Lung fibrosis occurs worldwide and carries a substantial burden of disease in affected persons. It is commonly characterized by dry, persistent cough and shortness of breath, with a detrimental effect on the quality of life. This disease, which is still without a cure, often progresses with a negative impact on blood oxygen levels, culminating in poor outcomes such as death or the need for lung transplantation. In the United States, up to 28 individuals per 100,000 persons are affected by the progressive form of lung fibrosis. While the cause is often unknown, it can occur following a broad range of causes such as sarcoidosis, connective tissue diseases, exposure to various medications, inhaled environmental particles and toxins, and following a genetic predisposition in specific individuals. It is also a well-recognized complication of acute respiratory distress syndrome, which frequently follows severe lung infections such as infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and other systemic diseases.
The diagnosis of lung fibrosis requires a comprehensive initial patient assessment, including a careful medical history, detailed physical examination, lung function tests, radiologic assessment with high resolution computed tomography scans, and blood tests to evaluate for the presence of underlying connective tissue diseases. As lung fibrosis symptoms are commonly observed in several other conditions, an early and accurate diagnosis requires the exclusion of similar diseases and often requires multidisciplinary team discussion. A review of the available clinical information by the multidisciplinary team may ascertain the diagnosis or guide the need for additional diagnostic tests such as lung biopsy.
The approach to treatment depends on the type of lung fibrosis. As no curative therapies are available presently, treatment is focused on reducing symptom burden, improving quality of life, slowing down disease progression, and treating comorbid conditions. The patient's type of lung fibrosis and disease course also guides the decision to initiate either antifibrotic medications or immunosuppressive therapies. These shared decision-making processes are performed in concurrence with the patient, balancing the benefits of preserving lung function with the potential adverse effects of medications. Supplemental oxygen when indicated, pulmonary rehabilitation, treatment of coexisting conditions, and caregiver education are all essential facets of care. In addition, lung transplantation and palliative care should be taken into consideration, especially in patients with persistent disease progression despite appropriate interventions.
Recent efforts geared towards a better understanding of the underlying mechanisms of disease, genetic associations, and clinical disease course continue to shape practical guidelines for classification and management of disease subtypes. We hope that the Lung Scarring and Fibrosis Week will highlight the need for further research and increased collaborative efforts in the quest for a cure to this enigmatic disease.
