It is often a case of eliminating the other conditions before getting the correct diagnosis due to there being no specific test for PR diagnosis. No single test can confirm a diagnosis. A doctor may make a diagnosis based on medical history and signs and symptoms. Palindromic rheumatism must be distinguished from acute gouty arthritis and an atypical, acute onset of rheumatoid arthritis (RA). Without specific tests (such as analysis of joint fluid), it may be difficult to distinguish palindromic rheumatism from other episodic joint problems. It is important to note that a person may experience more than one autoimmune disorder at the same time, as overlap syndrome. Laboratory findings are usually normal. Blood tests may show an elevation of the ESR and CRP, but are otherwise unremarkable. Rheumatoid factor may be present especially in the group that is likely to develop rheumatoid arthritis. Proposed classification by Guerne and Weismann in 1992:
A 6-month history of brief sudden-onset and recurrent episodes of monoarthritis or rarely polyarthritis or of soft tissue inflammation. Direct observation of one attack by a physician. Three or more joints involved in different attacks. No radiologic evidence of bone or joint erosion. Exclusion of other arthritides, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or gout

Management
Treatment may include nonsteroidal anti-inflammatory drugs (NSAIDs) for acute attacks. Antimalarials, such as hydroxychloroquine, have been helpful in reducing the frequency and duration of attacks and may reduce the likelihood that palindromic rheumatism will progress to rheumatoid arthritis.
In patients with a single site of involvement, survivability tends to be very favorable. However, those with multi-organ involvement have a mortality rate of 50-66%. One systematic review calculated the disease-specific survival as 27.2 months, or 58% at one year. One group found the death rate to be 50% in patients with LCS, compared to 31% in patients who had the related condition LCH. Survivability decreases dramatically with increased disease burden and spread. LCS may be associated with other malignancies as well, such as follicular lymphoma, adenocarcinoma, and germ cell tumors. Evidence of metastatic disease or relapse from a previously treated LCS usually signifies a worsened prognosis. Treatment of these metastatic lesions or relapsed tumors may improve the patients prognosis, but there is limited evidence as to a preferred therapy. See also
Langerhans cell histiocytosis
Sarcoma

References


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