What do systematic reviews say?

Summary of findings ‐ ivacaftor vs placebo

Summary of findings ‐ ivacaftor probably leads to lower time to first pulmonary exacerbation compared with placebo (moderate certainty).

• Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
  HR 0.46 [0.29, 0.73]
  1 study · 161 participants
  Moderate

Summary of findings ‐ ivacaftor vs placebo

Summary of findings ‐ ivacaftor showed no clear difference in time to first pulmonary exacerbation compared with placebo (moderate certainty).

• Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
  HR 0.93 [0.42, 2.08]
  1 study · 69 participants
  Moderate

Summary of findings ‐ ivacaftor vs placebo

The review found no eligible studies for this question.

• Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis 2019 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor vs placebo

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ dual therapy: tezacaftor plus ivacaftor vs placebo or ivacaftor alone

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  1 study · 506 participants
  Moderate

Summary of findings ‐ triple therapy: VX‐440‐tezacaftor‐ivacaftor vs placebo

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: VX‐152‐tezacaftor‐ivacaftor vs placebo

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor vs control (ivacaftor alone or tezacaftor plus ivacaftor)

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (once daily) vs placebo

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  2 studies · 1108 participants
  Moderate

Summary of findings ‐ dual therapy: lumacaftor plus ivacaftor (twice daily) vs placebo

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: VX‐659‐tezacaftor‐ivacaftor/deutivacaftor vs control

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found

Summary of findings ‐ triple therapy: elexacaftor‐tezacaftor‐ivacaftor/deutivacaftor vs control

The review found no eligible studies for this question.

• Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants… 2023 · Cochrane Headline outcomes only
  no estimate extracted
  Studies not reported
  No studies found