Palindromic Rheumatism (PR)

Symptoms

• Recurrent Joint Pain and Swelling: Typically affects multiple joints, including the wrists, knees, elbows, and hands. The pain usually comes on suddenly and is associated with joint redness, warmth, and swelling.
• Episodes of Inflammation: Flare-ups can last anywhere from a few hours to several days, followed by complete resolution of symptoms.
• Migratory Pattern: The affected joints often change from one episode to another, with different joints being involved in each flare.
• No Lasting Joint Damage: Unlike RA, joint damage is typically not seen in PR, although frequent flare-ups can occasionally lead to some wear and tear in the joints over time.
• Systemic Symptoms: Some people may experience fatigue, mild fever, or general malaise during flare-ups, but these are not as common or severe as in other inflammatory diseases.
• No Permanent Deformity: The condition is usually non-deforming, and joint mobility returns to normal between flares.

How is the Diagnosis Done?

• Medical History and Physical Exam: The doctor will evaluate symptoms, particularly the pattern of recurring joint pain and swelling with complete resolution in between. The migratory nature of the symptoms is an important clue.
• Blood Tests:
  • Rheumatoid Factor (RF) and Anti-Citrullinated Protein Antibody (ACPA): These are typically negative in PR, unlike in rheumatoid arthritis where they are usually positive.
  • Inflammatory Markers: C-Reactive Protein (CRP) and Erythrocyte Sedimentation Rate (ESR) are often elevated during flare-ups, indicating inflammation.
  • Anti-Nuclear Antibodies (ANA): Can be positive in some cases, but this is not specific to PR and can be seen in various autoimmune diseases.
  • Complete Blood Count (CBC): To check for signs of anemia or other systemic issues.
• Imaging: X-rays are typically normal in PR between flare-ups. However, joint erosion or damage may be seen if the condition progresses to rheumatoid arthritis.
• Exclusion of Other Conditions: Since palindromic rheumatism is often diagnosed by exclusion, other conditions that could cause similar symptoms (e.g., RA, systemic lupus erythematosus, gout, or infectious arthritis) need to be ruled out.
• Clinical Criteria: The ACR/EULAR classification criteria for inflammatory arthritis can sometimes be helpful in distinguishing PR from RA or other conditions, but they are not definitive.

Why is Early Diagnosis Important?

• Preventing Progression: Early diagnosis helps identify those at risk of progressing to rheumatoid arthritis (RA) or another form of chronic arthritis. Timely treatment can reduce the risk of joint damage and chronic disability.
• Symptom Control: Early intervention can help manage flare-ups, reduce pain, and improve the quality of life by preventing frequent episodes of inflammation.
• Differentiating from Other Conditions: Proper diagnosis ensures that other conditions with similar symptoms (e.g., gout, reactive arthritis) are ruled out, ensuring appropriate treatment.
• Treatment Planning: Identifying the disease early allows for better planning of treatment strategies to control inflammation and minimize flare-ups.
• Prognosis: Early recognition of PR provides an opportunity to monitor for potential progression to RA, allowing for proactive management and better long-term outcomes.

Treatment

• Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): These are commonly used to relieve pain, reduce inflammation, and manage flare-ups. Given on SOS basis.
• Corticosteroids: Oral prednisone or intra-articular steroid injections are effective for rapidly controlling inflammation during flare-ups.
• Disease-Modifying Antirheumatic Drugs (DMARDs):
  • Methotrexate or Sulfasalazine may be used for those with frequent or severe flare-ups, although they are more commonly used for patients who develop RA.
  • Hydroxychloroquine (used for other inflammatory conditions) may sometimes be considered, particularly when there are systemic symptoms like rashes or mild fever.
• Biologic Agents: TNF inhibitors (e.g., Etanercept, Adalimumab) or IL-6 inhibitors (e.g., Tocilizumab) may be used in refractory cases or if PR progresses into rheumatoid arthritis.

• Colchicine: Colchicine (an anti-inflammatory medication) may be used in the treatment of palindromic rheumatism. Colchicine is a medication that can help to reduce inflammation and pain. 
• Tofacitinib: Tofacitinib is a Janus kinase (JAK) inhibitor. While primarily used for rheumatoid arthritis, it may be considered in some cases of palindromic rheumatism, especially when other treatments have failed.
• Physical Therapy: Regular physical therapy and stretching exercises can help maintain joint mobility and prevent stiffness during periods of remission.
• Lifestyle Modifications: Stress management, weight control, and regular exercise can help reduce the frequency and severity of flare-ups. Avoiding known triggers, such as infections or injury, may also be helpful in reducing symptoms.

Prognosis

• Potential Progression to RA: In some individuals, PR can progress to rheumatoid arthritis (RA) over time, especially if flare-ups become more frequent and severe. This progression occurs in about 10-30% of patients with PR.
• Normal Life Expectancy: Palindromic rheumatism does not typically affect life expectancy. However, if it progresses to RA or other serious complications, those conditions may have a more significant impact.
• Overall Good Prognosis: With appropriate management, the disease is generally manageable, and many patients lead normal, active lives with few flare-ups and minimal lasting effects.

Scoring

• Palindromic Rheumatism Disease Activity Score (PRDAS): A proposed tool that evaluates the frequency, duration, and severity of flare-ups, as well as the impact on joint function and quality of life. It helps track flare frequency and disease progression.
• ESR/CRP: These inflammatory markers can be used to assess disease activity during flare-ups and provide insight into the level of inflammation present.
• Pain and Disability Scores: While not specific to PR, scales like the Visual Analog Scale (VAS) for pain and the Health Assessment Questionnaire (HAQ) for disability can be used to track symptoms during flare-ups and recovery.
• Patient Global Assessment: This score involves the patient's own assessment of how they feel overall (pain, fatigue, and functional status) during and between flare-ups, helping guide treatment decisions.