Introduction:
Cystic fibrosis is a rare genetic disorder affecting the cells producing sweat, mucus, and digestive juices. The mucus is thickened and accumulates as plugs in the passages and the organs. So, the lungs and the digestive system are affected primarily. In addition, it damages the intestine, pancreas, liver, and kidneys since it is progressive. About one lakh sixty thousand people are estimated to have cystic fibrosis worldwide. It can result in many complications and presents certain effects. Arthritis is considered one among them.
What Is Cystic Fibrosis Related Arthritis?
Cystic fibrosis-related arthritis (CFRA) is a condition developing signs of arthritis in people with cystic fibrosis. However, the data prevails with no formal or actual definition for CFRA. Not every individual with cystic fibrosis develops arthritis. But, a few could develop recurrent joint pain, tenderness, swelling, rigidity, and restricted movements in association with fever, chills, or rashes. Arthritis from cystic fibrosis involves the common joints such as the knee, elbow, shoulders, wrists, and knees.
What Causes Cystic Fibrosis Related Arthritis?
- The clear-cut ideology and cause are still unknown for cystic fibrosis-related arthritis. Nevertheless, certain theories demonstrate the association between cystic fibrosis and arthritis. The following specifies the insight of those theories.
- One theory states that joint pain and its concerns can result from hyperexcitable immune responses developed with chronic forms of lung infection and inflammation, as in cystic fibrosis. Also, the immune system is active for a longer span and so can induce an inflammatory state in the joints when the lung infection is persistent in cystic fibrosis. But, no antigen has been identified.
- Another theoretical view addresses the genetic predisposition of cystic fibrosis to develop cystic fibrosis-related arthritis. The theory stated that inflammatory arthritis could be one of the phenotypes of cystic fibrosis, just like other traits such as clubbing and occasional pancreatic sufficiency. However, further research is needed to elaborate the study.
- CFA can result in gastroenterology complications such as gastrointestinal tract concerns and malabsorption. As a result of this, another theory is that cystic fibrosis-related arthritis is one of those complications. In addition, a range of arthritis conditions is somehow related to gastrointestinal diseases like entheogenic reactive arthritis, intestinal bypass surgery arthritis, and inflammatory bowel syndrome. All these aspects require well, extensive research.
Who Is Commonly Affected?
The symptoms of cystic fibrosis-related arthritis usually begin in children over age ten. The standard age of diagnosis of this condition is between 13 and 17.3 years. This condition suddenly emerges with more prevalent joint pain and flu-like symptoms in children. The incidence of cystic fibrosis-related arthritis might be increasing since people with cystic fibrosis have a persistent illness. In addition, genetic predisposition also contributes to CFRA. Clinical researchers are studying the potential attributing factors.
What Are the Types of Cystic Fibrosis Related Arthritis?
Cystic fibrosis-related arthritis is of two types, namely.
- Cystic fibrosis-related arthropathy (CFA).
- Hypertrophy pulmonary osteoarthropathy (HPO).
What Are the Signs and Symptoms of Cystic Fibrosis Related Arthritis?
The signs, prevalence, and clinical features depend upon the type of CFRA. The symptoms, in accordance with their types, are discussed below.
Cystic Fibrosis-Related Arthropathy -
CFA is the most common form of joint illness in individuals with cystic fibrosis, with an incidence of about 2 to 8.5 percent of cystic fibrosis. Also, CFA is common among cystic fibrosis patients with severe degrees of lung disease, worsening with a lung infection. Joint pain is the predominant feature, which develops in about 12 to 24 hours. Flares do occur, lasting less than one week. But, the symptoms are not usually seen between the flares. One or multiple joints are involved. The following are the common symptoms of CFA.
- Joint pain occurs in episodes.
- Tenderness.
- Fever.
- Skin rashes.
- Swelling.
- Limitation of movements.
Hypertrophy Pulmonary Osteoarthropathy -
Hypertrophy pulmonary osteoarthropathy is a joint condition that can develop in a range of lung diseases, such as cystic fibrosis. So, HPO occurs with an incidence of two to seven percent in people with cystic fibrosis. The joint pain and symptoms usually develop in both the joints, such as the involvement of both knees or both wrists. In addition, cold weather conditions aggravate the symptoms of hypertrophic pulmonary osteoarthropathy. So, the typical signs include the following.
- Joint pain in episodes.
- Dull degrees of bone pain.
- Swelling and tenderness were felt at the bone termination.
- Digital clubbing of finger clubbing.
How Is Cystic Fibrosis Related Arthritis Diagnosed?
Healthcare professionals employ a range of means to diagnose the condition of arthritis, including a physical examination. The following diagnostic aids are commonly prescribed in the case of cystic fibrosis-related arthritis.
- Complete Blood Count (CBC): A blood test is commonly recommended to measure the blood cells by means of qualities and quantities.
- Acute Phase Reactant Levels (APR): Acute phase reactant is an inflammation marker that rises in cases of injury or infection. So, their levels help assess the disease.
- Fluid Analysis: Synovial fluid is the fluid of the joint, which is examined for its color, consistency, and the presence of microbes causing infection.
- X- Rays: Bone X-rays are done to examine the bone uncertainties.
How Is Cystic Fibrosis Related Arthritis Treated?
The intervention is quite intricate since cystic fibrosis treatment involves a range of drugs. The treatment options for CFRT depend upon the condition's severity and the underlying cystic fibrosis. However, healthcare professionals commonly prescribe the following medications.
Non-steroidal Anti-inflammatory Drugs (NSAIDs) - They are given as initial therapy since they are meant to reduce fever, signs of inflammation, and pain. These drugs perform their action like steroids, though they do not belong to steroids.
The following medications are given in severe and chronic forms. They act by weakening the immune system. As a result, inflammation and the deterioration of the damage are diminished.
- Corticosteroids - Prednisolone and Methylprednisolone.
- Disease-Modifying Antirheumatic Drugs (DMARDs) - This includes Hydroxychloroquine and Methotrexate.
Conclusion:
Cystic fibrosis-related arthritis is considered a complication of cystic fibrosis. Therefore, early recognition and intervention are the keys to the alleviation of concerning joint issues. Also, one should utilize the intervention and equipment of this new generation that made the treatment effective and advancing. So, the prognosis and outcome are favorable with appropriate medication, bed rests, and palliative care.
