The Musculoskeletal System

CF associated arthralgia (and rarely arthritis) is the commonest form of joint pain in CF and mostly affects the large joints (knee, ankle, wrist, elbow and shoulder). Episodes usually last less than a week but can be quite disabling. Rarely the attacks are associated with high swinging fevers, skin rashes, nodules and purpura [17].

Hypertrophic pulmonary osteoarthropathy (HPOA) has an insidious onset of pain, which is generally mild at the beginning. Clinically, findings range from a minimally swollen joint to tender, warm and swollen joints resembling those seen in rheumatoid arthritis. Symptoms are often worse in cold weather. It should be noted that HPOA also occurs in a variety of other lung diseases [17].

Both these forms of arthropathy can be associated with acute respiratory exacerbations.

Osteoporosis is twice as common in patients with CF and may present with sudden onset of severe back pain from vertebral collapse. A full neurological examination is necessary to exclude any resulting nerve compression. Patients may also present with ‘cough fractures’ of their ribs. Osteoporotic fractures of other areas are also likely with lesser degrees of trauma, and a high index of suspicion should be maintained when evaluating a seemingly innocuous mechanism of injury.

Patients with CF are also susceptible to joint disease unrelated to their underlying illness, and a careful history and examination is required to differentiate these.

Learning Bite

Have a high index of suspicion for fractures in patients with CF presenting with seemingly innocuous mechanisms of injury.