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AAP Grand Rounds 2:70 (1999)
© 1999 American Academy of Pediatrics

RHEUMATOLOGY

Rheumatologic Conditions Associated With Cystic Fibrosis

Source: Merkel PA. Rheumatic disease and cystic fibrosis. Arthritis Rheum. 1999;42(8):1563–1571.

The first 20% of the full text of this article appears below.

There are a number of musculoskeletal disorders that afflict patients with cystic fibrosis (CF). These conditions result from the multiple immunologic and inflammatory abnormalities present in these patients. They include the following:

  1. Cystic Fibrosis Arthropathy (CFA) is characterized by recurrent bouts of painful mono or polyarticular joint swelling. The prevalence reported by various authors ranges from 2.3–8.5% of CF patients, but it is generally recognized that affected patients tend to be older with an average age of diagnosis of 17 years. The etiology is unknown although circulating immune complexes have been invoked.1 Treatment is symptomatic, consisting of NSAIDs in mild cases and glucocorticoids in more severe or persistent ones. The arthritis is usually self-limited, lasting for 5–7 days.
  2. Hypertrophic Osteoarthropathy occurs in about . . . [Full Text of this Article]

Suzanne Bowyer, MD, FAAP
Rheumatology, James Whitcomb Riley Hospital, Indiana University School of Medicine, Indianapolis, IN