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We report on three patients who presented with polymyalgia but on subsequent clinical and laboratory assessment showed findings consistent with primary Sjögren’s syndrome.
Clinical, immunological, and genetic differences exist now to classify Sjögren’s syndrome (SS) more clearly into primary and secondary SS than in the past.1 2 Primary SS patients can present with a plethora of symptoms although most patients present with sicca complaints, lethargy or arthralgia. Polymyalgia, as presenting complaints of primary SS, has not been reported previously.
Our patients presented during a two year period with proximal aching and stiffness associated with a raised erythrocyte sedimentation rate (ESR) and all three responded characteristically as in polymyalgia rheumatica (PMR) patients to oral prednisolone therapy.3Subsequent investigations and clinical evaluations (table 1) however raised the possibility of primary SS as the underlying condition, confirmed by the usual immunological parameters and all three patients showed some features of the sicca syndrome, although none had a history of swelling of the salivary gland.1 2
Follow up over at least a two year period showed that patients were relieved of their PMR symptoms but other clinical and laboratory features persisted, except for normalisation of the ESR. None, however, has yet been able to stop taking prednisolone altogether.
PMR in itself is a distinct syndrome characterised by proximal aching and stiffness, associated with raised ESR and characteristically responding to adequate prednisolone therapy in a week or 10 days with a significant reduction in the ESR in that time period.3 All three patients in this report responded in a similar manner, although they continued to have dry eyes or xerostomia, or both; Ro and La antibodies persisted. Noteworthy in this series of polymyalgia patients is the occurrence of this syndrome at a somewhat younger age than the average PMR patient.1 4 Primary SS is a distinct entity with mainly sicca complaints but with a plethora of manifestations including arthralgia or arthritis without actual joint destruction.1 2 We are not aware of any previous report of PMR as a presenting manifestation in this condition. PMR-like presentations have been reported in other rheumatic disorders such as rheumatoid arthritis, lupus or in association with a malignancy.4 To our knowledge, it has not been reported either as a presenting manifestation or as a complaint in established primary SS, although inflammatory arthritis or arthralgia without joint erosions occur commonly.
It is conceivable that the diagnosis of primary SS would have been missed in these patients as their sicca complaints were not prominent, but our index of suspicion for this disorder is high as a result of our clinical and research interests. Moreover, some patients with this disorder are never diagnosed, wrongly diagnosed or diagnosed after a long delay.1 Given our patients’ circumstances and both their clinical and laboratory features we did not feel it was justifiable or even necessary to undertake an invasive test such as lip biopsy to prove that they do have primary SS.1
In view of our experience perhaps primary SS should be added to the list of polymyalgia-like syndromes. Longer term follow up of such patients may be of interest to further study their clinical and laboratory progression. In particular it would be important to continually evaluate these patients with regard to the sicca component of the condition. Although around 90% of patients with primary SS have dry eyes or mouth, or both, practitioners should be aware that an occasional patient may have the condition without these features as we reported in the past.5 Whether some patients with typical PMR have subclinical or unrecognised SS is not known but we have started evaluating such patients with a view to screening for the presence of SS both on clinical and laboratory grounds.
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