Musculoskeletal manifestations and rheumatic symptoms in patients with cystic fibrosis (CF) no observations of CF-specific arthropathy.
OBJECTIVE
To determine the character and frequency of musculoskeletal manifestations and rheumatic symptoms in patients with cystic fibrosis (CF).


METHODS
Rheumatic symptoms and signs of 70 patients with CF (age 6 to 61 yrs) were determined by interview and clinical assessment. Age and sex-matched healthy volunteers served as a control group. In CF patients, laboratory measures and bone mineral density (BMD) were investigated. The data were correlated with the CF phenotype [Shwachman Score (ShS), Chrispin-Norman Score (ChNS), and pulmonary function tests (PFT)].


RESULTS
The prevalence of joint pain in the CF patients was 12.9%, with a mean duration of 7 days. Swollen joints were found in 4 patients. None fulfilled the criteria for rheumatoid arthritis or connective tissue disease. Adult CF patients complained more often about noninflammatory back pain and myalgia, and demonstrated reduced spine mobility and impaired everyday life functions compared with the controls. Symptomatic CF patients had elevated erythrocyte sedimentation rate and C-reactive protein levels and performed worse on the ShS, ChNS, and PFT than asymptomatic patients. Antibodies against exotoxin A of Pseudomonas aeruginosa and recombinant Aspergillus fumigatus allergen f4 were found more frequently in CF patients with arthralgia. BMD was decreased in adult patients with more severe CF.


CONCLUSIONS
In CF patients, the prevalence of rheumatic symptoms increases with age and CF severity. Our data suggest an association of infections with P. aeruginosa and A. fumigatus with the occurrence of rheumatic symptoms. However, no association of CF with definite inflammatory joint or connective tissue diseases was observed, and no CF-specific pattern of musculoskeletal symptoms was seen.
Koch
Ann-Kristin
AK
Department of Internal Medicine I, University Hospital of Martin-Luther University Halle-Wittenberg, Halle Saale, Germany. ann-kristin.koch@medizin.uni-halle.de
Brömme
Sabine
S
Wollschläger
Bettina
B
Horneff
Gerd
G
Keyszer
Gernot
G
D000293:Adolescent; D000328:Adult; D001228:Aspergillosis / Q000150:complications / Q000276:immunology; D001232:Aspergillus fumigatus / Q000276:immunology; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007407:Interviews as Topic; D008297:Male; D008875:Middle Aged; D011552:Pseudomonas Infections / Q000150:complications / Q000276:immunology; D011550:Pseudomonas aeruginosa / Q000276:immunology; D012216:Rheumatic Diseases / Q000150:complications* / Q000503:physiopathology; D055815:Young Adult