PAC: Phenotyping the Arthropathy of CF
Research type
Research Study
Full title
Identification of a Cystic Fibrosis-associated Arthropathy Phentoype
IRAS ID
231937
Contact name
Pippa Watson
Contact email
Sponsor organisation
University Hospitals South Manchester
ISRCTN Number
ISRCTN34533467
Duration of Study in the UK
1 years, 0 months, 2 days
Research summary
Cystic Fibrosis (CF) is associated with a variety of musculoskeletal conditions, and a form of inflammatory arthritis specific to cystic fibrosis was first described in the late 1970s.
With input from Rheumatologists, Musculoskeletal Physiotherapists working with CF and CF consultants, we have designed a project to attempt to provide a good working definition and phenotype of the condition.
We will recruit patients with CF who have inflammatory-sounding joint symptoms into our observational cohort study. A baseline visit will enable us to gain background information, collect blood tests and complete an ultrasound of joints when they are not flaring. We then will see patients as and when their joint symptoms flare in order to characterise the patterns of events and to gain clinical examination data, ultrasound imaging and bloods to gain a better picture of the disease. A telephone call at the end of the study will be used to confirm patterns of disease flare over the study period.
25 patients with CF without joint disease will healthy control participants. These patients will have 1 visit combined with a routine clinic visit to include blood tests and ultrasound along with a clinical examination.Lay Results Summary
The first part of this project involved creating a questionnaire for people with CF, to identify those who might have inflammation in their joints (inflammatory arthritis). This showed that lots of people with CF have pain in their joints even though they may not have inflammation. About half of the 400 people who took part told us that joint or back pain had affected their activities of daily living in the last year. Most of these people do not have inflammatory arthritis; other possibilities need to be considered when thinking about joint pain in people with CF. This means that good assessment is vital. Other possible causes include sports injuries, low vitamin D, gout, fracture, and poor muscle function amongst others. In the second part of the study, we used clinical examination, blood tests and ultrasound scans to get a better picture of inflammatory arthritis patients with CF. Participants told us when their symptoms flared up so that we saw them when things are worst. Some of the treatments we use in other forms of inflammatory arthritis (like rheumatoid or psoriatic arthritis) interact with the way the immune system functions, which can be a worrying idea for anyone, but especially in CF where there are often chronic infections, and infective exacerbations are a big concern. For the data we have on people with CF treated with these medicines, no patients stopped their immunosuppressive medicine as a result of their chest worsening. This fits with some of the case reports available, and a study looking at CF lung disease post liver transplant (requiring immunosuppression for the liver transplant). Better assessment pathways and clearer definitions aid faster diagnosis and consideration of treatment options. It means that people who have CF an unrelated form of inflammatory arthritis are less likely to have it attributed to their CF; treatment can therefore be started more quickly. For people with arthritis related to their CF, we can plan trials into treatment with better understanding of how to identify it. Painful joints make everyday things much more difficult and it is really important that we address this.REC name
North West - Greater Manchester West Research Ethics Committee
REC reference
17/NW/0575
Date of REC Opinion
3 Nov 2017
REC opinion
Further Information Favourable Opinion