Idiopathic Pulmonary Fibrosis: Symptoms, Causes, Treatment
تليّف الرئة مجهول السبب
Idiopathic pulmonary fibrosis is a chronic scarring lung disease with no identified cause after evaluation. Breathlessness and dry cough may develop. Treatment aims to slow deterioration and improve activity and quality of life.
What is Idiopathic Pulmonary Fibrosis?
Scarring stiffens the lungs and impairs oxygen transfer. Not all pulmonary fibrosis is idiopathic: medicines, exposures and autoimmune diseases cause other forms with different management. The course varies from person to person.
Related topic: Pulmonary embolism: a different cause of sudden breathlessness.
Symptoms
Early symptoms may include breathlessness on stairs or walking and a persistent dry cough, followed by reduced exercise tolerance. Fatigue or clubbing of the fingers may occur. Similar symptoms have other cardiac or respiratory causes.
Causes
This diagnosis follows exclusion of recognized causes. Repeated injury and abnormal tissue repair are thought to contribute in susceptible people. Assessment therefore reviews work and environmental exposures, medicines, family history and possible autoimmune illness.
Risk factors
Increasing age, smoking, family history and some exposures increase susceptibility. No single risk factor establishes the diagnosis, and family history may be absent. Genetic or family assessment is discussed when the history supports it rather than routinely for everyone.
Diagnosis
Evaluation includes oxygen levels, lung-function testing and high-resolution CT. Respiratory and imaging specialists interpret findings together. Some, but not all, cases need a biopsy. Relevant autoimmune disease and exposures are assessed before confirming the diagnosis.
Treatment
Antifibrotic medicines may slow functional decline but do not remove existing scars. Pulmonary rehabilitation and nutritional support help; oxygen is prescribed according to assessed need. Selected patients may discuss transplantation, while medicines require benefit and adverse-effect monitoring.
Complications
Complications may include low oxygen, pulmonary hypertension, respiratory failure and acute exacerbations. Sleep, activity and mood may be affected. Symptom support and planning future care are part of management alongside medication.
Prevention
No strategy guarantees prevention. Avoid smoking and respiratory hazards, and discuss vaccination, infection precautions and suitable activity with the care team. Do not change prescribed oxygen flow or start immune medicines simply because a report mentions fibrosis.
When to see a doctor
Seek assessment for persistent unexplained cough or breathlessness. Rapid deterioration, chest pain, blue discolouration or altered consciousness needs emergency care. Infection, embolism or an acute exacerbation may be responsible and require assessment rather than assumption.