Cystic fibrosis (CF) is a serious genetic disorder that has effects on many organs (e.g. lung, pancreas). It commonly results in reduced bone mineral density (BMD), known as osteoporosis, which increases the susceptibility to fractures. The acute and chronic effects of fractures (e.g. rib and vertebral) may contribute to worsening of lung disease. Bisphosphonates are drugs that increase bone mineral density (BMD) by inhibiting bone resorption. They are used to treat osteoporosis caused by menopause or the use of corticosteroid drugs.
The evidence available was limited to four trials assessing participants without lung transplants (total of 111 adult participants) and one trial with 34 adult participants who had undergone lung transplantation. Bisphosphonates consistently increased BMD at the lumbar spine and hip regions. The rates of fractures (vertebral and non-vertebral) or deaths were not reduced by bisphosphonate therapy. However, this may be related to the small numbers of participants involved and the short duration of the trials. Severe bone pain and flu-like symptoms were commonly associated with intravenous bisphosphonates, especially in people not using corticosteroids. More research is needed to assess the effect of pre-treatment with corticosteroids. Additional trials are needed to determine if bone pain is more common or severe (or both) with the more potent zoledronate and if corticosteroids ameliorate or prevent these adverse events. Trials in larger populations are needed to determine effects on fracture rate and survival.