Catamenial Pneumothorax in the Setting of a Recent Stroke.

Catamenial pneumothorax is a unique condition associated with thoracic endometriosis. It often presents in females of reproductive age as a recurrent pneumothorax aligned with the menstrual cycle. We present a case of a young female diagnosed with catamenial pneumothorax within one year of experiencing a stroke. The clinical presentation related to the stroke allowed for a unique diagnostic process and management considerations. The patient was successfully treated with progesterone-based contraception in the setting of an estrogen contraindication.

The Role of Surgical Lung Biopsy in the Diagnosis of Fibrotic Interstitial Lung Disease: Perspective from the Pulmonary Fibrosis Foundation.

Diagnosis of interstitial lung disease (ILD) requires a multidisciplinary discussion approach that includes clinicians, radiologists, and pathologists. Surgical lung biopsy (SLB) is currently the recommended standard in obtaining pathologic specimens for patients with ILD requiring a tissue diagnosis. The increased diagnostic confidence and accuracy provided by microscopic pathology assessment of SLB specimens must be balanced with the associated risks in patients with ILD. This document was developed by the SLB Working Group of the Pulmonary Fibrosis Foundation, composed of a multidisciplinary group of ILD physicians, including pulmonologists, radiologists, pathologists, and thoracic surgeons. In this document, we present an up-to-date literature review of the indications, contraindications, risks, and alternatives to SLB in the diagnosis of fibrotic ILD; outline an integrated approach to the decision-making around SLB in the diagnosis of fibrotic ILD; and provide practical information to maximize the yield and safety of SLB.

Optimization of patient-specific inhaler regimens with the use of the aerosol inhalation monitor.

OBJECTIVES: To describe the addition of a Vitalograph Aerosol Inhalation Monitor (AIM) assessment to a pharmacy bundle to optimize inhaler devices in patients with asthma and chronic obstructive pulmonary disease (COPD). SETTING: Hospital-based outpatient pulmonary clinic. PRACTICE DESCRIPTION: Ambulatory pharmacy service for underserved pulmonary patients in Detroit, MI. PRACTICE INNOVATION: Patients with asthma and COPD received a pharmacy bundle service that included inhaler technique assessment with the use of the AIM. Based on the patient's performance, an optimized pulmonary regimen was developed for the patient in collaboration with the pulmonologists. Follow-up telephone calls were made 1 and 4 weeks after the visit. EVALUATION: A 1-group pretest-posttest quasiexperimental study was conducted over a 6-month period. The primary objective of the study was to describe the impact that a pharmacist had on optimizing individuals' inhaled regimens. In addition, clinical outcomes including changes in asthma control test (ACT) and COPD assessment test (CAT) scores, rescue inhaler use, and patient adherence were assessed. RESULTS: A total of 44 patients were included in the study, of which 27 (61%) were determined to be on an inappropriate inhaler regimen according to their AIM assessment. The pharmacist subsequently made recommendations to change the device(s) for those patients, with the most common recommendation being a change to a nebulized regimen. There was a significant improvement in ACT/CAT scores, patient-reported inhaler use, and patient adherence at week 4 compared with baseline. CONCLUSION: Assessing inhaler technique with the use of the AIM allows pharmacists to identify an optimized inhaled regimen for patients with asthma and COPD and may be a potential solution to the problem of poor inhaler technique.

Patients' perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases.

The effects of interstitial lung disease (ILD) create a significant burden on patients, unsettling almost every domain of their lives, disrupting their physical and emotional well-being and impairing their quality of life (QoL). Because many ILDs are incurable, and there are limited reliably-effective, life-prolonging treatment options available, the focus of many therapeutic interventions has been on improving or maintaining how patients with ILD feel and function, and by extension, their QoL. Such patient-centred outcomes are best assessed by patients themselves through tools that capture their perceptions, which inherently incorporate their values and judgements. These patient-reported outcome measures (PROs) can be used to assess an array of constructs affected by a disease or the interventions implemented to treat it. Here, we review the impact of ILD that may present with a progressive-fibrosing phenotype on patients' lives and examine how PROs have been used to measure that impact and the effectiveness of therapeutic interventions.

Predischarge bundle for patients with acute exacerbations of COPD to reduce readmissions and ED visits: a randomized controlled trial.

BACKGROUND: Hospital readmissions for acute exacerbations of COPD (AECOPDs) pose burdens to the health-care system and patients. A current gap in knowledge is whether a predischarge screening and educational tool administered to patients with COPD reduces readmissions and ED visits. METHODS: A single-center, randomized trial of admitted patients with AECOPDs was conducted at Henry Ford Hospital between February 2010 and April 2013. One hundred seventy-two patients were randomized to either the control (standard care) or the bundle group in which patients received smoking cessation counseling, screening for gastroesophageal reflux disease and depression or anxiety, standardized inhaler education, and a 48-h postdischarge telephone call. The primary end point was the difference in the composite risk of hospitalizations or ED visits for AECOPD between the two groups in the 30 days following discharge. A secondary end point was 90-day readmission rate. RESULTS: Of the 172 patients, 18 of 79 in the control group (22.78%) and 18 of 93 in the bundle group (19.35%) were readmitted within 30 days. The risk of ED visits or hospitalizations within 30 days was not different between the groups (risk difference, -3.43%; 95% CI, -15.68% to 8.82%; P = .58). Overall, the time to readmission in 30 and 90 days was similar between groups (log-rank test P = .71 and .88, respectively). CONCLUSIONS: A predischarge bundle intervention in AECOPD is not sufficient to reduce the 30-day risk of hospitalizations or ED visits. More resources may be needed to generate a measurable effect on readmission rates. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT02135744; URL: www.clinicaltrials.gov.

Longitudinal evaluation of PROMIS-29 and FACIT-dyspnea short forms in systemic sclerosis.

OBJECTIVE: To assess the sensitivity of the Patient-Reported Outcomes Measurement Information System 29-item Health Profile (PROMIS-29) and the Functional Assessment of Chronic Illness Therapy-Dyspnea 10-item short form (FACIT-Dyspnea) for measuring change in health status and dyspnea in systemic sclerosis (SSc). METHODS: One hundred patients with SSc completed the PROMIS-29, FACIT-Dyspnea, and traditional instruments [Medical Research Council Dyspnea Score, St. George's Respiratory Questionnaire (SGRQ), Health Assessment Questionnaire-Disability Index (HAQ-DI), and Medical Outcomes Study Short Form-36 (SF-36)] at baseline and 1-year visits. PROMIS-29, FACIT-Dyspnea, and traditional instrument change scores were compared across composite modified Medsger Disease Severity and modified Rodnan Skin score (mRSS) change groups. RESULTS: Moderately high Spearman correlation coefficients were observed between FACIT-Dyspnea and SGRQ (r = 0.57), FACIT-Dyspnea functional limitations and SF-36 physical component summary (PCS; r = 0.51), PROMIS-29 physical functioning and HAQ-DI (r = 0.50), and SF-36 PCS (r = 0.52) change scores. In most validity comparisons, PROMIS-29, FACIT-Dyspnea, HAQ-DI, and SF-36 scores performed similarly. While PROMIS-29 covers more content areas than SF-36 (e.g., sleep), it may do so at the expense of responsiveness of its 4-item physical function scale as compared to the multiitem-derived SF-36 PCS. Statistically significant increases in SF-36 role physical (p = 0.01) and physical component scale (p = 0.016), but not PROMIS-29, were observed in patients with mRSS improvement. CONCLUSION: PROMIS-29 and FACIT-Dyspnea are valid instruments to measure health status and dyspnea in patients with SSc. In physical function assessment, longer PROMIS short forms or computer adaptive testing should be considered to improve responsiveness to the effect of skin disease changes on physical function in patients with SSc.

Validity of two new patient-reported outcome measures in systemic sclerosis: Patient-Reported Outcomes Measurement Information System 29-item Health Profile and Functional Assessment of Chronic Illness Therapy-Dyspnea short form.

OBJECTIVE: Many patient-reported outcome (PRO) instruments used in systemic sclerosis (SSc) trials are limited by lack of validation, licensing fees, and complicated scoring systems. We assessed the construct validity for discriminative purposes of 2 new PRO instruments, the Patient-Reported Outcomes Measurement Information System 29-item Health Profile (PROMIS-29) and the Functional Assessment of Chronic Illness Therapy-Dyspnea short form (FACIT-Dyspnea), measuring health status and dyspnea in SSc patients. METHODS: Seventy-three patients participated in a cross-sectional study at a tertiary SSc program. PROMIS-29, FACIT-Dyspnea, and legacy PRO instruments used in clinical trials (Medical Research Council Dyspnea Score, St. George's Respiratory Questionnaire, Health Assessment Questionnaire disability index, and Short Form 36) were administered. Composite severity scores using an adaptation of the Medsger Disease Severity Index were generated using clinical, diagnostic, and laboratory information. PROMIS-29 and FACIT-Dyspnea scores were compared with legacy PRO measures and composite severity scores. RESULTS: The mean patient age (84% women) was 51 years (range 22-72 years). The mean SSc disease duration from the onset of the first non-Raynaud's phenomenon symptom was 7.2 years (range 0-45 years). Spearman's correlation coefficients across FACIT-Dyspnea and PROMIS physical functioning scores with legacy PRO instruments were generally high (range 0.50-0.86); those between PROMIS and FACIT-Dyspnea with composite disease severity scores were more modest, but statistically significant (range 0.33-0.48, P < 0.01). CONCLUSION: PROMIS-29 and FACIT-Dyspnea are valid instruments to measure the health status of SSc patients. PROMIS-29 and FACIT-Dyspnea may be preferable to legacy instruments because they are freely available in multiple languages and simple to administer, score, and interpret.

Role of von Hippel-Lindau protein in fibroblast proliferation and fibrosis.

Idiopathic pulmonary fibrosis (IPF) is characterized by exaggerated fibroblast proliferation and accumulation of collagens and fibronectin. The extracellular fibronectin and collagen network is regulated by von Hippel-Lindau protein (pVHL). However, it is unknown whether pVHL contributes to pulmonary fibrosis. We found that lungs from patients with IPF expressed increased levels of pVHL in fibroblastic foci. Bleomycin treatment also induced pVHL in lung fibroblasts, but not in alveolar type II cells. Overexpression of pVHL increased lung fibroblast proliferation, protein abundance of fibronectin and collagen, and extracellular fibronectin. In addition, overexpression of pVHL induced expression of the α5 integrin subunit. Overexpression of pVHL did not alter hypoxia-inducible factor luciferase reporter activity and mRNA expression of vascular endothelial growth factor. Fibroblasts overexpressing pVHL were more sensitive to RGD peptide-mediated reduction in proliferation. Activating α5 and ß1 integrin increased proliferation of fibroblasts overexpressing pVHL and those cells were more resistant to the inhibition of α5 integrin. Overexpression of pVHL also increased activation of focal adhesion kinase (FAK). Moreover, suppression of pVHL prevented TGF-ß1-induced proliferation of mouse embryonic fibroblasts. Taken together, our results indicate that elevated expression of pVHL results in the aberrant fibronectin expression, activation of integrin/FAK signaling, fibroblast proliferation, and fibrosis.

Pulmonary complications of tumor necrosis factor-targeted therapy.

Tumor necrosis factor (TNF)-targeted therapies are increasingly being prescribed in the management of a variety of inflammatory and autoimmune diseases. The use of this class of medications also pose risks of developing an assortment of pulmonary side effects including infections (TB, bacterial, and fungal infections), pulmonary nodules, chronic pneumonitis/fibrosis, SLE-like reactions, vasculitis, and exacerbations of underlying lung disease. In addition to surveillance for tuberculosis prior to initiation of TNF-targeted therapy, a high level of vigilance should be maintained during administration for infectious and non-infectious complications, even years into a patient's course. The available evidence argues for caution in using these agents in patients with pre-existing lung disease and heightened suspicion of accelerated nodule formation in those with pre-existing rheumatoid nodules. Management centers on excluding infection, identifying confounders (especially methotrexate or pre-existing lung disease), and promptly discontinuing TNF-targeted therapy. In some instances, invasive procedures (e.g. bronchoscopy or VATS lung biopsy) will be necessary to establish the proper diagnosis, and the administration of steroids may be beneficial.