Background and objective: The research term “interstitial pneumonia with autoimmune features” (IPAF) encompasses interstitial lung disease (ILD) patients with autoimmune features not meeting diagnostic criteria for a defined connective tissue disease (CTD). It remains unclear if IPAF is a distinct disease entity with implications for management and prognosis. We describe an Australian IPAF population and compare their baseline characteristics and outcomes with distinct cohorts of idiopathic interstitial pneumonia (IIP), CTD-ILD, and unclassifiable ILD. Methods: Review of 291 consecutive patients attending a specialist ILD clinic was performed. Patients with a diagnosis of IIP, CTD-ILD, and unclassifiable ILD by ILD-multidisciplinary meeting (ILD-MDM) were included. Patients meeting the IPAF criteria were identified. Baseline clinical data, survival, and progression were compared between ILD groups. Results: 226 patients were included, 36 meeting the IPAF criteria. IPAF patients demonstrated a high prevalence of autoantibodies to tRNA synthetase (35.3%), Ro52 (27.8%), and neutrophilic cytoplasmic antigens (ANCA; 20.0%). IPAF and CTD-ILD patients demonstrated similar clinical characteristics (mean age 66.6 and 63.7 years, respectively, female predominant, frequent CTD-manifestations). Lung function did not differ between ILD groups. Disease severity, pulmonary hypertension (PH), and ILD-MDM diagnosis were strong predictors of worse transplant-free survival (TFS). Meeting the IPAF criteria was not associated with TFS. Conclusions: We identified IPAF as a heterogeneous phenotype that overlaps considerably with CTD-ILD. Disease severity, PH, and ILD-MDM diagnosis were more powerful predictors of survival outcomes than meeting the IPAF criteria.
The role of non-invasive ventilation (NIV) in patients with cystic fibrosis (pwCF) includes use in both the management of hypercapnic respiratory failure and as an adjunct to airway clearance techniques. We performed a retrospective review of the Australian Cystic Fibrosis Data Registry to analyse the characteristics of pwCF requiring NIV. We demonstrated that despite improvements in overall health in pwCF there is still a significant role of NIV in this population.
Multidisciplinary meetings (MDM) are the current "gold standard" in interstitial lung disease (ILD) diagnosis and comprise inter-disciplinary discussion of multiple forms of information to provide diagnostic and management outputs. Although bias could be potentially inserted at any step in the discussion process, to date there has been no consensus regarding the appropriate constitution and governance of MDM. We sought to determine the features of ILD MDMs based within ILD centres of excellence around the world.An internet based questionnaire was sent to twelve expert centres in Europe, North America, and Australia seeking information regarding the structure and governance of their MDM. Data was analysed for consistent themes and points of contrast.Responses were received from 10 out of 12 centres. Similarities were demonstrated with regards to contributing attendees, meeting frequency and case numbers reviewed. Significant heterogeneity in attendee speciality group type, quantity and method of data presentation, approach to diagnosis formulation and documentation, and information provision was apparent.The constitution of ILD MDMs differs considerably between expert centres. Such differences may result in discordant outcomes, and emphasise the need for further evidence regarding the appropriate constitution and governance of ILD MDMs.
ABSTRACT Background and objective The significant and progressive morbidity associated with ILD mean that patients often struggle with the impact of this disease on their QOL and independence. To date, no studies have investigated the importance of multidisciplinary care on patient experience in ILD. We aimed to determine the expectations and priorities of patients attending a tertiary referral centre multidisciplinary ILD clinic. In particular, we sought to learn how important the multidisciplinary element of the clinic was to patients and which aspects of the clinic were most valued. Methods An 18‐item patient questionnaire was developed in conjunction with expert physicians and specialist nurses involved in the ILD clinic and sent to all patients on the centre's ILD registry at the time of the study ( n = 240). Patients rated the importance of different aspects of their experience of attending the clinic. Data collected were analysed using descriptive statistics. Comparisons across disease severity were made using two‐sided Z‐tests for independent proportions. Results A total of 100 respondents comprised the study group. Almost all respondents valued the multidisciplinary aspect of the clinic. Obtaining an accurate diagnosis and improving their disease understanding was most important to respondents. The importance of the ILD specialist nurse for both education and support increased with worsening disease severity. Conclusion Our results suggest that a multidisciplinary approach to the management of ILD with additional focus on patient education, as well as tailoring care to disease severity, is a plausible pathway to improving the patient experience with ILD.
Idiopathic pulmonary fibrosis (IPF) is progressive with a median survival of 2-3yrs. The natural history is highly variable and difficult to predict for an individual presenting with early IPF. Aim: To identify markers associated with disease progression for IPF patients; especially those with early disease. Method: The Australian IPF Registry recruits IPF patients across Australia collating clinical, physiological and questionnaire data. Using this real-world longitudinal cohort we compared mild disease(FVC >80%) with FVC<80%. Using Cox analysis and logistic regression,we examined associations between pre-specified markers and progression free survival(PFS;fall in FVC>10% or DLco>15% ± death). Result: Baseline data in 631 participants revealed: 433(69%) male, mean age 70.1±8.5yrs; mean FVC 81±22% and DLco 49±17%. Patients with mild disease(n=235) were older(p=0.001) and more often female(p<0.001), than those with FVC<80%. Reduced PFS was associated with: male gender, impaired quality of life(SGRQ), depression, cough severity, and lower baseline FVC and DLco. Patients with mild disease had improved PFS compared to those with FVC<80%(HR 1.61;95% CI1.3,2.0;P<0.001). In the subset of patients with mild disease, 41 (18%) had disease progression at 12mths. In mild IPF, disease progression at 12mths was associated with: 6MWT end SpO2 (OR 0.91, 95%CI 0.84-0.99; p=0.028), 6MWT distance and cough severity. On multivariate regression, the variable most closely associated with death at 12mths was 6MWT end SpO2. Conclusion: Impaired baseline lung function is associated with a worse PFS in IPF patients. However a significant proportion of patients with mild disease also show disease progression at 12mths.
Introduction: A variety of lung function, radiological and inflammatory markers are used to predict the clinical course of ILD. The perceived value of 99m Technetium-labelled diethylenetriamine penta-acetic acid (DTPA) clearance as one of these has waxed and waned over 20 years or more. One deficit has been the lack of long term studies addressing the value of DTPA clearance in predicting overall survival. Aim: To determine whether DTPA lung clearance predicts survival in ILD independent of lung function. Methods: We analysed DTPA lung clearance scans performed in the assessment of ILD at Concord Hospital from Jan 2000 to Dec 2010. Normal clearance was t 1/2 >25 minutes with mono-exponential pattern. Overall survival for normal and abnormal groups was evaluated using Kaplan-Meier method and the log-rank test and Cox proportional hazards regression models. Results: 227 DTPA scans were performed for initial assessment of ILD in the study period. 56% of subjects were male and the mean age at initial scan was 71 (13). Diagnoses were idiopathic pulmonary fibrosis in 89(39%), unspecified ILD in 54(24%), connective tissue disease-related ILD in 37(16%) and ILD/COPD in 20(9%). In univariate analysis, rapid DTPA clearance, hazard ratios (HR; 95% CI) 1.88 (1.11 - 3.18), DLCO Conclusions: Although rapid DTPA clearance predicts poorer survival in patients being initially evaluated for ILD, the predictive value of DTPA is embedded in DLCO.
Blood monocytes have been recently proposed as a potential prognostic marker for IPF. Data from the Australian IPF registry have shown that elevated monocytes, neutrophils and total leukocytes significantly predict poorer survival in IPF patients.http://bit.ly/38GP7f0
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