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Abstract Number: 1398

Musculoskeletal and Serologic Findings in an Adult Cystic Fibrosis Clinic Population-a Systematic Review and Collation with Disease Status

Mohamed Elarabi1,2, Katherine Griffin3, Luxme Mahendira1,4, Jenna Sykes3, Erin Norris1,2, Anne Stephenson1,3, Elizabeth Tullis1,3 and Laurence Rubin1,5, 1University of Toronto, Toronto, ON, Canada, 2Rheumatology, Saint Michael’s Hospital, Toronto, ON, Canada, 3Respirology, Saint Michael’s Hospital, Toronto, ON, Canada, 430 Bond Street, Saint Michael’s Hospital, Toronto, ON, Canada, 5Saint Michael’s Hospital, Toronto, ON, Canada

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Respiratory disease

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Session Information

Date: Monday, November 9, 2015

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster Session II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

Cystic fibrosis (CF) is a genetic disorder characterized by exocrine gland production of highly viscous mucus, leading to obstructive and inflammatory changes in the respiratory, digestive and reproductive systems. Musculoskeletal (MSK) burden in CF has historically been addressed in young adults when fewer therapies resulted in a greater infective and inflammatory pulmonary burden. Reported presentations included undifferentiated arthropathy, hypertrophic osteoarthropathy, fluoroquinolone induced arthropathy and osteoporosis. With an increasing lifespan and broader disease spectrum, the impact of CF on the MSK system warrants re-evaluation. Our objectives are to assess MSK burden, the prevalence of serologic and biochemical markers and the relationship of chronic infection to the MSK system in adult CF patients.

Methods:

We conducted a prospective cohort study of all adult patients in St. Michael’s Hospital CF clinic (n>400). To date, 163 subjects were recruited.  Subjects completed MSK specific questionnaires; multidimensional health assessment questionnaire (MDHAQ), WOMAC, Osteoporosis and Fracture History. They were examined for tender and swollen joints. Serology (RF, ANA, anti-cyclic Citrullinated peptide (anti-CCP) antibodies, ESR, CRP and carboxy-terminal collagen crosslinks were obtained. MSK specific data was linked with the Toronto CF database, a comprehensive record of pulmonary and nutritional status, microbiology, and CF genotype. 

Results:

Median age was 32 years; 86 males, 77 females. Median Routine Assessment of Patient Index data 3 (Rapid3) score was 1.33 (normal range: 0-10) and median WOMAC score was 1 (normal range: 0-96). Four subjects had at least 1 swollen joint and 24 had at least 1 tender joint. Seven subjects were RF positive (21-224), 14 were anti-CCP positive (5.1-229.5) and 20 were ANA positive (1.1-6.1). Two subjects had swollen joints and positive antibodies (RF and/or anti-CCP). There was no difference in median age (p=0.55), forced expiratory volume in 1 second (FEV1) % predicted (p=0.40), or proportion of females (p=0.13) between the MSK cohort and overall CF clinic patients. There was no significant association between lung function (FEV1), and antibody status. Stenotrophomonas maltophilia infections were associated with a significantly higher anti-CCP titre (p=0.03). 

Conclusion:

In adult CF, MSK manifestations are mild. However, the prevalence of confirmed seropositive inflammatory arthritis is slightly higher than the general population (1.22%). The relationship between Stenotrophomonas maltophilia and anti-CCP titre warrants further investigation.

Baseline characteristics of the cohort (n-163)

Variable Frequency/Median Proportion/Range

Gender

Female

Male

77

86

47.2%

52.8%

Age at study visit 31.6 18.4-69.1
BMI 22.8 15.9-39

Genotype

Heterozygous dF508

Homozygous dF508

Missing

Other

72

59

8

24

44.2%

36.2%

4.9%

14.70%

FEV1 % predicted 59.7 19.9-118.5
CF related diabetes 54 33.1%
Lung transplant 9 5.5%

Microbiology (n=144) 

Burkholderia cepacia complex

Pseudomonas

Staphylococcus aureus

Stenotrophomonas maltophilia

9

106

63

35

6.2%

73.6%

43.8%

24.3%

Joints (n=97)

Swollen

Tender

4

24

4.1%

24.7%

Rapid 3 score (n=160) 1.33 0-6.86
WOMAC score (n=161) 1 0-68

Rheumatoid factor (n=150)

<20

>20

143

7

95.3%

4.7%

Antinuclear antibodies (n=149) 0.5 0.1-6.1
Anti-cyclic Citrullinated peptide (n=150) 0.4 0-229.5
Carboxy-terminal collagen crosslinks (n=148) 286.8 40-1153
Vitamin D (n=150) 64.5 8-155.8
Fractures (n=158) 66 41.8%

Disclosure: M. Elarabi, None; K. Griffin, None; L. Mahendira, None; J. Sykes, None; E. Norris, None; A. Stephenson, None; E. Tullis, None; L. Rubin, None.

To cite this abstract in AMA style:

Elarabi M, Griffin K, Mahendira L, Sykes J, Norris E, Stephenson A, Tullis E, Rubin L. Musculoskeletal and Serologic Findings in an Adult Cystic Fibrosis Clinic Population-a Systematic Review and Collation with Disease Status [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/musculoskeletal-and-serologic-findings-in-an-adult-cystic-fibrosis-clinic-population-a-systematic-review-and-collation-with-disease-status/. Accessed .
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