Shane Shapera

Treatment of Gastroesophageal Reflux in Patients With Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis

Background: Gastroesophageal reflux (GER) is common in patients with idiopathic pulmonary fibrosis (IPF) and has been proposed as a potential contributor to disease progression and exacerbation. Whether treatment of GER improves health outcomes in patients with IPF is controversial. Our objective was to review the efficacy and safety of GER treatments in IPF. Methods: We performed a systematic review and meta‐analysis, using MEDLINE, Embase, Central (Cochrane Central Register of Controlled Trials), and ClinicalTrials.gov. These databases were searched from their inception, and Google Scholar and conference abstracts were searched until September 2017 without language restrictions for randomized and observational studies evaluating the effects of pharmacologic or nonpharmacologic treatment. Primary outcomes were IPF‐related mortality and all‐cause mortality. Evidence was evaluated according to GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The study was registered with PROSPERO (#CRD42017076257). Results: Thirteen observational studies were identified through the search strategy, and eight were included in the meta‐analysis. Pharmacologic treatment of GER was associated with a significant reduction in IPF‐related mortality as compared with no GER treatment (unadjusted risk: HR, 0.60; 95% CI, 0.38–0.97; P = .04; I2 = 0%; three studies; N = 2,033; adjusted risk: HR, 0.45; 95% CI, 0.24–0.84; P = .01; I2 = 0%; three studies; N = 2,033) but not all‐cause mortality (unadjusted: HR, 0.73; 95% CI, 0.45–1.2; P = .22; I2 = 46%; three studies; N = 1,316; adjusted: HR, 0.76; 95% CI, 0.31–1.84; P = .54; I2 = 89%; four studies; N = 1,585). The quality of evidence for these outcomes was low. Conclusions: Low‐quality evidence suggests pharmacologic treatment of GER is associated with a reduction in IPF‐related mortality but not overall mortality. Randomized trials of antacid therapy in IPF are needed.

Rheumatoid Arthritis Interstitial Lung Disease

Rheumatoid arthritis (RA) is a systemic, autoimmune, inflammatory disorder affecting 0.51% of the North American population (Gabriel, 2001). It has a predilection for young women with an incidence rate of up to 130 per 100,000 compared with 70 per 100,000 in men [Minaur et al, 2004]. It is associated with a median survival decrease of up to 11 years compared to the general population (Minaur et al., 2004). The disease course may be complicated by extra-articular manifestations that confer an added burden of morbidity and mortality. RA-associated cardiovascular and infectious complications are commonly highlighted as major causes of morbidity and mortality in these patients (Maradit-Kremers et al., 2005). However, pulmonary involvement, the third leading extra-articular manifestation of RA, is now also recognized as a major cause of morbidity and mortality in RA patients. This was demonstrated in an autopsy study of 81 RA patients where the cause of death was determined to be infectious in 23.5%, cardiovascular in 17.3% and respiratory in 9.9% of patients (Suzuki et al., 1994). Pulmonary complications are the presenting manifestation of RA in up to 20% of patients (Brown, 2007). These complications include airway disease, pleural effusion, pulmonary nodules, and interstitial lung disease (ILD). This chapter will discuss the epidemiology, clinical features, management of RA-associated ILD (RA-ILD) and highlight the links between pulmonary involvement and autoimmunity.

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

308 A 70-year-old-woman with idiopathic pulmonary fibrosis (IPF) presented with worsening dyspnea, nonproductive cough and increasing home oxygen requirements over a 24 h period. IPF had been previously diagnosed in accordance with American Thoracic Society guidelines (1); having a consistent clinical presentation, absence of alternative diagnosis and a definite usual interstitial pneumonia pattern on chest computed tomography (CT). History included hemochromatosis, diabetes, hypertension and osteoarthritis. Pirfenidone was initiated two months before presentation. Physical examination revealed clubbing and bilateral basal-predominant fine crackles, consistent with IPF without evidence of heart failure. A routine CT scan of the chest had been performed two days before developing increased dyspnea (Figure 1A) and a repeat contrastenhanced CT chest scan at the time of hospitalization (Figure 1B). There was interval development of diffuse ground-glass opacities (GGO) in both lungs without evidence of pulmonary embolism. Sputum cultures and a nasopharyngeal swab (for polymerase chain reaction) failed to identify bacterial, fungal or viral infection. Bronchoscopy could not be performed due to high oxygen requirements. Treatment with 1 g of intravenous methylprednisilone was given for three days, followed by 50 mg of prednisone. Dyspnea and oxygen requirements returned to baseline. Repeat imaging at one week (Figure 1C) showed interval resolution of GGO. The patient was discharged home with plans to taper off her prednisone over a four-week period. REFERENCES 1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ ALAT statement: Idiopathic pulmonary fibrosis: Evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788-824. 2. Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007;176:636-43.

Evaluation of patients with fibrotic interstitial lung disease: A Canadian Thoracic Society position statement

ABSTRACT The evaluation of a patient with fibrotic interstitial lung disease (ILD) includes assessment of clinical, radiological, and often histopathological data. There are currently no specific recommendations to guide the evaluation of a patient with fibrotic ILD within the context of the Canadian practice landscape. This position statement from a multidisciplinary panel of ILD experts provides guidance related to the diagnostic modalities commonly used in the evaluation of fibrotic ILD, including radiological studies, histopathologic sampling, assessment for rheumatologic disease and need for evaluation in a multi-disciplinary setting. Key messages are provided to guide clinical practice based on a thorough review of the scientific literature.

Diagnostic disparity of previous and revised American Thoracic Society guidelines for idiopathic pulmonary fibrosis.

BACKGROUND A revised guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) was formulated by the American Thoracic Society (ATS) in 2011 to improve disease diagnosis and provide a simplified algorithm for clinicians. The impact of these revisions on patient classification, however, remain unclear. OBJECTIVE To examine the concordance between diagnostic guidelines to understand how revisions impact patient classification. METHODS A cohort of 54 patients with either suspected IPF or a working diagnosis of IPF was evaluated in a retrospective chart review, in which patient data were examined according to previous and revised ATS guidelines. Patient characteristics influencing the fulfillment of diagnostic criteria were compared using one-way ANOVA and χ(2) tests. RESULTS Revised and previous guideline criteria for IPF were met in 78% and 83% of patients, respectively. Revised guidelines modified a classification based on previous guidelines in 28% of cases. Fifteen percent of patients meeting previous ATS guidelines failed to meet revised criteria due to a lack of honeycombing on high-resolution computed tomography and the absence of a surgical lung biopsy. Patients failing to meet previous and revised diagnostic criteria for IPF were younger. CONCLUSION The revised guidelines for the diagnosis of IPF classify a substantial proportion of patients differently than the previous guidelines.

The Canadian Registry for Pulmonary Fibrosis: Design and Rationale of a National Pulmonary Fibrosis Registry

Background. The relative rarity and diversity of fibrotic interstitial lung disease (ILD) have made it challenging to study these diseases in single-centre cohorts. Here we describe formation of a multicentre Canadian registry that is needed to describe the outcomes of fibrotic ILD and to enable detailed healthcare utilization analyses that will be the cornerstone for future healthcare planning. Methods. The Canadian Registry for Pulmonary Fibrosis (CARE-PF) is a prospective cohort anticipated to consist of at least 2,800 patients with fibrotic ILD. CARE-PF will be used to (1) describe the natural history of fibrotic ILD, specifically determining the incidence and outcomes of acute exacerbations of ILD subtypes and (2) determine the impact of ILD and acute exacerbations of ILD on health services use and healthcare costs in the Canadian population. Consecutive patients with fibrotic ILD will be recruited from five Canadian ILD centres over a period of five years. Patients will be followed up as clinically indicated and will complete standardized questionnaires at each clinic visit. Prespecified outcomes and health services use will be measured based on self-report and linkage to provincial health administrative databases. Conclusion. CARE-PF will be among the largest prospective multicentre ILD registries in the world, providing detailed data on the natural history of fibrotic ILD and the healthcare resources used by these patients. As the largest and most comprehensive cohort of Canadian ILD patients, CARE-PF establishes a network for future clinical research and early phase clinical trials and provides a platform for translational and basic science research.

Bronchial Associated Lymphoid Tissue Lymphoma in Systemic LupusErythematosus Successfully Treated with Rituximab

We present, for the first time to our knowledge, a patient with Systemic Lupus Erythematosus (SLE) with pulmonary Bronchial-Associated Lymphoid Tissue (BALT) lymphoma, refractory to chemotherapy but after a single four-week course of rituximab experienced significant regression of pulmonary lesions and remained progressionfree six months post- treatment. This case report demonstrates the promising role for rituximab in refractory BALT lymphoma.

Transbronchial lung cryobiopsy for ILD: Ready or not, here it comes?

Kerri A. Johannson, Martin Kolb, Charlene D. Fell, Deborah Assayag, Jolene Fisher, Andrew Churg, Kaïssa de Boer, Margaret M. Kelly, Andrew G. Lee, H el ene Manganas, Shikha Mittoo, Shane Shapera, Kazuhiro Yasufuku, and Christopher J. Ryerson Departments of Medicine, University of Calgary, Calgary, Alberta, Canada; Community Health Sciences, University of Calgary, Calgary, Alberta, Canada; Department of Medicine, McMaster University, Hamilton, Ontario, Canada; Department of Medicine, Montr eal Jewish General Hospital, Montr eal, Quebec, Canada; Department of Medicine, University of Toronto, Toronto, Ontario, Canada; Department of Pathology, University of British Columbia, Vancouver, British Columbia, Canada; Departments of Medicine, The Ottawa Hospital, Ottawa, Ontario, Canada; Department of Pathology, University of Calgary, Calgary, Alberta, Canada; Department of Radiology, University of Calgary, Calgary, Alberta, Canada; Department of Medicine, University of Montr eal, Montr eal, Quebec, Canada; Department of Thoracic Surgery, University of Toronto, Toronto, Ontario, Canada; Department of Medicine and Centre for Heart Lung Innovation, University of British Columbia and St. Paul’s Hospital, Vancouver, British Columbia, Canada

Comprehensive management of fibrotic interstitial lung diseases: A Canadian Thoracic Society position statement

Abstract The comprehensive management of patients with fibrotic interstitial lung disease (ILD) is multi-faceted and may include pharmacological and non-pharmacological therapies. There are no current recommendations and few resources to guide the management of patients with fibrotic ILD in Canada. This position statement provides recommendations for the management of patients with fibrotic ILD based on review of the scientific literature and consensus from a panel of ILD experts. These recommendations relate to important clinically relevant questions, and key messages are provided to guide clinical practice.