Understanding Cystic Fibrosis Associated Arthritis

As cystic fibrosis patients are surviving longer into their adult years and “middle age,” some may find themselves experiencing episodes of arthritis. Although cystic fibrosis associated arthritis has been observed to affect only 5-10% of the cystic fibrosis population, there’s no denying it happens.

Aches and pains of the joints and particularly large joints such as knees and ankles can cause extreme pain. During an episode, these sites may feel warm to the touch as well as appear swollen and red. Flu-like symptoms such as fatigue and fever may also be present. The interesting thing about cystic fibrosis related arthritis is that flare-ups are rather intermittent; patients typically experience no other symptoms in between episodes.

Defining CF Associated Arthritis

Cystic fibrosis associated arthritis (CFAA)is described as having episodes that last less than a week, involve the large joints (ankle, knee, wrist, shoulder) and responds to treatment with anti-inflammatory medication.

Non-arthritic Joint Pain

Since CF patients are often malnourished and have osteopenia or osteoporosis, aches and pains may not necessarily be from arthritis, but rather from a condition called hypertrophic pulmonary osteopathy (HPOA.) HPOA is not specifically tied to CF, but is observed in other lung diseases. HPOA is believed to be the culprit behind finger clubbing.

Diagnosis

Diagnosis cystic fibrosis associated arthritis can only be diagnosed once other likely possibilities for joint pain have been ruled out. A doctor will ask questions about when the symptoms appeared, whether the pain was the same on both sides of the body, and whether a rash or fever was present. The doctor also needs to know approximately how long the episodes of pain last. It is important for the patient to distinguish whether the pain is sharp or dull, throbbing or steady.

Treatment

As with most inflammatory conditions, non-steroidal anti-inflammatory (NSAID) medications are the first course of action. Ibuprofen in prescription strength doses (i.e., 600mg or greater) is usually enough to gain control over an episode cystic fibrosis associated arthritis. There is a small percentage of people with CF-associated arthritis that are resistant to NSAID treatment, but do respond to corticosteroids such as prednisone. An anti-inflammatory diet may also be helpful to lessen the severity of an episode and prevent further ones.