[1] Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing.

For example, a mutation in surfactant protein C (SP-C) has been found in some families with a history of pulmonary fibrosis.

[8] Autosomal dominant mutations in the TERC or TERT genes, which encode telomerase, have been identified in about 15% of pulmonary fibrosis patients.

In contrast, quadriplegia[16] and kyphosis[17] are examples of causes of restrictive lung disease that do not necessarily involve pulmonary fibrosis.

[24] Additionally, chromatin remodeler proteins affect the development of lung fibrosis, as they are crucial for gene expression regulation and their dysregulation can contribute to fibrotic disease progression.

[3] A video-assisted thoracoscopic surgery (VATS) under general anesthesia may be needed to obtain enough tissue to make an accurate diagnosis.

This kind of biopsy involves placement of several tubes through the chest wall, one of which is used to cut off a piece of lung for evaluation.

The removed tissue is examined histopathologically by microscopy to confirm the presence and pattern of fibrosis as well as other features that may indicate a specific cause, such as specific types of mineral dust or possible response to therapy, e.g. a pattern of so-called non-specific interstitial fibrosis.

Misdiagnosis is common because, while pulmonary fibrosis is not rare, each type is uncommon and evaluation of patients with these diseases is complex and requires a multidisciplinary approach.

[29] Slowing the progression and prevention depends on the underlying cause: The immune system is thought to play a central role in the development of many forms of pulmonary fibrosis.

The goal of treatment with immunosuppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring.

Typically, patients are in their forties and fifties when diagnosed, while the incidence of idiopathic pulmonary fibrosis increases dramatically after age 50.

The biggest concerns about pulmonary fibrosis and the increase of respiratory follow-up after COVID-19 are expected to be solved in the near future.