R-scale for pulmonary fibrosis: a simple, visual tool for the assessment of health-related quality of life.

RATIONALE: Patients with idiopathic pulmonary fibrosis (IPF) experience impaired health-related quality of life (HRQoL). Several tools have been developed to objectively assess HRQoL in this patient population, but none are in use in routine clinical practice. OBJECTIVES: To develop a rapid, specific tool that can be used for patients with IPF during routine clinic visits. METHODS: A novel and simple five-item numerical rating scale was developed and compared with two other previously validated tools. 100 consecutive patients with IPF managed at a centre for interstitial lung disease were recruited to complete the Raghu scale for pulmonary fibrosis (R-Scale-PF), King's Brief Interstitial Lung Disease questionnaire (K-BILD), and the EuroQol Five-Dimensional Five-Level questionnaire (EQ-5D-5L) in addition to pulmonary function and 6-min walk tests. MEASUREMENTS AND MAIN RESULTS: All 100 patients successfully completed the three HRQoL tools with 53 completing them again at follow-up visits. Internal consistency was high (Cronbach's α 0.825) with minimal floor/ceiling effect. Concurrent validity of the R-Scale-PF was moderate to high compared with the K-BILD (r=-0.713) and the EQ-5D-5L (r=-0.665). Concurrent validity was moderate with physiologic measures (forced vital capacity, r=-0.307, 6-min walking distance, r=-0.383). The R-Scale-PF demonstrated good known-groups validity when comparing scores across stages of disease severity. CONCLUSIONS: The R-Scale-PF correlates well with the K-BILD and EQ-5D-5L. It is hoped that this novel simple numerical rating scale tool, subject to validation in patients from other centres, will provide an opportunity to objectively measure HRQoL in routine clinical practice for patients with IPF.

Laparoscopic anti-reflux surgery for idiopathic pulmonary fibrosis at a single centre.

We sought to assess whether laparoscopic anti-reflux surgery (LARS) is associated with decreased rates of disease progression in patients with idiopathic pulmonary fibrosis (IPF).The study was a retrospective single-centre study of IPF patients with worsening symptoms and pulmonary function despite antacid treatment for abnormal acid gastro-oesophageal reflux. The period of exposure to LARS was September 1998 to December 2012. The primary end-point was a longitudinal change in forced vital capacity (FVC) % predicted in the pre- versus post-surgery periods.27 patients with progressive IPF underwent LARS. At time of surgery, the mean age was 65 years and mean FVC was 71.7% pred. Using a regression model, the estimated benefit of surgery in FVC % pred over 1 year was 5.7% (95% CI -0.9-12.2%, p=0.088) with estimated benefit in FVC of 0.22 L (95% CI -0.06-0.49 L, p=0.12). Mean DeMeester scores decreased from 42 to 4 (p<0.01). There were no deaths in the 90 days following surgery and 81.5% of participants were alive 2 years after surgery.Patients with IPF tolerated the LARS well. There were no statistically significant differences in rates of FVC decline pre- and post-LARS over 1 year; a possible trend toward stabilisation in observed FVC warrants prospective studies. The ongoing prospective randomised controlled trial will hopefully provide further insights regarding the safety and potential efficacy of LARS in IPF.

Sarcoidosis and IPF in the same patient-a coincidence, an association or a phenotype?

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) and Sarcoidosis are distinct clinical entities. Fibrotic disease in pulmonary sarcoidosis is typically upper lobe predominant. In IPF fibrosis is basilar and peripheral predominant [usual interstitial pneumonia (UIP) pattern]. Sarcoidosis and UIP have rarely been observed in the same patient. We sought to characterize patients manifesting both sarcoidosis and IPF and compare clinical features and survival to that of patients with "Lone-IPF" (IPF only) and pulmonary sarcoidosis with fibrosis in a non-UIP pattern. METHODS: Patients were identified from a clinical database and data abstracted from medical records (1995-2016): 1) 25 patients with combined sarcoidosis and IPF (CSIPF) defined by clinical and histological features of sarcoidosis and HRCT features of possible or definite UIP or UIP by histopathology; 2) Randomly selected control patients during the same period: 28 Lone-IPF, 25 stage III/IV pulmonary sarcoidosis. RESULTS: The gender and race of patients with CSIPF and Lone-IPF patients were similar (68% male and 84% Caucasian), as were survival outcomes. Mean time from IPF diagnosis to death: 3.2 years CSIPF, 3.6 years Lone-IPF (log rank p value 0.49). Among patients with pulmonary sarcoidosis, mean time from diagnosis to death: 21.4 years. CONCLUSIONS: Clinical characteristics/behavior of patients with CSIPF was similar to Lone-IPF patients. It is possible that patients with sarcoidosis coincidentally developed IPF and/or have occult genetic predisposition factors to manifest both diseases at different time points. Further study is needed.

Idiopathic Interstitial Pneumonia Associated With Autoantibodies: A Large Case Series Followed Over 1 Year.

BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia, particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease-associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF), or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: This was a retrospective single-center study. Analyses of variance compared differences in mean change in FVC and diffusion capacity (Dlco) over 1 year among 124 well-defined patients (20 patients with positive autoantibodies with or without symptoms of connective tissue disease [AI-ILD], 15 patients with IPAF, 36 patients with CTD-ILD, and 53 patients with IPF with negative CTD serologies [Lone-IPF]). RESULTS: Of the patients, 75% with AI-ILD, 33% with IPAF, and 33% with CTD-ILD had UIP. Initial FVC and Dlco were similarly moderately reduced across groups. Mean change in FVC over 12 months was as follows: -60 mL (IPAF), -110 mL (AI-ILD), -10 mL (CTD-ILD), and -90 mL (Lone-IPF) (P = .52). Mean change in Dlco was as follows: 2.39 mL/mm Hg/min (IPAF), -1.15 mL/mm Hg/min (AI-ILD), -0.27 mL/mm Hg/min (CTD-ILD), and -1.05 mL/mm Hg/min (Lone-IPF) (P < .001). By pattern of disease, the mean change in FVC was as follows: -140 mL (UIP), 10 mL (nonspecific interstitial pneumonia), and 12 mL (unclassifiable/other) (P = .001). CONCLUSIONS: No clinically significant differences in pulmonary function to distinguish between patients with AI-ILD, IPAF, CTD-ILD, and Lone-IPF were observed after 1 year. Longer periods of follow-up are needed to understand the outcomes of these patients. It is not yet clear whether AI-ILD is a distinct phenotype or a variant of the newly proposed entity IPAF.

Sole treatment of acid gastroesophageal reflux in idiopathic pulmonary fibrosis: a case series.

RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal disease despite the available treatment regimes. Increased acid gastroesophageal reflux (GER) occurs in IPF patients. OBJECTIVES: To follow the course of IPF in patients while being treated for acid GER alone. METHODS: A retrospective review of the clinical outcomes of four patients with newly diagnosed IPF and increased acid GER who chose to be treated solely with anti-acid GER therapy were followed up regularly with pulmonary function tests (PFTs) [measuring FVC and the diffusing capacity of the lung for carbon monoxide] over a period of 2 to 6 years. Anti-acid GER therapy was administered using proton-pump inhibitors and fundoplication, if needed. Adequate suppression of acid GER was ascertained by 24-h esophageal pH monitoring. MAIN RESULTS: PFT results in all four patients stabilized or improved while their conditions were maintained with adequate treatment for acid GER. All patients were alive at the last follow-up, and none manifested an acute exacerbation of IPF or needed treatment for respiratory problems during this period. After maintaining 4 years of improved status (based on PFT and exercise testing findings) while adhering to treatment for acid GER, one patient's deterioration correlated with poor compliance to daily treatment during the fifth year, although the PFT results at the sixth year showed stabilization compared to baseline values. The condition of another patient was stabilized by adhering to anti-acid GER treatment after an initial period of deterioration that was associated with nonadherence CONCLUSIONS: Future clinical studies are indicated to clarify the role of acid GER in IPF and to determine whether adequate treatment for increased acid GER in part improves the outcome of patients with IPF.