Patients with Idiopathic Pulmonary Fibrosis (IPF) often enjoy prolonged periods of stability where their disease does not progress. Other patients may have very gradual worsening of symptoms. On this background, IPF patients at times may experience more abrupt decline. Many patients first notice increased cough followed by increased shortness of breath. This pattern should prompt you to notify your lung doctor with some urgency.
Increased IPF Symptoms
There is a short list of common problems that can lead to increased shortness of breath that develops over a period of days to a few weeks in patients with IPF. There are four common disorders that need to be considered in addition to an exacerbation of IPF.
1. Pneumonia: it is very difficult to exclude pneumonia in a patient with IPF. The classic symptoms of pneumonia are cough, shortness of breath, fever and sputum in conjunction with an abnormal chest Xray. Many of these symptoms are always present in IPF patients.
2. Pulmonary Embolism: the sudden worsening of shortness of breath in an IPF patient should bring to mind the possibility of a blood clot in the lungs. Specialized CT scanning is the best test to evaluate for this.
3. Pneumothorax: sudden onset chest pain and shortness of breath can occur with a collapse of the lung. Chest Xray and CT scanning can rapidly identify this problem
4. Heart failure: the progressive increase in shortness of breath, cough and intolerance of lying down flat can be seen with heart failure. Excluding heart failure as a contributing factor can be a challenge. Echocardiography (ultrasound assessment of the heart), certain blood tests and response to diuretics can all be useful in excluding heart failure.
Patients with a diagnosis of IPF that experience more rapid decline that develops over a few days to a month that is not due to pneumonia, pulmonary embolism, pneumothorax, heart failure or worsening of another non-IPF disease are diagnosed as having an IPF exacerbation or flare.
Urgent evaluation with chest Xray, CT scanning of the lungs, blood tests and at times echocardiography are performed. Patients that are declining rapidly are hospitalized, as are patients that are very short of breath or have marked decline in their oxygen saturations.
Management of IPF Exacerbation
There are no good research studies that provide high quality evidence supporting a specific approach. However, lung doctors expert in IPF have developed a standard treatment approach.
1. Treat with broad spectrum antibiotics
2. Treat with high doses of steroids
3. Initiate anticoagulation until blood clots are fully excluded
4. Provide increased oxygen and mechanical ventilation if needed
5. Urgent lung transplantation evaluation if appropriate
6. Consider transfer to a facility that performs lung transplantation
7. Consider ECMO (artificial lung support) in patients that are candidates for lung transplantation who require mechanical ventilation
Prognosis for Pulmonary Fibrosis Exacerbation
Mild exacerbations generally improve with steroids and patience. More severe exacerbations may be fatal. Patients with IPF exacerbations that require mechanical ventilation (support from a breathing machine with a breathing tube down the throat) are at particularly high risk of dying.
Prevention of IPF Exacerbation
The two FDA approved agents Esbriet (Pirfenidone) and OFEV (Nintedanib) both probably decrease the likelihood of an exacerbation. Scientists are working diligently to better understand what causes exacerbations and how to prevent them.