CF patients are prone to five different types of arthritis: association of Cystic Fibrosis with other known causes of joint disease, as gout (Horsley A, 2012), adverse effect of medications (above all ciprofloxacin), osteoarticular infection, hypertrophic pulmonary osteo-arthropathy (HPO) (Merkel PA, 1999) and arthritis considered unique to CF (cystic fibrosis-related arthropathy=CFA) (Turner MA, 1997). HPO, in which long bones alteration and clubbing are present with joint symptoms, usually starts at a mean age of 20 years, is mostly associated with lung disease deterioration and, as a rule, joint symptoms become more severe during respiratory exacerbations. CFA occurs in younger age (mean age of onset is 13 years), is not associated with a severe lung condition, neither to respiratory exacerbation; it may develop over 12-24 hours, with fever and skin manifestations and may spontaneously disappear over one week, even if it may become persistent and disabling. Although arthritis is a relatively uncommon complication in CF (2%-7% for HPO and 2%-8% for CFA), it may have a severe impact of patients quality of life. So, the best management is important to define. Radiological changes are typical in HPO but they are often absent in CFA, even if a recent study (Fitch G, 2016) suggested that imaging (ultrasound and magnetic resonance) is able to identify inflammatory joint pathology in a considerable proportion of cases.
Symptomatic drugs efficacy (joint involvement and function, quality of life, laboratory inflammation markers must be considered as measurable outcomes) and safety (above all about gastrointestinal effects).
Disease-modifying anti-rheumatic drugs efficacy and safety for CFA.
What is known
One Cochrane review (Thornton J, 2012) related to disease-modifying anti-rheumatic drugs (DMARD) efficacy and safety for CFA is available.
One Cochrane review (Thornton J, 2016) evaluated anti-inflammatory drugs and analgesics for managing symptoms in arthritis in Cystic Fibrosis.
Both Cocrhane reviews did not find any RCT eligible to draw any conclusion about this topic.
Although arthritis has an important impact on the quality of life and it may impair physical training in CF patients only anecdotic experiences are available in the literature to study the best management of this complication.
Recently a pilot multicenter randomized controlled trial, that evaluated the impact of osteopathic manipulative treatment (OMT) on pain in adults with CF, demonstrated that OMT was feasible, even if no significant difference between the treatment and the control groups, in the decrease of chest/back pain, cervical pain, headache and quality of life was found.
Treatment strategy, safety and efficacy both to manage symptoms and to modify arthritis development. In particular it is important to determine if DMRDs treatment should be started at the earlier stages of the disease to prevent progressive damage as recommended in arthritis not associated with CF.
No RCT are ongoing about this issue.