Stages of pulmonary fibrosis

In the early stages of pulmonary fibrosis, patients initially suffer only a cough and shortness of breath on exertion.In most cases, however, the disease then develops further. In the advanced stage the symptoms are more severe. The lack of oxygen in the blood leads to cyanosis (blue coloration of the lips).

Patients have difficulty breathing even at rest. The ability to cope with stress is extremely limited. Due to the increased pressure in the pulmonary circulation, there is also right heart strain.

The final stage of pulmonary fibrosis is a “honeycomb lung“, the lung tissue has receded so much that it has been replaced to a large extent by connective tissue cavities. Pulmonary fibrosis is not a uniform clinical picture. Rather, it is a collective term for a wide variety of diseases in which connective tissue in the lung is proliferating.

The course of the disease is therefore often very different. It usually depends on whether the triggering factor can be identified and eliminated. Even though in most cases there is no causal therapy and the connective tissue remodeling is irreversible, early diagnosis is important to promote a lung-friendly lifestyle.

Smoking accelerates the course of the disease immensely. Absolute nicotine withdrawal is recommended for patients with pulmonary fibrosis. In the idiopathic form of pulmonary fibrosis, the trigger is unknown.

The disease is usually chronically progressive, i.e. it progresses over time. The symptoms worsen, in the final stage patients already suffer from respiratory distress at rest. A long-term oxygen therapy is necessary.

The increasing pressure in the pulmonary circulation leads to an increased load on the right side of the heart. The patients then suffer from right heart failure. In the final stage of pulmonary fibrosis, patients are very seriously ill and can hardly get out of bed.

Most patients in the final stage die. The prognosis of pulmonary fibrosis depends mainly on its cause. If the trigger is known and could be eliminated early, the prognosis is better.

However, if it is an idiopathic form whose progression can only be prevented with difficulty, the prognosis is poor. Basically, pulmonary fibrosis is a serious disease that usually leads to a significant deterioration in quality of life. Life expectancy is also significantly reduced.

It is usually two to five years after diagnosis, as there is unfortunately still no causal therapy for most forms. For younger patients with pulmonary fibrosis, lung transplantation is therefore usually the only hope. Patients with end-stage lung fibrosis are very seriously ill.

Most of them die. The patients are bedridden. They suffer from shortness of breath even at rest.

The lips are discolored bluish (cyanosis). Physical strain is hardly possible any more. All patients are dependent on additional oxygen almost every minute.

This can also be done at home as part of a long-term oxygen therapy. Due to the increased strain on the right heart, patients also suffer from the symptoms of right heart weakness with leg edema, neck vein congestion and abdominal fluid caused by congested liver. At such a stage, lung transplantation is no longer possible, so most patients die in the course of the disease.