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Fibrosis pulmonum
Lung tissue scars and stiffens — recognised early, the course can be slowed today.
Also known as: pulmonary fibrosis · interstitial lung disease
Open in the tool — with search and all the links →This compilation is for information and replaces neither medical diagnosis nor treatment. If you have symptoms, see a doctor.
In pulmonary fibrosis the fine lung tissue gradually scars and stiffens, so oxygen passes into the blood less easily. The main signs are slowly increasing breathlessness on exertion and a dry, irritating cough. The causes are varied: sometimes it stays unexplained (idiopathic), sometimes an autoimmune disease, dust inhaled over years or a medicine is behind it. The course varies widely. Scarred tissue does not reverse, but modern treatments can slow the progression and clearly improve quality of life — specialist care (pulmonology) is central.
The decisive step: determine the type and cause precisely, since treatment depends on it.
Protect the lung from further harm — avoid smoke, dust and irritants as far as possible.
For certain forms, medicines can slow the scarring.
Targeted training and, if needed, oxygen improve breathing and daily life.
Pirfenidone and nintedanib slow the loss of lung function by about half — they restore nothing, and scarred lung stays scarred. It is judged not by whether things get better but by how fast they get worse; that is an unfamiliar measure. Cortisone does not help in the classic form and can harm — in other forms of lung scarring it does, which is why the precise classification decides so much.
Lung function is measured every three to six months; a fall in vital capacity of more than ten per cent a year counts as progressive. Untreated, on average about 150 to 200 millilitres of vital capacity are lost per year; on treatment about half that. Oxygen is measured while walking, not at rest — the drop shows first under exertion. A rehabilitation programme over eight to twelve weeks improves the walking distance measurably.
At first only on exertion, later also at rest.
Stubborn and without phlegm, often over months.
Less stamina, because the body is less well supplied with oxygen.
Rounded, thickened fingertips can appear with long-standing oxygen shortage.
Some interstitial lung diseases belong to an underlying rheumatic illness. This search changes the treatment fundamentally and belongs at the start.
Part of the same question. Together with the CCP antibodies it separates the rheumatic cause from the standalone form.
Helps to tell an acute infection from a worsening of the underlying illness — the two look the same in everyday life.
With breathlessness, anaemia belongs ruled out before everything is attributed to the lung.
Low levels are common in chronic airway illnesses and go along with more infections. Correcting a deficiency is simple; for lung function itself it is no replacement for treatment.
Relaxes the smooth muscle of the airways — which is why it is also given as an infusion in an emergency. For everyday use the tablet replaces no inhaler.
Drinking enough keeps the mucus thin and easier to cough up. That is the simplest and most often overlooked lever with sticky sputum.
The breathing muscles are muscle too. With chronic breathlessness the need for energy and protein is raised, and a loss of weight clearly worsens what one can manage.
Clearly worthwhile and well studied when deficient; fewer data for the wider uses. Fat-soluble — check blood levels at high doses.
Effective when deficient; effects on sleep and stress plausible but moderate. Too much causes loose stool.
With the idiopathic form cortisone has no benefit — in a large trial the combination even did harm. With other forms of lung scarring it is the treatment. The difference decides everything.
Where cortisone does run over months, calcium, vitamin D and bone density belong in from the start. With sarcoidosis the opposite applies for vitamin D — that is the exception one has to know.
With limited lungs chewing and swallowing tire. Smaller, energy-dense meals and food that causes little gas — a full belly presses on the diaphragm — make breathing easier.
In pulmonary fibrosis reflux is more common than average and suspected of sustaining the scarring. Staying upright after eating and a 3-hour gap before night are therefore more than comfort.
Pursed-lip breathing and calm technique help with breathlessness in daily life. Pulmonary rehabilitation improves walking distance and quality of life in studies — the scarring itself does not reverse.