Fibrosing alveolitis (idiopathic pulmonary fibrosis)
Overview
Idiopathic pulmonary fibrosis (IPF), previously referred to as fibrosing alveolitis, is a chronic lung disease characterized by progressive scarring (fibrosis) of the lung tissue. This scarring primarily affects the interstitium (the area around the alveoli), leading to reduced oxygen transfer into the bloodstream over time.
The exact cause of IPF is unknown. It is thought to result from repeated injury to the lung tissue followed by abnormal repair and excessive scarring, rather than a primary autoimmune process. While the immune system may play a role in disease progression, IPF is not classified as a traditional autoimmune disease.
Common Symptoms
Dyspnea on exertion (the sensation of running out of air and not being able to breathe fast or deeply enough during physical activity), coughing, fatigue, unintentional weight loss, arthralgia (joint pain), and finger clubbing (abnormal, rounded shape of the nail bed).
Coexisting Diseases and Conditions
Rheumatoid arthritis, partial heart failure, and bronchogenic carcinoma.
Risk Factors and Prevalence
Studies have found that the risk of developing this condition increases dramatically with age. However, it can still develop at any age. Typically the symptoms will be present for about 3 years before the diagnosis of fibrosing alveolitis occurs. There have been findings that support the idea of equal incidence between the sexes and others that report males being at a slightly higher risk. Still, other studies have found that if there is also a connective tissue condition at play, females seem to be more likely to also express fibrosing alveolitis. Some studies have shown that those living in industrial areas tend to be more commonly affected by the condition. Individuals with fibrosing alveolitis have often been noted as having experienced a viral infection prior to the condition’s onset, which leads some experts to suspect viral illnesses as a potential trigger.
Recent Research
- Pathogenic Mechanisms Underlying Idiopathic Pulmonary Fibrosis (2022)
- Molecular pathways and role of epigenetics in the idiopathic pulmonary fibrosis (2022)
- The Genetic Landscape of Familial Pulmonary Fibrosis (2023)
- Viral Infection, Pulmonary Fibrosis, and Long COVID (2023)
- Mitochondrial quality control in lung diseases: current research and future directions (2023)
- Where you live matters: Roadways, air pollution and lung function in patients with idiopathic pulmonary fibrosis (2023)
Sources
- Sources
Bois, R. M. du, & Wells, A. U. (2001). Cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis. European Respiratory Journal, 18(32 suppl), 43s–55s. https://erj.ersjournals.com/content/18/32_suppl/43s
Bourke, S. C., & Clague, H. (2000). Review of cryptogenic fibrosing alveolitis, including current treatment guidelines. Postgraduate Medical Journal, 76(900), 618–624. https://doi.org/10.1136/pmj.76.900.618
Collins, F. F., & Sieker, H. O. (1978). Fibrosing alveolitis. Disease-a-Month, 24(7), 1–35. https://doi.org/10.1016/S0011-5029(78)80005-4
Fibrosing Alveolitis—An overview | ScienceDirect Topics. (n.d.). Retrieved July 15, 2021, from https://www.sciencedirect.com/topics/pharmacology-toxicology-and-pharmaceutical-science/fibrosing-alveolitis
Thomas, P. (1978). Fibrosing alveolitis. Canadian Medical Association Journal, 119(10), 1211–1216. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1818528/