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We read with interest the paper by Cantiniet al and would like to comment on it.1
In 1992 we performed a retrospective multicentre study of 27 RS3PE patients. We concluded that personal history of polymyalgia rheumatica (two patients), presence of erosions (one patient) and evolution to haematological diseases (two patients concomitantly developed a T lymphoma and one a myelodiplastic syndrome) suggested that RS3PE syndrome might not be a distinct clinical entity. At that moment 12 patients were asymtomatic and 12 required treatment. This was reported elsewhere.2
Now, six years later, we have reviewed the original cohort of patients with the RS3PE syndrome. A questionnaire was sent to the participating rheumatologists. The survey focused on articular symtoms, treatment and evolution. The current cohort was composed of 22 patients (male 16; female 6; mean age:77.9; range 64–91). Four patients died (the three with haematological diseases, one stroke) and one was not located. Thirteen patients were asymtomatic and without treatment, in contrast nine required treatment, namely corticosteroids (6), gold salts (1), cloroquine (1) and NSAID (1). Interestingly, two of the patients were identified by their rheumatologist as having a seronegative rheumatoid arthritis, another patient had a chronic disease with separate corticosteroid responsive episodes of bilateral hand oedema and polymyalgic symtons at different times. Last but not least one patient developed Raynaud’s phenomena, both hands had sclerodactily. A nailfold capillary microscopy showed a decreased number of capillary loops, which were widened, suggesting systemic sclerosis.
Our results suggests that RS3PE syndrome has a good prognosis as more than half of the patients are asymtomatic and without treatment six years later. However, there is a subset of patients that have other diseases. Although pure RS3PE syndrome does exist the evolution should be carefully monitored.
We thank the following rheumatologists for the contribution to the study: Jordi del Blanco, Miquel Pons, Isabel Rotés, Raimon Sanmartí, Eduardo Kantarewicz, Miquel Sala, Ivonne Breysse, Rosa Roselló, Javier Arasa, Marta Larrosa, Genonima Cañellas, Josep Pujol, Anna Lafont.
We appreciate the comment by Olivé et al on our article on RS3PE. They reviewed 27 previously described RS3PE patients after a follow up of six years.
As we suggested in a previous report,1-1 they confirm that RS3PE syndrome should be considered a heterogenous condition associated with different inflammatory rheumatic diseases and also with neoplastic disorders.
In our study1-2 none of the 23 patients with RS3PE syndrome developed clinical manifestations supporting the diagnosis for another disease. The different study design and selection of patients may in part explain the subset of patients with other diseases and with a worse prognosis observed by Olivé et al.
We designed a prospective follow up study excluding patients satisfying the criteria for the diagnosis of polymyalgia rheumatica, rheumatoid arthritis and seronegative spondylarthropathies. Moreover, patients with a clinical history of cancer were excluded from the study. In their original report1-3 these authors performed a retrospective study including all patients with remitting distal extremity swelling with pitting oedema. They recruited also patients not evaluated for spondylarthropathies, which may be associated with distal extremity swelling with pitting oedema.1-4
However, in their retrospective evaluation Olivéat al found that 13 of 22 (59%) patients were asymptomatic and drug free over a six year follow up period, confirming that RS3PE not associated with other conditions and with a good prognosis does exist.
The problem is how to label this clinical picture. As discussed in our article,1-2 the similarities of demographic, clinical and MRI findings between patients with “pure” RS3PE syndrome and those with polymyalgia rheumatica and the concurrence of the two syndromes suggest that these conditions may be part of the clinical spectrum of the same disease. In the series of Olivé et al the patient with a clinical course characterised by alternate relapses of bilateral hand pitting oedema or polymyalgic symptoms further supports our hypothesis. Even those RS3PE patients successively diagnosed as having seronegative rheumatoid arthritis (elderly onset rheumatoid arthritis) do not conflict with our conclusions. Healey described patients who developed episodes of polymyalgia rheumatica and seronegative rheumatoid arthritis at different times during follow up.1-5 Similar clinical characteristics have been recently described in a population based cohort of patients with giant cell arteritis followed up over a 42 year period. Four of the six patients who fulfilled the criteria for the diagnosis of rheumatoid arthritis during the follow up experienced multiple separate episodes of symmetrical arthritis, proximal symptoms of polymyalgia rheumatica and distal extremity swelling with pitting oedema.1-6
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