Effectiveness and safety evaluation of Chinese medicine in the treatment of patients with PPF with phlegm heat-blood stasis obstructing in the lung: a randomized controlled, open-label clinical trial

INTERVENTION: experimental group:Mahuang, Kuxingren, Gancao, Fengfang, Dilong, Chishao, Jiezi, Laifuzi, Zisuzi, Huangqi, Huangqin, Qinghao;control group:1. All patients will take pulmonary rehabilitation therapy; 2. Patients with hypoxia at rest, will take long‐term oxygen therapy; 3. Patients with pulmonary infection will take empirical anti‐infective treatment, and targeted anti‐infective treatment will be given after obtaining the results of drug sensitivity test. For the dosage of hormones and immunomodulators, our recommendations are summarised below: (1) Glucocorticoid: the initial dose of glucocorticoid can range from oral prednisone (1mg·kg‐1·d‐1) to intravenous methylprednisolone (500‐1000 mg/d) for 3 days, then reduced to prednisone or equivalent dose, gradually reduced to maintenance dose in 4‐8 weeks according to the patient's condition and treatment response, and maintained close follow‐up; (2) Cyclophosphamide: adhere to the daily use of 2mg, the initial dose is prescribed daily oral 25mg‐50mg, and increase 25mg every other week to two weeks, according to this cycle until the maximum dose reaches the daily 150mg; (3) Azathioprine: administered at 2‐3 mg·kg‐1·d‐1. The initial dose is 25‐30mg/d, and then 25mg will be increased every 7 to 14 days until the maximum amount of 150mg/d is reached; In addition, if the subjects had taken oral corticosteroids for more than one month before entering the trial, and the hormone had reached the maintenance dose, or if they had taken oral immunomodulators for more than one month before entering the trial, the original drug treatment regimen should not be changed during the clinical observation, the investigators should record it completely and truthfully.; CONDITION: progressive pulmonary fibrosis(PPF) PRIMARY OUTCOME: Leicester Cough Questionnaire (LCQ); SECONDARY OUTCOME: The University of California, San Diego (UCSD) Shortness of Breath Questionnaire (SOBQ);The Fatigue Assessment Scale (FAS);Forced Vital Capacity (FVC) decline value (absolute value, mL) and percentage (proportion, %);Carbon Monoxide Dispersion (DLco) decline value (absolute value, mL) and percentage (proportion, %);The six‐minute walking distance;Visual Analogue Scales (VAS); INCLUSION CRITERIA: 1. Adult patients aged 18‐80 years; 2. Patients meeting the diagnostic criteria for PPF in the 2022 latest ATS/ERS/JRS/ALAT diagnostic guideline; 3. Patients meeting the syndrome of phlegm heat‐blood stasis obstructing in the lung; 4. Patients with the baseline lung function: FVC% >= 45%, DLCO >= 30% and < 80%; 5. Patients voluntarily participate and sign the informed consent form.