P45 Treat the arthritis, not just the chest

Abstract

Abstract Background Very little literature exists in supporting the assessment and management of cystic fibrosis related arthritis (CFA) particularly in paediatric and adolescent cases. Methods A 12 year-old male with cystic fibrosis (CF) was admitted via A&E with severe joint pain that was thought to be associated with a respiratory infection and was treated accordingly. Repeated exacerbations of joint disease occurred every 1-2 months, lasting approximately 1 week with significant joint pain, stiffness, and reduced function. The patient did not feel his exacerbations of CFA were related to his respiratory symptoms, admitting he under reported arthritis symptoms to avoid being admitted for IV antibiotics. Initial rheumatology review resulted in treatment with IV antibiotics and piroxicam. Flares continued, prompting his CF physiotherapist and multidisciplinary team to seek further specialist review for his now longstanding symptoms. He scored 16 out of 50 on the McGill Pain Questionnaire (MPQ) with a visual analogue score (VAS) of 46 out of 100. At 16 years old he was seen by an MSK physiotherapy and rheumatology team working within a wider CF team. He had a 4 year history of symmetrical joint pain affecting knees, wrists and hands, on a background of bronchiectasis with previous non-tuberculous mycobacteria infection, cystic fibrosis related diabetes, and poor nutrition. He reported some episodes being so bad he was unable to get out of bed, and struggling with the stairs at college. He had 6 swollen and 8 tender joints including wrists, MCPs and knees. Ultrasound imaging of the wrists and hands showed confirmed active synovitis with grade 3 greyscale changes and grade 2 power Doppler. Hydroxychloroquine was commenced with naproxen as required, and wrist splints were provided. Results At follow-up he reported significant symptomatic improvement with occasional minor stiffness in wrists and knees which responded quickly to naproxen and was no longer limiting his activities. Repeat ultrasound confirmed improvement with no active power Doppler, but some ongoing grade 1 greyscale changes and a small wrist effusion. He has returned to previous function with an MPQ score of 0, and VAS of 10 out of 100. He reported improved quality of life due to reduced pain and fewer, shorter, and more manageable exacerbations. Conclusion Specialist review and commencement on disease modifying anti-rheumatic medication helped to manage CFA in this case. All cases of suspected CFA should be referred for specialist rheumatology opinion. CF physiotherapists are in an excellent position to identify cases and empowering the multidisciplinary team has been vital in this case. Further trials are required to investigate optimal management of CFA, but it seems likely that directly addressing the arthritis with disease modifying therapy may play an important role. Conflicts of Interest The authors declare no conflicts of interest.