Drug-eluting stents - interventional cardiology progress in the next century. In 69 years, Japanese scholars described DPB from the perspective of pathomorphology and reported it as an independent disorder. In the United States and Europe, it wasn't until 1983 that English medical journals (such as Chest) recognized this. Japan has the same incidence rate, and China may have 10 million or more potential DPB patients. China's first confirmed DPB by pathology was found in a 74-year-old male patient in 1995, who was reported in Japan's Therapeutm Research in 1996.
The main clinical manifestations of DPB are very similar to chronic bronchitis and bronchiectasis, with characteristic changes including: (1) chronic sinusitis; (2) increased cold agglutinin titer in the majority of patients; (3) chest CT showing diffuse or scattered centrilobular shadows; (4) lung function similar to COPD, but lung diffusing capacity (DLCO) is not reduced. Diagnosing DPB with typical clinical manifestations is not difficult, and suspicious patients can also be tested with macrolide lactone (14, 15 ring) therapy. Obtaining typical pathological specimens is more challenging, with only about one-third positive rate for bronchoscopic lung biopsy (TBLB). Thoracoscopic or open lung biopsy can improve the detection rate. When inexperienced doctors or the patients' clinical performance is not typical, DPB is often confused with chronic bronchitis, bronchiectasis, pneumoconiosis, miliary tuberculosis, bronchial alveolar cancer, and other diseases. The first case report of a patient had been misdiagnosed for a long time. Before diagnosis, the patient experienced respiratory failure, became bedridden, lost life confidence, and attempted suicide twice. Clearly, in the course of advanced disease, the discovery of macrolide therapy in DPB can be described as one of the rare dramatic discoveries in the history of modern medicine. Before macrolide therapy, the 5-year survival rate for respiratory failure in DPB was only about 5%, which increased to 95% after initiating macrolide therapy. In Japan alone, macrolides have saved thousands of DPB patients' lives. About three months after we administered the first patient macrolide (clarithromycin), their condition significantly improved. Over the next two years, the patient progressed from being bedridden to walking a few kilometers.
The mechanism of macrolide treatment for DPB is still unclear. Late-stage DPB is often associated with Pseudomonas aeruginosa lung infection, but macrolide's anti-infective angle is ineffective. It is estimated that its efficacy comes from an anti-inflammatory (antiinflamation) mechanism. The discovery of macrolide therapy was accidental, made possible because a doctor in Japan prescribed it to a DPB patient. Not all macrolide antimicrobial agents can treat DPB; only 14-membered ring (such as erythromycin, clarithromycin) and 15-membered ring (azithromycin) macrolides are effective, while 16-membered ring drugs are ineffective. Initially, only 14-membered ring drugs were used, and the first case of application of 15-membered ring came from China. Since then, Japan has also used a small number of cases treated with 15-membered ring azithromycin orally twice weekly to improve patient tolerability and compliance. As for the 16-membered ring, Japanese practice has proven it to be ineffective during the treatment course. Led by Kudo Cheung, the Japanese Ministry of Health conducted two specific surveys on disorders, recommending primarily using erythromycin at 400-600mg daily, usually for two to three months, to see results, with at least six months of medication required. If early symptoms disappear and clinical examination results stabilize after 6 to 12 months, medication should continue. There are reports that some patients experience symptom recurrence after discontinuation, and re-medication remains effective in some cases. An invalid switch to clarithromycin, erythromycin, roxithromycin can also be seen, so no specific drug is recommended.
I believe that the discovery process of DPB with macrolide therapy provides the following inspiration: (1) A new scientific discovery, as long as there is sufficient evidence, must be pursued even if temporarily lacking recognition; (2) Clinical accidental phenomena should be seized well and perseverance in exploration maintained until the truth is uncovered; (3) Due to genetic and ethnic relations, illness types and responses to medication cannot be identical to foreigners, so imitation and copying should be avoided, and evidence-based medicine practices should be followed, summarizing one's own information and laws.
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