Introduction to Idiopathic Pulmonary Fibrosis

We know relatively little about idiopathic pulmonary fibrosis in our daily lives, mainly because the incidence of this disease is relatively small. However, idiopathic pulmonary fibrosis may cause joint pain and difficulty breathing. We recommend that everyone pay attention to it in life so that we can detect the presence of this disease in time.

Idiopathic pulmonary fibrosis is an inflammatory disease of the lung interstitium of unknown cause. Unexplained alveolar fibrosis and diffuse pulmonary fibrosis are synonyms. Typical IPF is mainly manifested by dry cough and progressive dyspnea, which gradually worsens over months or years and often progresses to terminal respiratory failure or death within 3 to 8 years of the onset of symptoms.

The main pathological features are the mixed presence of fibrosis and inflammatory cell infiltration in the lung interstitium and alveolar cavity. Although the pathogenesis of the disease has not been fully elucidated, its clinical features and pathology are sufficient to indicate that it is a characteristic disease. There is still a lack of objective and decisive prognostic factors or treatment responses in the treatment of IPF. Corticosteroids (hereinafter referred to as hormones) or immunosuppressants and cytotoxic drugs are still the main treatment drugs, but less than 30% of patients have a treatment response, and toxic and side effects may occur.

Symptoms initially include dry cough and difficulty breathing. For most patients, progressive dyspnea is the primary and most prominent symptom. Another common symptom is an irritating, dry cough that often does not respond to cough suppressants. Generally there are no extrapulmonary manifestations, but there may be weight loss, discomfort, fatigue, etc. Fever is rare, but high fever may occur when combined with infection. Sometimes joint pain also occurs, and rarely chest pain.

The respiratory rate increases when at rest, and critically ill patients all have cyanosis and hypoxemia. Auscultation of the lungs may reveal fine inspiratory crackles (velcro rales), usually located at the lung bases. In 80% of patients, as the disease progresses, lung rales may spread to the lung apex; bronchial breath sounds indicate lung consolidation; and 25% to 50% of patients have clubbing.

If we discover idiopathic pulmonary fibrosis in life, we recommend that patients should deal with it well in life, take care of their bodies according to the doctor's advice, pay more attention to their respiratory tract in life, and try to go out and breathe some fresh air, so as to avoid the recurrence of idiopathic pulmonary fibrosis.