Remitting seronegative symmetrical synovitis with pitting edema: Difference between revisions

From Wikipedia, the free encyclopedia
Next edit →
Content deleted Content added
VisualWikitext
new article
(No difference)

Revision as of 23:49, 29 January 2009

In rheumatology, remitting seronegative symmetrical synovitis with pitting edema (abbreviated RS3PE or sometimes RS3PE) is a rare syndrome which consists of synovitis (inflammation of the synovial membrane of the joints) that is usually symmetrical (bilateral) and is associated with sudden pitting edema of the back of the hands and/or feet.[1] If no underlying disorder can be identified (idiopathic RS3PE), this entity has an excellent prognosis and responds well to treatment.[2] Blood tests for other rheumatological conditions (more specifically, rheumatoid factor) are typically negative (hence the label seronegative).

RS3PE is typically a polyarthritis (inflammation of multiple joints) of the extremities, involving the metacarpophalangeal and proximal interphalangeal joints, wrists, shoulders, elbows, knees and ankles.[3] It appears to be more common in older adults (mean age between 70 and 80 in most studies).[4][3] It appears to occur more often in men than in women with a 2:1 ratio.[3][5] It is unknown how common this condition occurs.

Causes

RS3PE is a constellation of symptoms that can be caused by many other conditions. Since there is no definitive diagnostic test, other conditions have to be ruled out before this rare condition can be diagnosed.

The main differential diagnosis is polymyalgia rheumatica (PMR), although pain, stiffness and weakness at the level of the shoulders and pelvic girdle with associated systemic symptoms (fever, malaise, fatigue, weight loss) is more typical of PMR. Prospective studies have found a subgroup of PMR patients with hand edema, as well as other similarities.[4] Thus, RS3PE has been proposed as a condition related to PMR or even that they are both part of the same disorder.[4] However, PMR typically requires protracted courses of steroids, whereas corticosteroids can be tapered more quickly with persisting remission in RS3PE.[4]

Other rheumatological disorders that can cause the features typical for RS3PE include late onset (seronegative) rheumatoid arthritis, acute sarcoidosis, ankylosing spondylitis and other spondyloarthropathies such as psoriatic arthropathy, mixed connective tissue disease, chondrocalcinosis and amyloid arthropathy.[5][6]

RS3PE has been documented in patients with cancers (gastric cancer, pancreatic cancer, lung cancer, breast cancer, colon cancer, prostate cancer and Non-Hodgkin's lymphoma, among others), in whom it might represent a paraneoplastic manifestation.[7][8] Other underlying disorders include vasculitides such as polyarteritis nodosa.[6]

Other causes of edema include heart failure, hypoalbuminemia, nephrotic syndrome and venous stasis. The key distinguishing feature is that these conditions don't tend to manifest with pitting edema at the back of the hands.

Diagnosis

Ultrasonography and magnetic resonance imaging of the hands and/or feet have been proposed as useful diagnostic investigations in RS3PE.[9]

Some studies linked RS3PE to HLA-B27 whereas others have not.

Treatment

RS3PE responds excellently to low dose corticosteroids, with sustained and often complete remission. Non-steroidal anti-inflammatory drugs (NSAIDs) have also been used. Hydroxychloroquine has proven effective in some cases.[5]

Disease mechanism

The disease mechanism (pathophysiology) of RS3PE remains unknown. One study suggested a possible role for vascular endothelial growth factor.[10] A study using magnetic resonance imaging found that tenosynovitis of the extensors of the hands and feet is the major contributor to edema.[11]

History

In a 1985 paper published in the Journal of the American Medical Association, McCarty and colleagues first described a case series of patients with this disorder, for which they coined the abbreviation RS3PE.[12]

Footnotes

  1. ^ Olivieri I, Salvarani C, Cantini F (2000). "RS3PE syndrome: an overview". Clin. Exp. Rheumatol. 18 (4 Suppl 20): S53–5. PMID 10948764.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. ^ Queiro R (2004). "RS3PE syndrome: a clinical and immunogenetical study". Rheumatol. Int. 24 (2): 103–5. doi:10.1007/s00296-003-0330-3. PMID 12750942. {{cite journal}}: Unknown parameter |month= ignored (help)
  3. ^ a b c Olivé A, del Blanco J, Pons M, Vaquero M, Tena X (1997). "The clinical spectrum of remitting seronegative symmetrical synovitis with pitting edema. The Catalán Group for the Study of RS3PE". J. Rheumatol. 24 (2): 333–6. PMID 9034993. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  4. ^ a b c d Cantini F, Salvarani C, Olivieri I; et al. (1999). "Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome: a prospective follow up and magnetic resonance imaging study". Ann. Rheum. Dis. 58 (4): 230–6. PMC 1752869. PMID 10364902. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  5. ^ a b c Salam A, Henry R, Sheeran T (2008). "Acute onset polyarthritis in older people: Is it RS3PE syndrome?". Cases J. 1 (1): 132. doi:10.1186/1757-1626-1-132. PMC 2543002. PMID 18759976.{{cite journal}}: CS1 maint: multiple names: authors list (link) CS1 maint: unflagged free DOI (link)
  6. ^ a b Díez-Porres L, Muñoz-Fernández S, Aguado P, Alonso M, Martín-Mola E (2002). "Remitting seronegative symmetrical synovitis with pitting oedema as the first manifestation of psoriatic arthropathy". Rheumatology (Oxford). 41 (11): 1333–5. PMID 12422012. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  7. ^ Russell EB (2005). "Remitting seronegative symmetrical synovitis with pitting edema syndrome: followup for neoplasia". J. Rheumatol. 32 (9): 1760–1. PMID 16142875. {{cite journal}}: Unknown parameter |month= ignored (help)
  8. ^ Fietta P, Manganelli P (2006). "Remitting seronegative symmetrical synovitis with pitting edema syndrome: followup for neoplasia". J. Rheumatol. 33 (11): 2365–6, author reply 2366. PMID 17086622. {{cite journal}}: Unknown parameter |month= ignored (help)
  9. ^ Agarwal V, Dabra AK, Kaur R, Sachdev A, Singh R (2005). "Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome: ultrasonography as a diagnostic tool". Clin. Rheumatol. 24 (5): 476–9. doi:10.1007/s10067-004-1061-x. PMID 15856369. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  10. ^ Arima K, Origuchi T, Tamai M; et al. (2005). "RS3PE syndrome presenting as vascular endothelial growth factor associated disorder". Ann. Rheum. Dis. 64 (11): 1653–5. doi:10.1136/ard.2004.032995. PMC 1755286. PMID 16227418. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  11. ^ Olivieri I, Salvarani C, Cantini F (1997). "Remitting distal extremity swelling with pitting edema: a distinct syndrome or a clinical feature of different inflammatory rheumatic diseases?". J. Rheumatol. 24 (2): 249–52. PMID 9034979. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  12. ^ McCarty DJ, O'Duffy JD, Pearson L, Hunter JB (1985). "Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome". JAMA. 254 (19): 2763–7. PMID 4057484. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
Retrieved from "https://en.wikipedia.org/w/index.php?title=Remitting_seronegative_symmetrical_synovitis_with_pitting_edema&oldid=267298213"