The process involves phases of injury, inflammation, and repair. Pathophysiology Īlthough there are numerous known causes of non-IPF, the pathogenesis is similar between most diseases. Further studies are required to understand the role of chemical mediators in patients presenting with PF, so that theraputic interventions can be progressed in years to come to limit the burden of this prevalent health condition. Although there are a number of compensatory and redundant processes which occur in the body to contribute to proficient healing and remodeling, there is a lack of therapeutic intervention and new therapies to treat the diease. In patients presenting with PF, the development and progression of the healing response has slipped out of control, disrupting many delicate cycles occuring in the body's respiratory system. Though, it might be also seen in middle-aged adults, particularly in those with familial risk for pulmonary fibrosis IPF tends to be commoner in males, with most cases presenting in those over 60 years of age.The estimated prevalence ranges from 25 to 74 per 100,000 population. The overall incidence of ILD of all types is 31.5 per 100,000 in men and 26.1 per 100,000 in women. Pulmonary fibrosis of all types is slightly more common in men than women and often occurs in the fifth or sixth decades of life in both the United States and worldwide.The epidemiology of non-IPF varies based on the underlying cause of the disease. The frequency of testing varies however, it’s most often completed annually Epidemiology Patients with non-IPF typically have a restrictive pattern and a decrease in DLCO. A complete PFT should include spirometry, body plethysmography, DLCO along with resting and ambulatory pulse oximetry. PFTs are essential in the evaluation and diagnosis of non-IPF and are often used to assess disease severity and monitor disease progression.
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Pulmonary function testing (PFT) should be obtained in all patients. PF clearly associated with another disease, such as scleroderma or rheumatoid arthritis, would be referred to as pulmonary fibrosis secondary to scleroderma or secondary to rheumatoid arthritis.If there is a clear association with another illness or the lung fibrosis is the result of a side effect from a medication or an exposure to an agent known to cause PF, then the cause of the disease is no longer considered idiopathic.Testing is tailored to the patient’s history and physical findings. The diagnois of this disease requires the use of a multidisciplinary team which will include pulmonologists, radiologists, and pathologists. In some cases, it may be necessary to perform a biopsy. Patients with suspected non-IPF should undergo laboratory and radiographic testing. Ageing - some studies report thin-section CT findings associated with interstitial lung disease to some degree are frequently seen in "asymptomatic" elderly individuals.Granulomatous conditions ( eg sarcoidosis, tuberculosis, granulomatosis with polyangiitis).Congenital conditions eg cystic fibrosis, Hermansky-Pudlak syndrome.Radiation: radiation-induced pulmonary fibrosis.Inhaled substances eg coal/silica: progressive massive fibrosis, asbestos: asbestos-related pulmonary fibrosis.Significant acute insult to the lungs eg adult respiratory distress syndrome, from a significant pulmonary infection, diffuse alveolar damage from any source.The etiology for the most common causes are often categorized, as shown below: There are over 200 known causes of non-IPF.
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Pulmonary fibrosis can be localised, segmental, lobar, or affect the entirety of the lung(s). Fibrosis in the lung is a process that occurs in the interstitium.