A prospective cohort study was used to observe the efficacy and safety of different immunosuppressive agents with/wo pirfenidone on CTD-ILD patients in Qilu Hospital of Shandong University for 36 months.The main research endpoints are lung function, patient dyspnea score, 6-minute walking distance, imaging indicators, primary disease activity, adverse reactions, etc.
This study will enroll 200 cases of connective tissue disease-related interstitial lung disease (CTD-ILD) patients in China, including inflammatory myopathy (IIM) patients, rheumatoid arthritis (RA) patients, systemic sclerosis (SSc) patients, Sjogren's syndrome (SS) patients and other connective tissue disease patients.According to the patient's condition,the participants plan to be treated with different immunosuppressive agents with/wo pirfenidone .Participants can choose to continue the study up to 36 months.The efficacy and safety of the treatment in CTD-ILD patients will be evaluated with lung function, quality of life / cardiopulmonary function assessment and other disease activity indices.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
200
CTD-ILD patients treated with pirfenidone up to the maximum tolerable dose
CTD-ILD patients treated with DMARDs according to the condition of the disease
Qilu Hospital
Jinan, Shandong, China
Change in FVC
Change in forced vital capacity(FVC) from 12 months to baseline
Time frame: 12 months
Change in DLCo
Change in carbon monoxide diffusing capacity (DLCo) from 12 months to baseline
Time frame: 12 months
Changes in FVC
Changes from baseline in forced vital capacity(FVC)
Time frame: 6 months,24 months,36 months
Changes in DLCo
Changes from baseline in carbon monoxide diffusing capacity (DLCo)
Time frame: 6 months,24 months,36 months
Changes from baseline in Dyspnea score
Changes from baseline in Dyspnea score. Dyspnea score:activity tolerance of patients with dyspnea in daily life: 0: normal, 1: dyspnea when climbing the fifth floor, 2: dyspnea when climbing the third floor, 3: dyspnea when climbing the second floor, 4: Difficulty in breathing when walking on the flat ground 100-500m, 5: Difficulty in breathing when walking on flat ground for 50-100m, 6: Difficulty in breathing when walking on flat ground for 15-50m, 7: Difficulty in breathing when walking on flat ground 5-15m, 8: Difficulty in breathing during daily dressing, 9: Difficulty in breathing when eating, 10: Difficulty in breathing when resting.The higher the score, the more severe the disease.
Time frame: 6 months,12 months, 24 months,36 months
Imaging changes
Changes from baseline in high-resolution computed tomography (HRCT)
Time frame: 6 months,12 months, 24 months,36 months
Changes from baseline in 6 minutes walking distance
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Changes from baseline in 6 minutes walking distance
Time frame: 6 months,12 months, 24 months,36 months
Changes from baseline in C-reactive protein(CRP)
Changes from baseline in C-reactive protein(CRP)
Time frame: 6 months,12 months, 24 months,36 months
Changes from baseline in Erythrocyte Sedimentation Rate(ESR)
Changes from baseline in Erythrocyte Sedimentation Rate(ESR)
Time frame: 6 months,12 months, 24 months,36 months
Changes from baseline in VAS score
The visual analogue scale (VAS) is used for pain assessment. The basic method is to use a moving ruler with a length of about 10cm. One side is marked with 10 scales. The two ends are respectively "0" and "10" points. A point of 0 means no pain, and a point of 10 means the most unbearable. Severe pain. It can reflect the degree of activity of the primary disease.
Time frame: 6 months,12 months, 24 months,36 months
Number of participants with "adverse events (AEs)"
An AE is any untoward medical occurrence in a subject, temporally associated with the use of a medicinal product, whether or not considered related to the medicinal product. An AE can therefore be any unfavorable and unintended sign (including an abnormal laboratory finding), symptom, or disease (new or exacerbated) temporally associated with the use of a medicinal product.
Time frame: Up to month 36