Evaluation of pulmonary hypertrophic osteoarthropathy in cystic fibrosis. A comprehensive study.

A questionnaire survey of 375 patients with cystic fibrosis was performed to seek evidence of pulmonary hypertrophic osteoarthropathy (PHOA). Three hundred responses were obtained. Forty-five patients (15%) who described long-bone or joint pain met clinical criteria for the diagnosis of PHOA, 25 of whom had roentgenographic evidence of periostitis. Compared with an age- and sex-matched control group (group 3), the patients with cystic fibrosis and PHOA as well as roentgenographic evidence of periostitis (group 1) had worse Shwachman scores and pulmonary function and a significantly increased mortality rate (36%). The patients with PHOA but no roentgenographic evidence of periostitis (group 2) had Shwachman scores intermediate between groups 1 and 3. We believe PHOA is more common than previously suspected. Its incidence appears related to severity of disease.