these complex disorders. In the present review, key aspects of the imaging of CTD-reated diseases are discussed.

Identifying causation in hypersensitivity pneumonitis: a British perspective.

Background: Establishing whether patients are exposed to a 'known cause' is a key element in both the diagnostic assessment and the subsequent management of hypersensitivity pneumonitis (HP). Objective: This study surveyed British interstitial lung disease (ILD) specialists to document current practice and opinion in relation to establishing causation in HP. Methods: British ILD consultants (pulmonologists) were invited by email to take part in a structured questionnaire survey, to provide estimates of demographic data relating to their service and to rate their level of agreement with a series of statements. A priori 'consensus agreement' was defined as at least 70% of participants replying that they 'Strongly agree' or 'Tend to agree'. Results: 54 consultants took part in the survey from 27 ILD multidisciplinary teams. Participants estimated that 20% of the patients in their ILD service have HP, and of these, a cause is identifiable in 32% of cases. For patients with confirmed HP, an estimated 40% have had a bronchoalveolar lavage for differential cell counts, and 10% a surgical biopsy. Consensus agreement was reached for 25 of 33 statements relating to causation and either the assessment of unexplained ILD or management of confirmed HP. Conclusions: This survey has demonstrated that although there is a degree of variation in the diagnostic approach for patients with suspected HP in Britain, there is consensus opinion for some key areas of practice. There are several factors in clinical practice that currently act as potential barriers to identifying the cause for British HP patients.

The King's Brief Interstitial Lung Disease (KBILD) questionnaire: an updated minimal clinically important difference.

Introduction: The King's Brief Interstitial Lung Disease (KBILD) is a 15-item validated health-related quality of life (HRQOL) questionnaire. The method of scoring the KBILD has recently changed to incorporate a logit-scale transformation from one that used raw item responses, as this is potentially a more linear scale. The aim of this study was to re-evaluate the KBILD minimal clinically important difference (MCID) using the new logit -transformed scoring. Methods: 57 patients with interstitial lung disease (17 idiopathic pulmonary fibrosis, IPF) were asked to complete the KBILD questionnaire on two occasions in outpatient clinics. At the second visit, patients also completed a 15-item global rating of change of health status questionnaire (GRCQ). The MCID was calculated as the mean of four different methods: the change in KBILD for patients indicating a small change in GRCQ, patients with a 7%-12% change in FVC, 1 SE of measurement of baseline KBILD and effect size (ES) of 0.3. Results: The mean (SD) KBILD total score for all patients was 55.3 (15.6). 16 patients underwent a therapeutic intervention. 36 patients reported a change in their condition on the GRCQ; 22 deteriorated, 14 improved and 21 were unchanged. There was a significant change in KBILD total score in patients reporting a change in GRCQ; mean (SD) 57.0 (13.6) versus 50.0 (9.7); mean difference 7.0; 95% CI of difference 3.0 to 11.0; p<0.01. The change in KBILD total score correlated with the GRCQ scale; r=-0.49, p<0.01. The mean KBILD total score MCID was 5. The MCID of KBILD domains were 6 for Psychological, 7 for Breathlessness and Activities, and 11 for Chest Symptoms. Conclusion: The KBILD is a responsive tool for longitudinal assessment of HRQOL in patients with ILD. The MCID of the KBILD total score is a 5-unit change.

Safety and tolerability of nintedanib for the treatment of idiopathic pulmonary fibrosis in routine UK clinical practice.

In IPF, commencement of antifibrotic therapy in patients with preserved lung volume may increase the duration of therapy http://ow.ly/4HeM30lFS67.

Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia.

RATIONALE: Pulmonary hypertension (PH) associated with fibrotic idiopathic interstitial pneumonia (IIP; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) confers important additional morbidity and mortality. OBJECTIVES: To evaluate the safety and clinical efficacy of the dual endothelin-1 receptor antagonist bosentan in this patient group. METHODS: In a randomized, double-blind, placebo-controlled study, 60 patients with fibrotic IIP and right heart catheter confirmed PH were randomized 2:1 to bosentan (n = 40) or placebo (n = 20). The primary study endpoint was a fall from baseline pulmonary vascular resistance index (PVRi) of 20% or more over 16 weeks. MEASUREMENTS AND MAIN RESULTS: Sixty patients (42 men; mean age, 66.6 ± 9.2 yr), with a mean pulmonary artery pressure of 36.0 (± 8.9) mm Hg, PVRi 13.0 (± 6.7) Wood Units/m(2) and reduced cardiac index of 2.21 (± 0.5) L/min/m(2) were recruited to the study. Accounting for deaths and withdrawals, paired right heart catheter data were available for analysis in 39 patients (bosentan = 25, placebo = 14). No difference in the primary outcome was detected, with seven (28.0%) patients receiving bosentan, and four (28.6%) receiving placebo achieving a reduction in PVRi of greater than or equal to 20% (P = 0.97) at 16 weeks. There was no change in functional capacity or symptoms between the two groups at 16 weeks, nor any difference in rates of serious adverse events or deaths (three deaths in each group). CONCLUSIONS: This study shows no difference in invasive pulmonary hemodynamics, functional capacity, or symptoms between the bosentan and placebo groups over 16 weeks. Our data do not support the use of the dual endothelin-1 receptor antagonist, bosentan, in patients with PH and fibrotic IIP. Clinical trial registered with www.clinicaltrials.gov (NCT 00637065).

Alveolar proteinosis with hypersensitivity pneumonitis: a new clinical phenotype.

BACKGROUND AND OBJECTIVE: The aim of the present study was to report the features of five patients with concurrent histopathological features of pulmonary alveolar proteinosis (PAP) and hypersensitivity pneumonitis (HP) and their high-resolution CT (HRCT) appearances. METHODS: Patients with histopathological features of both HP and PAP on surgical lung biopsy referred for tertiary review were retrospectively identified. The pathology and HRCT images were semi-quantitatively scored to evaluate the relative contribution to HP and PAP. RESULTS: Five patients had histopathological features of HP and PAP but had varied HRCT appearances. All had imaging features of PAP to a varying degree with two patients also showing characteristics of HP but three patients had ill-defined thickened interlobular septa, not typical of either disease. CONCLUSIONS: We describe the coexistence of PAP and HP in five patients and discuss possible linkages between these two distinct pathologies.