Musculoskeletal involvement in cystic fibrosis

The purpose of this study was to review the published evidence of the effects of cystic fibrosis (CF) on the musculoskeletal system, and their implications on the quality of life of these patients. With increasing longevity of CF patients, musculoskeletal manifestations have become important. Decreased bone mineral density (BMD) and bone mineral content (BMC) are common at all ages in CF patients, and its cause is probably multifactorial. Risk factors contributing to decreased BMC include age of the patient, imbalance between bone accretion and degradation, body mass, nutrition, activity, exposure to glucocorticoid therapy, and gonadal dysfunction. BMC correlates strongly to fat-free mass in CF patients. Lung transplant, while increasing life span, can worsen osteoporosis. Twenty-nine percent of patients are below the 10th percentile for height. Fracture rates are approximately twofold greater in females aged 16 to 32 years and males aged 25 to 34 years. Female patients aged 6 to 16 have a higher than normal fracture rate and a higher rate than their male counterparts. Spinal consequences of osteoporosis include excessive kyphosis in up to 62% of patients. Back pain was noted in 94% of patients. Arthritis in CF, although less common, can be very incapacitating, and it is mainly of three types: CF arthritis, hypertrophic osteoarthropathy, and arthritis due to co-existent conditions and drug reactions. The treatment of arthritis is mainly symptomatic in the majority of cases.