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RS3PE Syndrome: Bad or Good Prognosis? To the Editor: We read with great interest the article by Russell regarding the potential relationship between remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome and neoplasia1. As the author concluded, prospective followup studies would help clarify longterm risk of neoplasia in patients with RS3PE syndrome. Since 1988 we have followed 15 cases of RS3PE syndrome in a standardized manner at a single university institution. Reports on 12 cases have been published, and we describe here the clinical, immunogenetic, and development characteristics of the whole cohort, adding 3 new cases seen in the last 3 years2. All cases fulfilled the main features of RS3PE syndrome given in the seminal article by McCarty, et al3. Our study protocol for these cases has been published, and these 3 new cases were followed according to it2. The main results are shown in Table 1. No patient needed disease modifying antirheumatic drugs to achieve remission. Although initially RS3PE syndrome appeared to be a well characterized entity with a good prognosis, in the last few years several groups have questioned this view, as the syndrome has been associated with several solid tumors, hematological diseases, immunostimulants, infections, and rheumatic conditions4. Since its initial description, most cases have shown clinical, developmental, and immunogenetic features that distinguished RS3PE from the 2 most closely related conditions, late-onset rheumatoid arthritis (RA) and polymyalgia rheumatica (PMR)3. In that sense, an association between RS3PE and HLA-DR antigens has not yet emerged, whereas both RA and PMR are correlated with HLA-DR4, although the putative epitopes responsible for these 2 conditions differ in location5. In our view, RS3PE syndrome may be seen from 3 different perspectives. The first plausible explanation is that this entity may be a type of reactive arthritis favored by some HLA antigens, such as B27 and B7-CREG antigens. In the present series, 46% showed at least one of these antigens, but we did not investigate the presence of habitual pathogens for this type of arthritis. Also favoring this notion, a late-onset undifferentiated spondyloarthropathy syndrome resembling the features of RS3PE has been described in men over 50 years of age, and over time some of them developed ankylosing spondylitis6. A second conception has been supported by some authors, that both PMR and RS3PE syndrome are similar conditions, emphasizing the idea that the presence of distal edemas only indicates a better prognosis and not a real difference between the 27. However, we must keep in mind that the HLA profile of these 2 conditions is quite different, as we cited above and also show here. Finally, a third conception is that the condition could involve a type of paraneoplastic syndrome linked to the synthesis of a factor such as interleukin 6, among others8. This view is supported by some recent case reports and small series, but we found no case of neoplasia in the present report. We found one patient who presented an IgG-kappa monoclonal paraproteinemia, but he has developed neither multiple myeloma nor malignant disease over a followup of 9 years. Two of our patients died, one with acute myocardial infarction and one with a cerebral hemorrhage. In our opinion, RS3PE syndrome is a well characterized entity with a spectrum of severity ranging from the less severe and most common forms, represented by cases with one attack and no relapse; then an intermediate form, in which there are relapses successfully treated with corticosteroids (20% of the present series); and in the extreme of the spectrum, cases associated with neoplasia, which fortunately represent a minority among RS3PE cases. In our context, RS3PE still remains a definite condition with an excellent prognosis, but some questions remain unsolved. For example, which patients will have a benign, self-limiting course and which will have more protracted disease, and more importantly, whether the disease course and the risk of neoplasia can be predicted on the basis of the initial presentation or the HLA profile. RUBEN QUEIRO, MD, PhD; MERCEDES ALPERI, MD; JOSE LUIS RIESTRA, MD, PhD; JAVIER BALLINA, MD, PhD, Rheumatology Service, Hospital Universitario Central de Asturias, C/ Celestino Villamil s/n, 33006 Oviedo, Asturias, Spain. Address reprint requests to Dr. Queiro. E-mail: ruquei@mixmail.com REFERENCES 1. Russell EB. Remitting seronegative symmetrical synovitis with pitting edema syndrome: followup for neoplasia. J Rheumatol 2005;32:1760-1. [MEDLINE] 2. Queiro R. RS3PE syndrome: a clinical and immunogenetical study. Rheumatol Int 2004;24:103-5. [MEDLINE] 3. McCarty DJ, O'Duffy JD, Pearson L, Hunter JB. Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome. JAMA 1985;254:2763-7. [MEDLINE] 4. Schaeverbeke T, Fatout E, Marce S, et al. Remitting seronegative symmetrical synovitis with pitting edema: disease or syndrome? Ann Rheum Dis 1995;54:681-4. [MEDLINE] 5. Weyand CM, Hunder NN, Hicok KC, Hunder GG, Goronzy JJ. HLA-DRB1 alleles in polymyalgia rheumatica, giant cell arteritis, and rheumatoid arthritis. Arthritis Rheum 1994;37:514-20. [MEDLINE] 6. Olivieri I, Padula A, Pierro A, Favaro L, Oranges GS, Ferri S. Late onset undifferentiated seronegative spondyloarthropathy. J Rheumatol 1995;22:899-903. [MEDLINE] 7. Cantini F, Salvarani C, Olivieri I, et al. Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome: a prospective follow up and magnetic resonance imaging study. Ann Rheum Dis 1999;58:230-6. [MEDLINE] 8. Sibilia J, Friess S, Schaeverbeke T, et al. Remitting seronegative symmetrical synovitis with pitting edema (RS3PE): a form of paraneoplastic polyarthritis? J Rheumatol 1999;26:115-20. [MEDLINE]
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