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Cystic Fibrosis-Related Arthritis

Arthritis is known for its joint pain and swelling. Some people may think of arthritis as a condition for older people. However, people with cystic fibrosis (CF) may develop a type of arthritis too. Cystic fibrosis-related arthritis, sometimes referred to as CFRA, occurs in 5 percent to 10 percent of people with CF.1

CF-related arthritis symptoms and diagnosis

Doctors typically diagnose cystic fibrosis-related arthritis if the joint pain and swelling lasts less than a week per episode. Plus, the pain and swelling will appear in large joints such as the knees and elbows.2 When CF-related arthritis episodes occur, they can be quite painful and may require bed rest.

A CF-related arthritis flare often begins with a dull pain that grows over 12 to 24 hours into more severe joint pain. Sometimes, fever or a skin rash also appears with a flare.2,3

If the pain or joint swelling begins to last longer than a few days at a time and spread to smaller joints such as in the fingers, it may not be CF-related arthritis but an autoimmune disorder or drug side effect.2

In most cases, the arthritis flares come and go every so often. Symptoms generally disappear completely between episodes. X-rays tend to show no joint damage. Permanent joint damage is rare in CF-related arthritis.2

Who is affected?

Episodes of cystic fibrosis-related arthritis normally begin in those over age 10. The average age at diagnosis ranges from 13 to 17.3. When CF-related arthritis appears in young people it often crops up suddenly, with widespread joint pain and flu-like symptoms.2

Because people with CF are living longer, cystic fibrosis-related arthritis may be increasing. This increase could also be due to more people reporting the condition. There may also be a genetic predisposition for CF-related arthritis. Researchers are studying both possibilities.3

Types of cystic fibrosis-related arthritis

There are 2 types of CF-related arthritis: cystic fibrosis‐related arthropathy (CFA) and hypertrophic pulmonary osteoarthropathy (HPO).2

CFA is the most common form of joint disease in people with cystic fibrosis.

  • Appears in 2 percent to 8.5 percent of people with cystic fibrosis
  • Joint pains tend to develop over 12 to 24 hours
  • Flares last less than 1 week
  • Symptoms may disappear completely between flares
  • One or more joints may be affected
  • Fever and skin rashes may occur
  • Often seen in CF patients with more severe lung disease
  • Can worsen during a lung infection3

HPO occurs in a variety of lung diseases, including cystic fibrosis.

  • Appears in 2 percent to 7 percent of people with CF
  • May cause finger clubbing
  • May cause swelling and tenderness of the ends of the long bones
  • Joint symptoms tend to appear on both sides of the body, for example both wrists, both knees or both ankles
  • Symptoms get worse in cold weather3

Treatments for CF-related arthritis

Nonsteroidal anti-inflammatory drugs (NSAIDs) are usually the first treatment for CF-related arthritis. Over-the-counter NSAIDs include aspirin and ibuprofen. Stronger NSAIDs may be prescribed by your doctor. If NSAIDs fail to bring relief, your physician may suggest steroids.2

Resting the affected joint and applying a warm compress may help reduce pain, stiffness, and swelling.2

It is important to remember that patients with cystic fibrosis are susceptible to other joint and bone diseases such as osteoporosis. In addition, many people with cystic fibrosis take a host of drugs that may cause a reaction such as joint pain and swelling. The acne drug minocycline, for instance, can cause lupus-like symptoms. Your doctor may want to rule out these other causes with more tests.2

  1. Cystic Fibrosis Trust. Bone disease and cystic fibrosis. Available at: https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/how-does-cystic-fibrosis-affect-the-body/symptoms-of-cystic-fibrosis/bone-disease. Accessed 11/22/19.
  2. Cystic Fibrosis Medicine. Joint pain and disease in cystic fibrosis - A practical approach. Available at: http://cfmedicine.com/htmldocs/CFText/arthritis.htm. Accessed 11/22/19.
  3. Thornton J, Rangaraj S. Anti‐inflammatory drugs and analgesics for managing symptoms in people with cystic fibrosis‐related arthritis. Cochrane Database of Systematic Reviews 2016, Issue 1. Art. No.: CD006838. doi: 10.1002/14651858.CD006838.pub4.