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Phase 2 N=138 Randomized Quadruple-blind Treatment

Efficacy and Safety of Inhaled Molgramostim (rhGM-CSF) in Autoimmune Pulmonary Alveolar Proteinosis

Autoimmune Pulmonary Alveolar Proteinosis

Enrolled (actual)
138
Serious AEs
13.8%
Results posted
Apr 2022
Primary outcome: Primary: Absolute Change From Baseline of Alveolar-arterial Oxygen Concentration (A-a(DO2)) After 24 Weeks of Treatment — 28.6; 29.3; 32.0 mmHg — p=0.1688

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Molgramostim (Drug); Placebo (Drug); PARI eFlow nebulizer system (Device)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Savara Inc.
Primary completion
Apr 2019

Outcome Measures

OutcomeResultp-value
PRIMARY
Absolute Change From Baseline of Alveolar-arterial Oxygen Concentration (A-a(DO2)) After 24 Weeks of Treatment
28.6; 29.3; 32.0 0.1688
SECONDARY
Change From Baseline in 6-minute Walking Distance (6MWD) After 24 Weeks of Treatment
39.6; 11.3; 6.0 0.3159
SECONDARY
Change From Baseline in St. George's Respiratory Questionnaire (SGRQ) Total Score After 24 Weeks of Treatment
-12.3; -12.0; -4.7 0.0103 sig
SECONDARY
Number of Whole Lung Lavage During 24 Weeks of Treatment
9; 7; 17 0.1918
SECONDARY
Number of Adverse Events (AEs) During 24 Weeks of Treatment
215; 191; 192
SECONDARY
Number of Serious Adverse Events (SAEs) During 24 Weeks of Treatment
13; 5; 16
SECONDARY
Number of Adverse Drug Reactions (ADRs) During 24 Weeks of Treatment
53; 27; 33
SECONDARY
Number of Severe AEs During 24 Weeks of Treatment
28; 11; 38
SECONDARY
Number of Participants With at Least 1 AE Leading to Treatment Discontinuation During 24 Weeks of Treatment
2; 1; 1

Summary

This study evaluates inhaled molgramostim (recombinant human granulocyte macrophage-colony stimulating factor [rhGM-CSF]) in the treatment of autoimmune pulmonary alveolar proteinosis (aPAP) patients. A third of the patients will receive inhaled molgramostim once daily for 24 weeks, a third will receive inhaled molgramostim intermittently (7 days on, 7 days off) for 24 weeks and a third will receive inhaled matching placebo for 24 weeks.

Eligibility Criteria

Inclusion Criteria

  • aPAP diagnosed by computed tomography, or by biopsy, or by Broncho Alveolar Lavage (BAL), and by increased GM-CSF autoantibodies in serum.
  • Stable or progressive aPAP during a minimum period of 2 months prior to the Baseline visit.
  • Arterial oxygen tension (PaO2) 4 percentage points on the 6MWT
  • An alveolar-arterial oxygen difference [(A-a)DO2] of minimum 25 mmHg/3.33 kPa
  • Female or male ≥18 years of age
  • Females who have been post-menopausal for >1 year or females of childbearing potential after a confirmed menstrual period using a highly efficient method of contraception (i.e. a method with <1% failure rate such as combined hormonal contraception, progesterone-only hormonal contraception, intrauterine device, intrauterine hormone-releasing system, bilateral tubal occlusion, vasectomised partner, sexual abstinence), during and until 30 days after last dose of double-blind trial treatment. Females of childbearing potential must have a negative serum pregnancy test at Screening (Visit 1) and a negative urine pregnancy test at dosing at Baseline (Visit 2) and must not be lactating
  • Males agreeing to use condoms during and until 30 days after last dose of double-blind medication, or males having a female partner who is using adequate contraception as described above
  • Willing and able to provide signed informed consent
  • Willing and able to comply with scheduled visits, treatment plan, laboratory tests, and other trial procedures specified in the protocol as judged by the investigator

Exclusion Criteria

  • Diagnosis of hereditary or secondary PAP
  • WLL within 1 month of Baseline
  • Treatment with GM-CSF within 3 months of Baseline
  • Treatment with rituximab within 6 months of Baseline
  • Treatment with plasmapheresis within 3 months of Baseline
  • Treatment with any investigational medicinal product within 4 weeks of Screening
  • Concomitant use of sputum modifying drugs such as carbocysteine or ambroxol
  • History of allergic reactions to GM-CSF
  • Connective tissue disease, inflammatory bowel disease or other autoimmune disorder requiring treatment associated with significant immunosuppression, e.g. more than 10 mg/day systemic prednisolone
  • Previous experience of severe and unexplained side-effects during aerosol delivery of any kind of medicinal product
  • History of, or present, myeloproliferative disease or leukaemia
  • Known active infection (viral, bacterial, fungal or mycobacterial)
  • Apparent pre-existing concurrent pulmonary fibrosis
  • Any other serious medical condition which in the opinion of the investigator would make the participant unsuitable for the trial
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02702180). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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