Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) remains a disease with poor survival. The pathogenesis is complex and encompasses multiple molecular pathways. The first-generation antifibrotics pirfenidone and nintedanib, approved more than 10 years ago, have been shown to reduce the rate of progression, increase the length of life for patients with IPF, and work for other fibrotic lung diseases. In the last two decades, most clinical trials on IPF have failed to meet the primary endpoint and an urgent unmet need remains to identify agents or treatment strategies that can stop disease progression. The pharmacotherapeutic landscape for IPF is moving forward with a number of new drugs currently in clinical development, mostly in phase I and II trials, while only a few phase III trials are running. Since our understanding of IPF pathogenesis is still limited, we should keep focusing our efforts to deeper understand the mechanisms underlying this complex disease and their reflection on clinical phenotypes. This review discusses the key pathogenetic concepts for the development of new antifibrotic agents, presents the newest data on approved therapies, and summarizes new compounds currently in clinical development. Finally, future directions in antifibrotics development are discussed.

Fatigue presentation, severity, and related outcomes in a prospective cohort following post-COVID-19 hospitalization in British Columbia, Canada.

Introduction: Increasing evidence on long-term health outcomes following SARS CoV-2 infection shows post-viral symptoms can persist for months. These symptoms are often consistent with those of Myalgic Encephalomyelitis or Chronic Fatigue Syndrome (ME/CFS). The aim of the present study was to examine the prevalence and outcome predictors of post-viral fatigue and related symptoms 3- and 6-months following symptom onset. Methods: A prospective cohort of patients hospitalized with Coronavirus disease (COVID-19) (n = 88) were recruited from a Post-COVID-19 Respiratory Clinic (PCRC) in Vancouver, Canada to examine predictors of long-term fatigue and substantial fatigue. Multivariable mixed effects analyses examined the relationship between patient predictors, including pre-existing comorbidities, patient reported outcome measures, and fatigue and substantial fatigue at follow-up. Results: The number of patients experiencing fatigue or substantial fatigue at 3 months post-infection were 58 (67%) and 14 (16%) respectively. At 6 months these numbers declined to 47 (60%) patients experiencing fatigue and 6 (6%) experiencing substantial fatigue. Adjusted analysis, for sex, age, and time, revealed the number of pre-existing comorbidities to be associated with fatigue (OR 2.21; 95% CI 1.09-4.49; 0.028) and substantial fatigue (OR 1.73; 95% CI 1.06-2.95; 0.033) at 3 months follow-up. Except for shortness of breath, self-care, and follow-up time, all follow-up variables were found to be associated with fatigue and substantial fatigue at 3 months. Conclusion: Fatigue and substantial fatigue are common after COVID-19 infection but often diminish over time. A significant number of patients continue to exhibit long-term fatigue at 6 months follow-up. Further research is needed to clarify the causality of viral infections in the development and severity of fatigue as a symptom and in meeting post-viral fatigue syndrome or ME/CFS diagnostic criteria.

Biopsy-proven recurrent, acute, familial hypersensitivity pneumonitis: A case report and literature review.

Hypersensitivity pneumonitis (HP) is characterized by inflammation of the lung parenchyma that is induced by exposure to an inhaled organic antigen. We present a case of recurrent, acute HP caused by repeated transient exposure to a down sleeping bag in a patient with a family history of chronic bird-associated hypersensitivity pneumonitis. The patient's recurrent symptoms, changes in physiology, and radiographic findings coincided with repeated exposure to this source. It was later confirmed that the patient's sister had also developed chronic HP from recurrent exposure to household birds. This case highlights recent studies implicating gene-exposure interactions in the development of HP.

Organizing Pneumonia in an Adult With Chronic Recurrent Noninfectious Osteomyelitis: A Case Report and Literature Review.

Chronic recurrent multifocal osteomyelitis (CRMO) is an idiopathic inflammatory disorder primarily of children and adolescents that is characterized by multifocal nonpyogenic relapsing and remitting inflammatory bone lesions. Pulmonary abnormalities are rarely associated with CRMO, with two reported cases of consolidation on chest CT that occurred in children. We present a case of organizing pneumonia in an adult patient with CRMO. The concurrent worsening of pulmonary and bone disease suggests that CRMO may be a rare cause of organizing pneumonia.

A pleural mass with pulmonary infiltrates.

This case study describes a 34-year-old woman presenting with a subacute history of dyspnoea associated with pleural based masses and pulmonary infiltrates. A computed tomography guided biopsy confirmed the presence of a pleural based thymoma, and a video assisted thoracoscopic lung biopsy revealed the pulmonary infiltrates to be a lymphocytic interstitial pneumonitis (LIP). The thymomas responded successfully to chemotherapy and the LIP improved following corticosteroid therapy.