Study of Subcutaneous Injection of Low-dose rhGM-CSF +/- WLL in PAP. (PAP)
Pulmonary Alveolar Proteinosis
About this trial
This is an interventional treatment trial for Pulmonary Alveolar Proteinosis focused on measuring Pulmonary, Pulmonary Alveolar Proteinosis, PAP
Eligibility Criteria
Inclusion Criteria:
- Diagnosed PAP patients
- Aged 17-80
- Signed informed consent
Exclusion Criteria:
- Secondary PAP
- Received whole lung lavage therapy within 4 weeks before enrollment
- Received previous GM-CSF therapy within 6 months before enrollment
- WBC≥12,000/ul
- fever≥38℃
- Severe edema, severe liver, kidney, lung and cardiovascular disease.
- Pregnant,planning to get pregnant or nursing
- Inability to express the subjective discomfort
- Serious drug allergy history, E.coli preparation or rhGM-CSF serious allergy history
Sites / Locations
- Shanghai Pulmonary HospitalRecruiting
Arms of the Study
Arm 1
Arm 2
Arm 3
Arm 4
Experimental
Experimental
Experimental
Active Comparator
D1
D2
D3
D4
Patients diagnosed with PAP will be received rhGM-CSF 1.25 ug/kg/d subcutaneously for 1 month, before evaluation on the 30th day (±3 day). If the treatment is effective, participants may be entered into low-dose group(D1)(rhGM-CSF administration 1.25 ug/kg/d, qd, sc, for 2 months,then rhGM-CSF administration 1.25 ug/kg/d, qod, sc, for 3 months), when the chest CT absorption≥25% , and /or the PaO2 elevated by 5 mm Hg.
Patients diagnosed with PAP will be received rhGM-CSF 1.25 ug/kg/d subcutaneously for 1 month, before evaluation on the 30th day (±3 day). When the treatment is ineffective, and anti-GM-CSF antibody titers level ≥1:1000,the dose will be increased to 2.5 ug/kg/d. After 2 months, if the clinical response was optimal, that dose is continued for 3 months, and defined as group 2(D2).
Patients diagnosed with PAP will be received rhGM-CSF 1.25 ug/kg/d subcutaneously for 1 month, before evaluation on the 30th day (±3 day). When the treatment is ineffective, and anti-GM-CSF antibody titers level ≥1:1000,the dose will be increased to 2.5 ug/kg/d. After 2 months, if the clinical response was not optimal, the patients will receive whole lung lavage(WLL), who are defined as group 3(D3).
Patients diagnosed with PAP will be received rhGM-CSF 1.25 ug/kg/d subcutaneously for 1 month, before evaluation on the 30th day (±3 day). When the treatment is ineffective, and anti-GM-CSF antibody titers level <1:1000,the patients will receive whole lung lavage(WLL), then give rhGM-CSF administration (1.25 ug/kg/d) for 3 months, which belong to group4(D4).