Newly Diagnosed: Taking the First Step on Your Journey

Pulmonary fibrosis (PF) is a disease marked by scarring — or fibrosis — of lung tissues, making breathing difficult. This disease is part of a larger group of some 200 different lung conditions all marked by inflammation and scarring, and known as interstitial lung diseases (ILD). Idiopathic pulmonary fibrosis (IPF) is its most common form and, like all ILDs, is progressive and serious.

Different PF types all lead to scarring in the lungs but can be caused by different factors. Most PF cases are caused by unknown reasons, which are diagnosed as IPF. In some cases, doctors observe a pattern of pulmonary fibrosis in families, which suggests that genetics may cause PF in certain diagnosed patients. Those types of cases are referred to as familial pulmonary fibrosis.

PF can be caused by an autoimmune disease such as rheumatoid arthritis or systemic scleroderma, or by certain muscle diseases including polymyositis, dermatomyositis, or anti-synthetase syndrome. Occupational and environmental exposure to toxic substances can cause irritation of the cells in the lungs and result in scarred tissue. There are also medications that can cause lung damage and PF. Genetics may also play a role in some cases.

The most common symptoms of PF are shortness of breath and coughing. Patients often experience severe symptoms only in advanced stages of the disease; these can include breathing alterations, weight loss, fatigue, muscle and joint pain, and clubbing of the hands and feet. Every patient has their own individual experience with the disease, which means it can progress faster in some patients and slower in others.

When a patient experiences symptoms that can indicate PF, the diagnostic process starts by analyzing their medical history. Additionally, the physician may order a series of tests and examinations to confirm a diagnosis. These include an arterial blood gas test, bronchoscopy, chest X-ray, spirometry, electrocardiogram, high-resolution computed tomography scan, exercise test, pulse oximetry, and surgical biopsy.

Lifestyle changes may make living with PF easier. There are a number of things people living with pulmonary fibrosis can do to maintain or improve their quality of life, including doing some gentle exercise, adopting a healthy diet, resting as necessary, quitting smoking, using relaxation techniques to reduce stress, joining a support group, and getting help from healthcare professionals.