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CF Guidelines - Arthropathy
    Arthropathy - Joint pain & stiffness in CF:
        There are a number of different patterns of joint disease suffered by ~10% of individuals with CF, with a median age of
onset of 13-20 years.
        CF arthropathy (CFA) is a specific condition, which is probably immune mediated and related to chronic pulmonary infection and
inflammation. Typically, it presents as an episodic arthritis with pain and swelling, usually of large joints such as knees, ankles
and wrists. It is often accompanied by low-grade fever and there may be erythema nodosum or an erythematous rash or purpura.
Joint x-rays are usually normal. Episodes tend to settle spontaneously after 3-4 days, but can be quite disabling. They tend to
respond well to non-steroidal anti-inflammatory drugs (NSAIDs e.g. Ibuprofen). Intensification of chest therapy may also help
control joint symptoms. Beware renal toxicity when using IV aminoglycosides in those on regular Ibuprofen.
        Hypertrophic pulmonary osteoarthropathy (HPOA) can occur in those with advanced lung disease and may worsen during chest
exacerbations. It affects 2-7% of adults with median onset at age 20 years, and occurs almost exclusively in those with finger
clubbing. It causes joint pain and stiffness typically at wrists, knees and ankles, and is often accompanied by joint effusions,
and features of periostitis. The latter consists of tenderness and pain over the long bones with periosteal elevation on x-ray.
Periosteal changes may be seen on radioisotope bone scan. Treatment is with NSAIDs, but is less effective than for CFA.
Increased chest treatment may help, as may elevation of painful limbs.
        Drug induced arthropathy must be considered. 2 offending agents are fluoroquinolones (eg ciprofloxacin) and minocycline.
Onset of symptoms may occur between three weeks and two months but tend to respond within two weeks once the drug
is stopped. Fluoroquinolones may cause tendon thickening (distinguished from arthritis by MRI). Patients should be warned
of the small risk of Achilles tendon rupture when commenced on fluoroquinolones.
    Other less common patterns include:
      - A small joint arthritis similar to rheumatoid arthritis in which metacarpo-phalangeal joint space narrowing and subluxation may
occur. Diagnosis may be complicated by non-specific positive Rheumatoid factor (IgG > IgM) in those with CF.
      - A sero-negative reactive arthritis can occur after viral contacts, diarrhoea and sexually acquired infection.
      - Despite some individuals having mildly raised urate (historically was associated with some pancreatin preparations), gout has
not been reported in CF.
      - Septic arthritis needs to be excluded (aspiration, gram staining and culture) in those with acute monoarthritis, particularly
those with TIVADs who are at increased risk of bacteraemia.
      - Although non-specific lower back pain is common in the CF as well as general population, sudden onset of severe back pain
may be caused by vertebral collapse in those with osteoporosis.
        Rheumatology referral should be made if diagnosis is in question, or response to NSAIDs poor. Other treatment options include oral Prednisolone, Sulphasalazine and Hydroxychloroquine.
        Dowloadable PDF File - PDF File    
Document approved - December 2011
Document due for review - December 2013
        Acknowledgements: The Peninsula CF team acknowledges the use of guidelines produced by The CF Trust, Manchester, Papworth, Leeds and Brompton CF teams during development of these local Peninsula protocols and guidelines.
Disclaimer: While efforts have been made to ensure that all the information published on this web site is correct, the authors take no responsibility for the accuracy of information, or for harm arising as a consequence of errors contained within this web site. If you have concerns regarding treatment, drugs or doses then consult your local CF consultant.