Publications by authors named "Margaret Wilsher"

37 Publications

A computational model of contributors to pulmonary hypertensive disease: impacts of whole lung and focal disease distributions.

Pulm Circ 2021 Oct-Dec;11(4):20458940211056527. Epub 2021 Nov 18.

Auckland Bioengineering Institute, University of Auckland, Auckland, New Zealand.

Pulmonary hypertension has multiple etiologies and so can be difficult to diagnose, prognose, and treat. Diagnosis is typically made via invasive hemodynamic measurements in the main pulmonary artery and is based on observed elevation of mean pulmonary artery pressure. This static mean pressure enables diagnosis, but does not easily allow assessment of the severity of pulmonary hypertension, nor the etiology of the disease, which may impact treatment. Assessment of the dynamic properties of pressure and flow data obtained from catheterization potentially allows more meaningful assessment of the strain on the right heart and may help to distinguish between disease phenotypes. However, mechanistic understanding of how the distribution of disease in the lung leading to pulmonary hypertension impacts the dynamics of blood flow in the main pulmonary artery and/or the pulmonary capillaries is lacking. We present a computational model of the pulmonary vasculature, parameterized to characteristic features of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension to help understand how the two conditions differ in terms of pulmonary vascular response to disease. Our model incorporates key features known to contribute to pulmonary vascular function in health and disease, including anatomical structure and multiple contributions from gravity. The model suggests that dynamic measurements obtained from catheterization potentially distinguish between distal and proximal vasculopathy typical of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. However, the model suggests a non-linear relationship between these data and vascular structural changes typical of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension which may impede analysis of these metrics to distinguish between cohorts.
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http://dx.doi.org/10.1177/20458940211056527DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8607494PMC
November 2021

Systemic Associations of Sarcoid Uveitis: Correlation With Uveitis Phenotype and Ethnicity.

Am J Ophthalmol 2021 09 15;229:169-175. Epub 2021 Mar 15.

From the Department of Ophthalmology, Auckland District Health Board (R.L.N., J.L.S.); Department of Ophthalmology, University of Auckland (R.L.N.), Auckland, New Zealand; Royal Victoria Eye and Ear, Melbourne, Australia (S.P.M., L.L.L.); Respiratory Services, Auckland District Health Board (M.L.W.); Faculty of Medical and Health Sciences, University of Auckland (M.L.W.), Auckland, New Zealand; Sydney Eye Hospital, Sydney, Australia (N.Q.A.); Department of Ophthalmology, Carmel Medical Centre, Technion, Haifa, Israel (O.T.-N.); University College London, London, United Kingdom (S.L.L.); and Centre for Eye Research Australia, University of Melbourne, Melbourne, Australia (L.L.L.).

Purpose: To examine systemic associations of sarcoid uveitis and association with uveitis clinical phenotype and ethnicity.

Design: Retrospective cross-sectional study.

Subjects: A total of 362 subjects with definite or presumed sarcoid uveitis from Moorfields Eye Hospital, Royal Victorian Eye and Ear, and Auckland District Health Board.

Methods: Data were collected from the review of clinical notes, imaging, and investigations. Sarcoidosis was diagnosed in accordance with the International Workshop on Ocular Sarcoidosis guidelines.

Main Outcome Measure: Diagnosis of associated systemic disease secondary to sarcoidosis.

Results: A total of 362 subjects with sarcoid uveitis were identified. Median age was 46 years, and 226 (62.4%) were female. Granulomatous anterior uveitis (47.8%), intermediate uveitis with snowballs (46.4%), and multifocal choroiditis (43.1%) were the most frequent clinical presentations, and disease was bilateral in 313 (86.5%). Periphlebitis was observed in 21.0%, and solitary optic nerve or choroidal granuloma in 11.3%. Lung parenchymal disease was diagnosed in 200 subjects (55.2%), cutaneous sarcoid in 98 (27.1%), sarcoid arthritis in 57 (15.7%), liver involvement in 21 (5.8%), neurosarcoid in 49 (13.5%), and cardiac sarcoid in 16 subjects (4.4%). Subjects with cardiac sarcoid were less likely to have granulomatous anterior uveitis (P = .017). Caucasian subjects were older at presentation (48 vs 41 years; P = .009), had less granulomatous anterior uveitis (26.4% vs 51.7%; P < .001), and were less likely to present with cutaneous involvement (23.1% vs 35.4%; P = .040).

Conclusions: Ophthalmologists need to be aware of the systemic associations of sarcoid uveitis, in particular potentially life-threatening complications such as cardiac sarcoidosis. Differences observed in uveitis phenotype and between ethnicities require further investigation.
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http://dx.doi.org/10.1016/j.ajo.2021.03.003DOI Listing
September 2021

Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand.

Respirology 2021 01 24;26(1):23-51. Epub 2020 Nov 24.

Department of Respiratory Medicine, Princess Alexandra Hospital, Brisbane, QLD, Australia.

Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Limited data are available to guide management decisions in CTD-ILD. Autoimmune-mediated pulmonary inflammation is considered a key pathobiological pathway in these disorders, and immunosuppressive therapy is generally regarded the cornerstone of treatment for severe and/or progressive CTD-ILD. However, the natural history of CTD-ILD in individual patients can be difficult to predict, and deciding who to treat, when and with what agent can be challenging. Establishing realistic therapeutic goals from both the patient and clinician perspective requires considerable expertise. The document aims to provide a framework for clinicians to aid in the assessment and management of ILD in the major CTD. A suggested approach to diagnosis and monitoring of CTD-ILD and, where available, evidence-based, disease-specific approaches to treatment have been provided.
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http://dx.doi.org/10.1111/resp.13977DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7894187PMC
January 2021

Lung and fissure shape is associated with age in healthy never-smoking adults aged 20-90 years.

Sci Rep 2020 09 30;10(1):16135. Epub 2020 Sep 30.

Auckland Bioengineering Institute, University of Auckland, Auckland, 1010, New Zealand.

Lung shape could hold prognostic information for age-related diseases that affect lung tissue mechanics. We sought to quantify mean lung shape, its modes of variation, and shape associations with lung size, age, sex, and Body Mass Index (BMI) in healthy subjects across a seven-decade age span. Volumetric computed tomography from 83 subjects (49 M/34 F, BMI [Formula: see text]) was used to derive two statistical shape models using a principal component analysis. One model included, and the other controlled for, lung volume. Volume made the strongest contribution to shape when it was included. Shape had a strong relationship with age but not sex when volume was controlled for, and BMI had only a small but significant association with shape. The first principal shape mode was associated with decrease in the antero-posterior dimension from base to apex. In older subjects this was rapid and obvious, whereas younger subjects had relatively more constant dimension. A shift of the fissures of both lungs in the basal direction was apparent for the older subjects, consistent with a change in tissue elasticity with age. This study suggests a quantifiable structure-function relationship for the healthy adult lung that can potentially be exploited as a normative description against which abnormal can be compared.
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http://dx.doi.org/10.1038/s41598-020-73117-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7528089PMC
September 2020

Bring out the stethoscope.

Respirology 2020 12 6;25(12):1227-1228. Epub 2020 Jul 6.

Respiratory Medicine, Christchurch Hospital, Christchurch, New Zealand.

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http://dx.doi.org/10.1111/resp.13906DOI Listing
December 2020

Diagnostic Likelihood Thresholds That Define a Working Diagnosis of Idiopathic Pulmonary Fibrosis.

Am J Respir Crit Care Med 2019 11;200(9):1146-1153

Center for Interstitial Lung Disease, University of Washington, Seattle, Washington.

The level of diagnostic likelihood at which physicians prescribe antifibrotic therapy without requesting surgical lung biopsy (SLB) in patients suspected of idiopathic pulmonary fibrosis (IPF) is unknown. To determine how often physicians advocate SLB in patient subgroups defined by IPF likelihood and risk associated with SLB, and to identify the level of diagnostic likelihood at which physicians prescribe antifibrotic therapy with requesting SLB. An international cohort of respiratory physicians evaluated 60 cases of interstitial lung disease, giving: ) differential diagnoses with diagnostic likelihood; ) a decision on the need for SLB; and ) initial management. Diagnoses were stratified according to diagnostic likelihood bands described by Ryerson and colleagues. A total of 404 physicians evaluated the 60 cases (24,240 physician-patient evaluations). IPF was part of the differential diagnosis in 9,958/24,240 (41.1%) of all physician-patient evaluations. SLB was requested in 8.1%, 29.6%, and 48.4% of definite, provisional high-confidence and provisional low-confidence diagnoses of IPF, respectively. In 63.0% of provisional high-confidence IPF diagnoses, antifibrotic therapy was prescribed without requesting SLB. No significant mortality difference was observed between cases given a definite diagnosis of IPF (90-100% diagnostic likelihood) and cases given a provisional high-confidence IPF diagnosis (hazard ratio, 0.97;  = 0.65; 95% confidence interval, 0.90-1.04). Most respiratory physicians prescribe antifibrotic therapy without requesting an SLB if a provisional high-confidence diagnosis or "working diagnosis" of IPF can be made (likelihood ≥ 70%). SLB is recommended in only a minority of patients with suspected, but not definite, IPF.
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http://dx.doi.org/10.1164/rccm.201903-0493OCDOI Listing
November 2019

Do pulmonary cavity shapes influence lung function?

J Biomech Eng 2019 Jun 24. Epub 2019 Jun 24.

Auckland Bioengineering Institute, The University of Auckland, Auckland, New Zealand.

Distribution of lung tissue within the chest cavity is a key contributor to delivery of both blood and air to the gas exchange regions of the lung. This distribution is multifactorial with influences from parenchyma, gravity and level of inflation. We hypothesize that the manner in which lung inflates, for example, the primarily diaphragmatic nature of normal breathing, is an important contributor to regional lung tissue distribution. To investigate this hypothesis, we present an organ-level model of lung tissue mechanics which incorporates pleural cavity change due to change in lung volume or posture. We quantify the changes using shape and density metrics in ten healthy subjects scanned supine at end-inspiratory and end-expiratory volumes and ten subjects scanned at both supine and prone end-inspiratory volumes. Comparing end-expiratory to end-inspiratory volume, we see primarily a change in the cranial-caudal dimension of the lung, reflective of movement of diaphragm. In the diaphragmatic region there is greater regional lung expansion than in the cranial aspect, which is restricted by the chest wall. When moving from supine to prone, a restriction of lung was observed anteriorly, resulting in a generally reduced lung volume and a redistribution of air volume posteriorly. In general, we see the highest in lung tissue density heterogeneity in regions of the lung that are most inflated. Using our computational model, we quantify the impact of pleural cavity shape change on regional lung distribution, and predict the impact on regional elastic recoil pressure.
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http://dx.doi.org/10.1115/1.4044092DOI Listing
June 2019

Managing disease behaviour: A team approach.

Authors:
Margaret Wilsher

Respirology 2018 11 20;23(11):968-969. Epub 2018 Aug 20.

Auckland District Health Board - Respiratory, Auckland City Hospital, Auckland, New Zealand.

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http://dx.doi.org/10.1111/resp.13386DOI Listing
November 2018

Sarcoidosis: a state of the art review from the Thoracic Society of Australia and New Zealand.

Med J Aust 2018 06 7;208(11):499-504. Epub 2018 May 7.

Prince of Wales Clinical School, UNSW Sydney, Sydney, NSW.

Sarcoidosis is a systemic disease of unknown aetiology, characterised by non-caseating granulomatous inflammation. It most commonly manifests in the lungs and intrathoracic lymph nodes but can affect any organ. This summary of an educational resource provided by the Thoracic Society of Australia and New Zealand outlines the current understanding of sarcoidosis and highlights the need for further research. Our knowledge of the aetiology and immunopathogenesis of sarcoidosis remains incomplete. The enigma of sarcoidosis lies in its immunological paradox of type 1 T helper cell-dominated local inflammation co-existing with T regulatory-induced peripheral anergy. Although specific aetiological agents have not been identified, mounting evidence suggests that environmental and microbial antigens may trigger sarcoidosis. Genome-wide association studies have identified candidate genes conferring susceptibility and gene expression analyses have provided insights into cytokine dysregulation leading to inflammation. Sarcoidosis remains a diagnosis of exclusion based on histological evidence of non-caseating granulomas with compatible clinical and radiological findings. In recent years, endobronchial ultrasound-guided transbronchial needle aspiration of mediastinal lymph nodes has facilitated the diagnosis, and whole body positron emission tomography scanning has improved localisation of disease. No single biomarker is adequately sensitive and specific for detecting and monitoring disease activity. Most patients do not require treatment; when indicated, corticosteroids remain the initial standard of care, despite their adverse side effect profile. Other drugs with fewer side effects may be a better long term choice (eg, methotrexate, hydroxychloroquine, azathioprine, mycophenolate), while tumour necrosis factor-α inhibitors are a treatment option for patients with refractory disease.
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http://dx.doi.org/10.5694/mja17.00610DOI Listing
June 2018

Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary.

Med J Aust 2018 02;208(2):82-88

Royal Prince Alfred Hospital, Sydney, NSW.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand. Main suggestions: A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis. Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum. Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management. Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.
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http://dx.doi.org/10.5694/mja17.00799DOI Listing
February 2018

Ethnicity, socioeconomic status and the severity and course of non-cystic fibrosis bronchiectasis.

Intern Med J 2018 07;48(7):845-850

Respiratory Services, Auckland District Health Board, Auckland, New Zealand.

Background And Aims: This study evaluated whether there are ethnic factors which affect the severity and progression of bronchiectasis in our adult multi-ethnic population in Auckland, New Zealand.

Methods: Clinical records were reviewed from patients attending the outpatient facilities of our institution between 2007 and 2010. Data collected included demographics, clinical features, smoking status, self-reported ethnicity, socioeconomic status (NZDep), pulmonary function and sputum microbiology.

Results: A total of 437 patients was identified: median age 65 years, 66% female, mean forced expiratory volume in the first second (FEV ) 62.4% predicted, and 10.5% of patients had recurrent growth of Pseudomonas aeruginosa. Patients of Maori and Pacific ethnicity were overrepresented compared to the institution population catchment and had more severe impairment of lung function: mean % predicted FEV for Pacific 52.0, Maori 58.6, European 68.6, Asian 64.2 (P < 0.0001). This was independent of socioeconomic status. However, no overall decline was seen in serial lung function measurements, either across the whole cohort or in any particular ethnic group.

Conclusions: Patients of Maori and Pacific ethnicity are both overrepresented and have more severe bronchiectasis in this cohort, independent of socioeconomic status. Ethnicity did not predict decline in pulmonary function. Further studies into genetic predisposition to bronchiectasis in Maori or Pacific people may be warranted.
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http://dx.doi.org/10.1111/imj.13739DOI Listing
July 2018

Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study.

Eur Respir J 2017 08 31;50(2). Epub 2017 Aug 31.

University Hospital of Bellvitge-IDIBELL-CIBERES, Barcelona, Spain.

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts.A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κ). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index.A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κ=0.65, IQR 0.53-0.72, p<0.0001) than academic physicians (κ=0.56, IQR 0.45-0.65, p<0.0001) or physicians with access to multidisciplinary team (MDT) meetings (κ=0.54, IQR 0.45-0.64, p<0.0001). The prognostic accuracy of academic physicians with >20 years of experience (C-index=0.72, IQR 0.0-0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70-0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72-0.75).Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts.
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http://dx.doi.org/10.1183/13993003.00936-2017DOI Listing
August 2017

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia.

Respirology 2017 10 27;22(7):1436-1458. Epub 2017 Aug 27.

Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.
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http://dx.doi.org/10.1111/resp.13146DOI Listing
October 2017

Characteristics of sarcoidosis in Maori and Pacific Islanders.

Respirology 2017 02 12;22(2):360-363. Epub 2016 Oct 12.

Respiratory Services, North Shore Hospital, Waitemata District Health Board, Auckland, New Zealand.

Background And Objective: Ethnicity is strongly associated with variable clinical presentation in sarcoidosis but the association between ethnicity and clinical characteristics has not previously been described in patients of Polynesian ancestry, Maori and Pacific Islander (PI). The objective of this study was to describe the clinical characteristics of sarcoidosis in Maori and PI patients and determine if those were different to European patients.

Methods: A retrospective review of the medical records of 406 patients (69 Maori/PI) attending a specialist interstitial lung disease (ILD) clinic.

Results: The population (207 females, mean age at presentation: 36) reflected the current New Zealand census data (2013) with only people of Indian ethnicity over-represented. Parenchymal lung involvement was uncommon in Maori and PI patients (21% Scadding stage 2, 2% stage 3), and no patient had extensive pulmonary fibrosis (stage 4). Computed tomography (CT) patterns of sarcoid parenchymal lung involvement were less commonly reported for Maori/PI. There were no differences in respect of baseline lung function or requirement for treatment. Ocular and skin involvement occurred more frequently in Maori and PI (P = 0.0045, P = 0.03), and erythema nodosum was more common in Caucasians (P = 0.0008).

Conclusion: People of Polynesian ancestry appear to have less pulmonary and more extra-pulmonary manifestations of sarcoidosis. This adds to our knowledge that sarcoidosis heterogeneity is influenced by ethnicity.
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http://dx.doi.org/10.1111/resp.12917DOI Listing
February 2017

Randomized Controlled Trial of Humidified High-Flow Nasal Oxygen for Acute Respiratory Distress in the Emergency Department: The HOT-ER Study.

Respir Care 2016 Mar 17;61(3):291-9. Epub 2015 Nov 17.

Respiratory Medicine Department, Auckland City Hospital, Auckland, New Zealand.

Background: Humidified high-flow nasal cannula (HFNC) is a novel method of oxygen delivery with increasing use in emergency departments and intensive care settings despite little evidence showing benefit over standard oxygen delivery methods (standard O2). The aim of this study was to determine whether HFNC compared with standard O2 given to subjects in acute respiratory distress would reduce the need for noninvasive ventilation or invasive ventilation.

Methods: This was a pragmatic open randomized controlled trial in adult subjects with hypoxia and tachypnea presenting to a tertiary academic hospital emergency department. The primary outcome was the need for mechanical ventilation in the emergency department.

Results: We screened 1,287 patients, 322 met entry criteria and 19 were excluded from analysis. Of these, 165 randomized to HFNC and 138 to standard O2 were analyzed. Baseline characteristics were similar. In the HFNC group, 3.6% (95% CI 1.5-7.9%) versus 7.2% (95% CI 3.8-13%) in the standard O2 group required mechanical ventilation in the emergency department (P = .16), and 5.5% (95% CI 2.8-10.2%) in HFNC versus 11.6% (95% CI 7.2-18.1%) in the standard O2 group required mechanical ventilation within 24 h of admission (P = .053). There was no difference in mortality or stay. Adverse effects were infrequent; however, fewer subjects in the HFNC group had a fall in Glasgow coma score due to CO2 retention, 0% (95% CI 0-3%) versus 2.2% (95% CI 0.4-6%). One in 12 subjects did not tolerate HFNC.

Conclusions: HFNC was not shown to reduce the need for mechanical ventilation in the emergency department for subjects with acute respiratory distress compared with standard O2, although it was safe and may reduce the need for escalation of oxygen therapy within the first 24 h of admission.
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http://dx.doi.org/10.4187/respcare.04252DOI Listing
March 2016

Current Australasian practice for diagnosis and management of idiopathic pulmonary fibrosis: Where are we now?

Respirology 2015 May 22;20(4):647-53. Epub 2015 Mar 22.

Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.

Background And Objective: Recent international consensus statements have refined evidence-based guidelines for the diagnosis and management of idiopathic pulmonary fibrosis (IPF). This study sought to investigate how closely these guidelines are adhered to and to compare current practices with those of a similar cohort 15 years ago.

Methods: A questionnaire on IPF diagnosis and management was distributed to respiratory physicians practising in Australia and New Zealand, in 2012-2013, and results were compared with a similar survey conducted in 1999.

Results: A total of 172 and 144 questionnaires were completed in 1999 and 2012-2013, respectively. The most important investigations in both survey populations were high-resolution computed tomography scans, spirometry, diffusing capacity for carbon monoxide, chest X-ray, static lung volumes and autoimmune serology. In 1999, physicians were more likely to perform arterial blood gases, bronchoalveolar lavage and transbronchial lung biopsy. In the 2012-2013 cohort, 6-min walk tests and pulse oximetry were more widely utilized. Treatment choices differed considerably between the two survey populations. In 1999, the majority would offer a steroid-based regimen, whereas most would not use any specific treatment or would refer for trial participation in 2012-2013.

Conclusions: Approach to IPF diagnosis and management is not uniform and has changed over 15 years. Surveyed respiratory physicians were generally practising in accordance with clinical guidelines, although significant variation in practice was identified in both cohorts. This study identifies the need to standardize care of IPF patients across Australia and New Zealand.
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http://dx.doi.org/10.1111/resp.12512DOI Listing
May 2015

The relationships among dyspnoea, health-related quality of life and psychological factors in sarcoidosis.

Respirology 2014 Oct 14;19(7):1019-24. Epub 2014 Aug 14.

Green Lane Respiratory Services, Auckland City Hospital, Auckland District Health Board, Auckland, New Zealand.

Background And Objectives: Dyspnoea is a common symptom in sarcoidosis and is not predictably related to pulmonary function or radiology. A subjective symptom of dyspnoea is likely to be influenced by patient perception and experience. The aim of this study was to determine the prevalence and nature of dyspnoea in sarcoidosis and describe the relationship of dyspnoea to psychological factors and health-related quality of life (HRQL).

Methods: Fifty-six subjects (31 men, mean age 51 years) with sarcoidosis completed an HRQL measure, St George's Respiratory Questionnaire (SGRQ), Hospital Anxiety and Depression Scale (HADS) and Nijmegen questionnaire. The presence of symptoms of dyspnoea was noted and qualitative descriptors for dyspnoea were chosen at peak exercise. Resting pulmonary function was performed.

Results: Sixty-four per cent of the subjects reported dyspnoea. Those with symptoms were older, had a longer duration of disease and with lower forced expiratory volume in 1 s (FEV1 ) and FEV1 /forced vital capacity (FVC) (all P < 0.05). Symptoms of dyspnoea were associated with worse HRQL (P < 0.005) and higher scores on the Nijmegen questionnaire (P < 0.05). Anxiety was not associated with dyspnoea and only a trend to greater depression was observed (P = 0.066). In multivariate analysis, SGRQ and Nijmegen scores predicted dyspnoea independent of demographic factors and resting pulmonary function.

Conclusion: Dyspnoea is common in sarcoidosis and is associated with worse HRQL irrespective of baseline pulmonary function. Hyperventilation appears to be a factor contributing to dyspnoea and the Nijmegen questionnaire may be helpful in assessing dyspnoea and hyperventilation in sarcoidosis patients.
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http://dx.doi.org/10.1111/resp.12359DOI Listing
October 2014

Comparison of the modified shuttle walk test and cardiopulmonary exercise test in sarcoidosis.

Respirology 2014 May 25;19(4):604-7. Epub 2014 Mar 25.

Green Lane Respiratory Services, Auckland City Hospital, Auckland, New Zealand.

Background And Objective: Dyspnoea and exercise intolerance are common in sarcoidosis and are often poorly correlated with resting lung function. Measurement of peak exercise capacity is likely to be helpful in assessing and monitoring disease. The aim of this study was to compare the modified shuttle walk test (MSWT) to cardiopulmonary exercise test (CPET) as a measure of peak exercise capacity in sarcoidosis.

Methods: Thirty-three (17 male, mean age 48 years) patients with sarcoidosis completed a standardized exponential exercise protocol cycle ergometer CPET and a single corridor MSWT in random order.

Results: Subjects has a mean forced expiratory volume in 1 s (FEV1) 2.4 L (75.7%predicted), forced vital capacity (FVC) 3.43 L (88.7%predicted) and diffusing capacity for carbon monoxide (DLCO) 20.3 mL/min/mm Hg (71.4%predicted). There was a strong correlation between MSWT distance and peak oxygen uptake (VO2) during CPET (r = 0.87; P < 0.0001), and between maximum heart rate during MSWT and CPET (r = 0.82; P < 0.0001). There was a moderate correlation between FEV1 , FVC and DLCO with MSWT distance (r = 0.55, r = 0.61, r = 0.61, respectively; all P < 0.001) and with peak VO2 (r = 0.62, r = 0.63, r = 0.62, respectively; all P < 0.0001).

Conclusions: Peak VO2 achieved during CPET strongly correlated with MSWT distance. MSWT is a measure of peak exercise capacity in sarcoidosis that does not require equipment and can be readily available in the clinic.
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http://dx.doi.org/10.1111/resp.12276DOI Listing
May 2014

Randomised controlled trial of vitamin D supplementation in sarcoidosis.

BMJ Open 2013 Oct 23;3(10):e003562. Epub 2013 Oct 23.

Department of Medicine, University of Auckland, Auckland, New Zealand.

Objectives: The role vitamin D intake/production plays in sarcoidosis-associated hypercalcaemia is uncertain. However, authoritative reviews have recommended avoiding sunlight exposure and vitamin D supplements, which might lead to adverse skeletal outcomes from vitamin D insufficiency. We investigated the effects of vitamin D supplementation on surrogate measures of skeletal health in patients with sarcoidosis and vitamin D insufficiency.

Design: Randomised, placebo-controlled trial.

Setting: Clinical research centre.

Participants: 27 normocalcaemic patients with sarcoidosis and 25-hydroxyvitamin D (25OHD) <50 nmol/L.

Intervention: 50 000 IU weekly cholecalciferol for 4 weeks, then 50 000 IU monthly for 11 months or placebo.

Primary And Secondary Outcome Measures: The primary endpoint was the change in serum calcium over 12 months, and secondary endpoints included measurements of calcitropic hormones, bone turnover markers and bone mineral density (BMD).

Results: The mean age of participants was 57 years and 70% were women. The mean (SD) screening 25OHD was 35 (12) and 38 (9) nmol/L in the treatment and control groups, respectively. Vitamin D supplementation increased 25OHD to 94 nmol/L after 4 weeks, 84 nmol/L at 6 months and 78 nmol/L at 12 months, while levels remained stable in the control group. 1,25-Dihydroxy vitamin D levels were significantly different between the groups at 4 weeks, but not at 6 or 12 months. There were no between-groups differences in albumin-adjusted serum calcium, 24 h urine calcium, markers of bone turnover, parathyroid hormone or BMD over the trial. One participant developed significant hypercalcaemia after 6 weeks (total cholecalciferol dose 250 000 IU).

Conclusions: In patients with sarcoidosis and 25OHD <50 nmol/L, vitamin D supplements did not alter average serum calcium or urine calcium, but had no benefit on surrogate markers of skeletal health and caused one case of significant hypercalcaemia.

Trial Registration: This trial is registered at the Australian New Zealand Clinical Trials Registry (http://www.anzctr.org.au). The registration number is ACTRN12607000364471, date of registration 5/7/2007.
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http://dx.doi.org/10.1136/bmjopen-2013-003562DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3808783PMC
October 2013

Ventilatory and cardiac responses to pulmonary embolism: consequences for gas exchange and blood pressure.

Annu Int Conf IEEE Eng Med Biol Soc 2012 ;2012:6657-60

Auckland Bioengineering Institute,The University of Auckland, Private Bag 92019, Auckland, New Zealand.

Acute thromboembolic pulmonary embolism (PE) is a life threatening condition that can lead to pulmonary hypertension and right ventricular dysfunction or failure. There is typically an increase in ventilation rate and cardiac output as a response to PE prior to cardiac failure, which is at least in part due to systemic hypoxemia. Here we assess the response of the lungs to changes in these parameters using anatomically-based computational models of pulmonary perfusion, ventilation and gas exchange. We show that increases in ventilation and cardiac output improve overall gas exchange in PE. However, this comes at the cost of an increased pulmonary blood pressure, which may contribute to pulmonary hypertension as a result of PE.
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http://dx.doi.org/10.1109/EMBC.2012.6347521DOI Listing
September 2013

Prevalence of airway and parenchymal abnormalities in newly diagnosed rheumatoid arthritis.

Respir Med 2012 Oct 13;106(10):1441-6. Epub 2012 Jul 13.

Green Lane Respiratory Services, Auckland District Health Board, Private Bag 92024, Auckland 1142, New Zealand.

Background: Pulmonary disease is a well recognised and important extra-articular manifestation of rheumatoid arthritis (RA). The objective of this study was to determine the prevalence of airway and parenchymal abnormalities in newly diagnosed patients with RA and to correlate these with clinical measures of RA severity and laboratory tests.

Methods: 60 patients with a new (symptom duration <12 months) diagnosis of RA (43 females, 42 European, mean age 54, 33 ever smoker, (17 current) underwent lung function testing and high resolution computed tomography (HRCT) scored by two independent radiologists.

Results: Eighteen (30%) patients reported respiratory symptoms: dyspnoea (11), cough (11), and wheeze (8). Twelve (20%) patients had physiologic evidence of airflow obstruction and 24 (40%) had reduced gas transfer. The prevalence of HRCT abnormalities (in any lobe) was as follows: decreased attenuation 67%, bronchiectasis 35%, bronchial wall thickening 50%, ground glass opacification 18%, reticular changes 12%. All abnormalities were more common in the lower lobes. With the exception of reduced DLCO, there were no significant differences in the prevalence of HRCT patterns or lung function parameters between smokers and non smokers. Anti-CCP antibodies and rheumatoid factor (RF) correlated strongly with DLCO and variably with other physiologic measures but poorly with radiologic abnormalities.

Conclusion: Patients with newly diagnosed RA have a moderate prevalence of airway and parenchymal abnormalities on HRCT and lower than predicted lung function parameters which cannot entirely be explained by smoking. These data suggest that pulmonary involvement is present early in the disease course in RA.
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http://dx.doi.org/10.1016/j.rmed.2012.06.020DOI Listing
October 2012

Psychological stress in sarcoidosis.

Curr Opin Pulm Med 2012 Sep;18(5):524-7

Green Lane Respiratory Services, Auckland District Health Board, Auckland, New Zealand.

Purpose Of Review: Sarcoidosis is a chronic illness associated with emotional and physical consequences which impact on quality of life. Although the impact of fatigue is well understood, emotional impacts of sarcoidosis are less commonly recognized and addressed in routine clinical practice. The purpose of this review is to highlight that sarcoidosis can result in considerable psychological distress.

Recent Findings: Not only is there a high prevalence of depressive symptoms in sarcoidosis, but clinical depressive and anxiety disorders are more common than seen in the general population. Patients with sarcoidosis have perceptions and beliefs about their disease that may impact on their willingness to engage in recommended therapies. They may also exhibit a disordered perception of their disease and a personality profile of neuroticism. Understanding the minimally important clinical difference in the Fatigue Assessment Scale (FAS) and validation of the Sarcoidosis Health Questionnaire (SHQ) across different populations supports the use of these tools in routine clinical practice and clinical trials.

Summary: Understanding the global impact of sarcoidosis is important for patients and clinicians, and use of validated instruments, such as the SHQ and FAS, allows for more comprehensive assessment of the disease and the impact of any interventions.
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http://dx.doi.org/10.1097/MCP.0b013e3283547092DOI Listing
September 2012

The prevalence and predictors of airway hyperresponsiveness in sarcoidosis.

Respirology 2012 May;17(4):653-9

Green Lane Respiratory Services, University of Auckland, Auckland, New Zealand.

Background And Objective: Obstructive airflow limitation is the most common physiological impairment in sarcoidosis. This study determined the prevalence of airway hyperresponsiveness (AHR) in sarcoidosis, the correlation between responses to direct (using histamine) and indirect (using hypertonic saline) bronchial challenge, and the clinical, physiological and radiological predictors of AHR.

Methods: Subjects with sarcoidosis and a baseline forced expiratory volume in 1 s (FEV(1)) >35% predicted underwent hypertonic and histamine challenge, lung function testing and high resolution computed tomography (HRCT) of the chest. AHR was defined as a 15% fall in FEV(1) to hypertonic saline and a 20% fall in FEV(1) to histamine.

Results: The 52 subjects had well-preserved lung function (FEV(1) = 2.8 ± 0.7 L, 87% predicted). AHR was detected in 5/47 (11%) to hypertonic saline and 19/43 (44%) to histamine challenge. On univariate analysis, response to histamine challenge was predicted by conglomerate fibrosis (P = 0.02) and reticular pattern (P = 0.03) on HRCT. The baseline % predicted forced expiratory volume in 1 s was significantly inversely associated with AHR on univariate (P = 0.004) and multivariate analysis (P = 0.01) when adjusted by HRCT patterns.

Conclusions: The higher prevalence of AHR using histamine challenge than hypertonic saline challenge and the association with baseline % predicted FEV(1) suggest that the AHR in sarcoidosis may reflect the consequences of airway remodelling following inflammation.
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http://dx.doi.org/10.1111/j.1440-1843.2012.02137.xDOI Listing
May 2012

Validation of the Sarcoidosis Health Questionnaire in a non-US population.

Respirology 2012 Apr;17(3):519-24

Green Lane Respiratory Services, Auckland City Hospital, Auckland, New Zealand.

Background And Objective: Sarcoidosis is a multi-system disease with an unpredictable course and variable clinical manifestations that are often associated with impaired quality of life. The Sarcoidosis Health Questionnaire (SHQ), which was developed for an 80% African American population, assesses health-related quality of life in sarcoidosis patients. The aim of this study was to validate the SHQ in a predominantly European population of sarcoidosis patients.

Methods: Consecutive outpatients (n = 92) with sarcoidosis, who were attending a teaching hospital clinic, completed three questionnaires (SHQ, Short Form (SF) 36, Fatigue Assessment Scale (FAS)) and pulmonary function tests were performed.

Results: The mean age of the patients was 51 years, 52% were males and 74% were of European ethnicity. The mean number of organs involved was 1.3, with pulmonary involvement in 95% of patients (mean forced expiratory volume in 1 s 74.4%, forced vital capacity 84.6%). Seventy percent of patients had current symptoms and 26.5% were receiving immunosuppressant therapy. The SHQ total score (mean 5.13) was significantly correlated with the SF 36 physical component score (46.7, r = 0.78) and the FAS (20.8, r = -0.7) but only weakly correlated with pulmonary function. There were significant differences in SHQ scores when patients were stratified according to symptoms, oral therapy, health status (P < 0.0001 for all), forced expiratory volume in 1 s ≥70% (P = 0.008) and forced vital capacity ≥70% (P = 0.01).

Conclusions: The SHQ correlated well with health-related quality of life and fatigue measures in a predominantly European population of sarcoidosis patients, despite differences in organ involvement and disease burden, when compared with the development study.
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http://dx.doi.org/10.1111/j.1440-1843.2012.02134.xDOI Listing
April 2012

The six-minute walk test using forehead oximetry is reliable in the assessment of scleroderma lung disease.

Respirology 2012 May;17(4):647-52

Green Lane Respiratory, Auckland District Health Board, University of Auckland, Auckland, New Zealand.

Background And Objective: The six-minute walk test (6MWT) is a validated field test in the assessment of interstitial lung disease but may not be so useful in scleroderma (SSc) lung disease. The aim of this study was to determine the reliability of the 6MWT in patients with SSc and correlate results with morphological and functional measures of disease severity.

Methods: Thirty patients (24 female, mean age 47, mean diffusing capacity of carbon monoxide 65%, vital capacity 77% predicted) with American College of Rheumatology classification of SSc performed two 6MWT using various oximetry sites, 1 week apart, and underwent SSc-specific disease severity and quality-of-life measurements, lung function, high-resolution computed tomography and echocardiography.

Results: There was good reliability between the two 6MWT (distance; intraclass correlation coefficient 0.95, r = 0.89, Borg; intraclass correlation coefficient 0.85, r = 0.91, both P < 0.00 for r), and Bland Altman plots demonstrate good agreement between measures 1 week apart. Forehead and finger oximetry were more reliable than earlobe (intraclass correlation coefficient 0.64, 0.60, 0.24; r = 0.46, 0.47, 0.14; n = 22, 17, 7, respectively). Forehead desaturation correlated with forced expiratory volume in 1 s (r = 0.55, P = 0.01) and forced vital capacity (r = 0.59, P = 0.01). Distance correlated with all physiological measures: forced expiratory volume in 1 s (r = 0.55, P = 0.01), forced vital capacity (r = 0.61, P = 0.01) and diffusing capacity of carbon monoxide (r = 0.42, P = 0.05). Computed tomography extent and patterns of disease correlated poorly with 6MWT measures, and global measures of SSc correlated only with post-test Borg score.

Conclusions: The 6MWT is feasible and reliable in SSc lung disease, but forehead oximetry should be used. The test measurements correlate reasonably but variably with functional and morphological measures of disease severity.
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http://dx.doi.org/10.1111/j.1440-1843.2012.02133.xDOI Listing
May 2012

Prevalence of asthma and atopy in sarcoidosis.

Respirology 2012 Feb;17(2):285-90

Respiratory Services, Auckland District Health Board, Auckland, New Zealand.

Background And Objective: We hypothesized that the prevalence of allergic disorders, characterized by the release of type 2 cytokines (IL-4, IL-5, IL-10), would be lower in sarcoidosis in which there is a dominant type 1 immune response (IL-2, interferon-gamma). The objective was to measure the prevalence of atopy and self-reported asthma in patients with sarcoidosis.

Methods: Sarcoidosis patients (n = 136, 72 M, age range 22-75), recruited in the outpatient setting, completed a modified European Community Respiratory Health Survey. 123 of these patients provided blood for allergy testing.

Results: For the cohort as a whole the self-reported prevalence of asthma ever (21.5%) and asthma attack in the last 12 months (7.5%), was high as was wheezing (42.1%), breathlessness with wheeze (22.3%) and use of an asthma medication (13.1%). The prevalence of atopy was 34%. These data are not different from the previously reported prevalence of asthma and atopy in New Zealand.

Conclusions: The same prevalence of asthma symptoms and atopy as in the normal population suggests that the immune system is not skewed away from mounting T helper type 2 immune responses in sarcoidosis.
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http://dx.doi.org/10.1111/j.1440-1843.2011.02066.xDOI Listing
February 2012

Review series: Aspects of interstitial lung disease. Sarcoidosis.

Chron Respir Dis 2010 ;7(4):247-58

Green Lane Respiratory Services, Auckland District Health Board, Auckland, New Zealand.

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http://dx.doi.org/10.1177/1479972310388352DOI Listing
March 2011
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