Remitting seronegative symmetrical synovitis with pitting edema

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Remitting seronegative symmetrical synovitis with pitting edema
Other namesRemitting seronegative symmetrical synovitis with pitting oedema [1]
RS3PE
Specialty Rheumatology

Remitting seronegative symmetrical synovitis with pitting edema (or sometimes RS3PE) is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema (swelling) of the back of the hands and/or feet, and a negative serum rheumatoid factor. [2] If no underlying disorder can be identified (idiopathic RS3PE), this entity has an excellent prognosis and responds well to treatment. [3]

Contents

RS3PE typically involves the joints of the extremities, specifically the metacarpophalangeal and proximal interphalangeal joints, wrists, shoulders, elbows, knees and ankles. [4] It is more common in older adults, with the mean age between 70 and 80 years in most studies. [4] [5] It occurs more often in men than in women with a 2:1 ratio. [4] [6] [7] It is unknown how common this condition is.

Signs and symptoms

Individuals affected by RS3PE typically have repeated episodes of inflammation of the lining of their synovial joints and swelling of the end portion of the limbs. [8] The arms and hands are more commonly affected than the legs and feet. [8] Both sides are usually involved though RS3PE can affect only one side in certain cases. [8]

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. [5] Thus, RS3PE has been proposed as a condition related to PMR or even that they are both part of the same disorder. [5] However, PMR typically requires protracted courses of steroids, whereas corticosteroids can be tapered more quickly with persisting remission in RS3PE. [5]

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 arthropathy due to amyloidosis. [6] [9]

RS3PE has been documented in patients with cancers (Non-Hodgkin's lymphoma, gastric cancer, pancreatic cancer, lung cancer, breast cancer, colon cancer, prostate cancer and bladder cancer, among others), in whom it might represent a paraneoplastic manifestation. [10] [11] [12] Other underlying disorders include vasculitides such as polyarteritis nodosa. [8]

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.

Pathogenesis

The disease mechanism (pathophysiology) of RS3PE remains unknown. One study suggested a possible role for vascular endothelial growth factor. [13] A study using magnetic resonance imaging found that tenosynovitis of the extensors of the hands and feet is the major contributor to edema. [14]

Diagnosis

Ultrasonography and magnetic resonance imaging of the hands and/or feet have been proposed as useful diagnostic investigations in RS3PE. [15]

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. [6]

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. [16] RS3PE was initially thought to represent a form of seronegative rheumatoid arthritis but is now believed to be a separate syndrome. [8]

Related Research Articles

<span class="mw-page-title-main">Lymphedema</span> Swelling due to a compromised lymphatic system

Lymphedema, also known as lymphoedema and lymphatic edema, is a condition of localized swelling caused by a compromised lymphatic system. The lymphatic system functions as a critical portion of the body's immune system and returns interstitial fluid to the bloodstream.

<span class="mw-page-title-main">Rheumatoid arthritis</span> Type of autoimmune arthritis

Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involved, with the same joints typically involved on both sides of the body. The disease may also affect other parts of the body, including skin, eyes, lungs, heart, nerves, and blood. This may result in a low red blood cell count, inflammation around the lungs, and inflammation around the heart. Fever and low energy may also be present. Often, symptoms come on gradually over weeks to months.

Rheumatology is a branch of medicine devoted to the diagnosis and management of disorders whose common feature is inflammation in the bones, muscles, joints, and internal organs. Rheumatology covers more than 100 different complex diseases, collectively known as rheumatic diseases, which includes many forms of arthritis as well as lupus and Sjögren's syndrome. Doctors who have undergone formal training in rheumatology are called rheumatologists.

<span class="mw-page-title-main">Tenosynovitis</span> Inflammation of the fluid sheath (synovium) around a tendon

Tenosynovitis is the inflammation of the fluid-filled sheath that surrounds a tendon, typically leading to joint pain, swelling, and stiffness. Tenosynovitis can be either infectious or noninfectious. Common clinical manifestations of noninfectious tenosynovitis include de Quervain tendinopathy and stenosing tenosynovitis.

<span class="mw-page-title-main">Psoriatic arthritis</span> Long-term inflammatory arthritis

Psoriatic arthritis (PsA) is a long-term inflammatory arthritis that occurs in people affected by the autoimmune disease psoriasis. The classic feature of psoriatic arthritis is swelling of entire fingers and toes with a sausage-like appearance. This often happens in association with changes to the nails such as small depressions in the nail (pitting), thickening of the nails, and detachment of the nail from the nailbed. Skin changes consistent with psoriasis frequently occur before the onset of psoriatic arthritis but psoriatic arthritis can precede the rash in 15% of affected individuals. It is classified as a type of seronegative spondyloarthropathy.

<span class="mw-page-title-main">Dactylitis</span> Medical condition

Dactylitis or sausage digit is inflammation of an entire digit, and can be painful.

<span class="mw-page-title-main">Synovial fluid</span> Fluid found in the cavities of synovial joints

Synovial fluid, also called synovia,[help 1] is a viscous, non-Newtonian fluid found in the cavities of synovial joints. With its egg white–like consistency, the principal role of synovial fluid is to reduce friction between the articular cartilage of synovial joints during movement. Synovial fluid is a small component of the transcellular fluid component of extracellular fluid.

<span class="mw-page-title-main">Polymyalgia rheumatica</span> Pain or stiffness usually in the shoulders and/or hips

Polymyalgia rheumatica (PMR) is a syndrome experienced as pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but which may occur all over the body. The pain can be sudden or can occur gradually over a period. Most people with PMR wake up in the morning with pain in their muscles; however, cases have occurred in which the person has developed the pain during the evenings or has pain and stiffness all day long.

<span class="mw-page-title-main">Polyarthritis</span> Medical condition

Polyarthritis is any type of arthritis that involves 5 or more joints simultaneously. It can be associated with autoimmune conditions; it may be experienced at any age and is not sex specific.

SAPHO syndrome includes a variety of inflammatory bone disorders that may be associated with skin changes. These diseases share some clinical, radiologic, and pathologic characteristics.

<span class="mw-page-title-main">Arthropathy</span> Medical condition

An arthropathy is a disease of a joint.

Palindromic rheumatism (PR) is a syndrome characterised by recurrent, self-resolving inflammatory attacks in and around the joints (rheumatism), and consists of arthritis or periarticular soft tissue inflammation. The course is often acute onset, with sudden and rapidly developing attacks or flares. There is pain, redness, swelling, and disability of one or multiple joints. The interval between recurrent palindromic attacks and the length of an attack is extremely variable from few hours to days. Attacks may become more frequent with time but there is no joint damage after attacks. It is thought to be an autoimmune disease, possibly an abortive form of rheumatoid arthritis.

<span class="mw-page-title-main">Winchester syndrome</span> Rare hereditary connective tissue disease

Winchester syndrome is a rare hereditary connective tissue disease described in 1969, of which the main characteristics are short stature, marked contractures of joints, opacities in the cornea, coarse facial features, dissolution of the carpal and tarsal bones, and osteoporosis. Winchester syndrome was once considered to be related to a similar condition, multicentric osteolysis, nodulosis, and arthropathy (MONA). However, it was discovered that the two are caused by mutations found in different genes; however they mostly produce the same phenotype or clinical picture. Appearances resemble rheumatoid arthritis. Increased uronic acid is demonstrated in cultured fibroblasts from the skin and to a lesser degree in both parents. Despite initial tests not showing increased mucopolysaccharide excretion, the disease was regarded as a mucopolysaccharidosis. Winchester syndrome is thought to be inherited as an autosomal recessive trait.

<span class="mw-page-title-main">Arthritis mutilans</span> Medical condition

Arthritis mutilans is a rare medical condition involving severe inflammation damaging the joints of the hands and feet, and resulting in deformation and problems with moving the affected areas; it can also affect the spine. As an uncommon arthropathy, arthritis mutilans was originally described as affecting the hands, feet, fingers, and/or toes, but can refer in general to severe derangement of any joint damaged by arthropathy. First described in modern medical literature by Marie and Leri in 1913, in the hands, arthritis mutilans is also known as opera glass hand, or chronic absorptive arthritis. Sometimes there is foot involvement in which toes shorten and on which painful calluses develop in a condition known as opera glass foot, or pied en lorgnette.

Crystal arthropathy is a class of joint disorder that is characterized by accumulation of tiny crystals in one or more joints. Polarizing microscopy and application of other crystallographic techniques have improved identification of different microcrystals including monosodium urate, calcium pyrophosphate dihydrate, calcium hydroxyapatite, and calcium oxalate.

<span class="mw-page-title-main">Blau syndrome</span> Medical condition

Blau syndrome is an autosomal dominant genetic inflammatory disorder which affects the skin, eyes, and joints. It is caused by a mutation in the NOD2 (CARD15) gene. and is classified as an inborn errors of immunity. Symptoms usually begin before the age of four, and the disease manifests as early onset cutaneous sarcoidosis, granulomatous arthritis, and uveitis.

<span class="mw-page-title-main">Enteropathic arthropathy</span> Medical condition

Enteropathic arthropathy commonly referred to as enteropathic arthritis, is a type of arthritis linked to Crohn's disease, ulcerative colitis, and chronic inflammatory bowel diseases.

<span class="mw-page-title-main">Intermittent hydrarthrosis</span> Recurring swelling primarily in the knee

Intermittent hydrarthrosis (IH), also known as periodic synoviosis, periodic benign synovitis, or periodic hydrarthritis, is a chronic condition of unknown cause characterized by recurring, temporary episodes of fluid accumulation (effusion) in the knee. While the knee is mainly involved, occasionally other joints such as the elbow or ankle can additionally be affected. Fluid accumulation in the joint can be extensive causing discomfort and impairing movement, although affected joints are not usually very painful. While the condition is chronic, it does not appear to progress to more destructive damage of the joint. It seems to affect slightly more women than men.

Ross E. Petty is a Canadian pediatric rheumatologist. He is a professor emeritus in the Department of Pediatrics at the University of British Columbia and a pediatric rheumatologist at BC Children’s Hospital in Vancouver, Canada. He established Canada’s first formal pediatric rheumatology program at the University of Manitoba in 1976, and three years later, he founded a similar program at the University of British Columbia.

<span class="mw-page-title-main">Trabecular oedema</span> Medical condition

Trabecular edema, also known as bone marrow edema (BME), is a traditional term describing the interstitial fluid accumulation at the trabecular bone marrow. The term was first used in 1988, referring to the changes in the bone marrow due to inflammation. Bone marrow edema was later renamed to bone marrow lesion (BML), as later studies show that the increased fluid content in the trabecular bone was more likely caused by inflammatory responses instead of fluid influx. Hence, this narrows down the condition to the damage at the articular surface of the trabecular bones. Despite so, the terms BME and BML are still used interchangeably in radiology.

References

  1. ↑ 'Oedema' is the standard form defined in the Concise Oxford English Dictionary (2011), with the precision that the spelling in the United States is 'edema'.
  2. ↑ Olivieri I, Salvarani C, Cantini F (2000). "RS3PE syndrome: an overview". Clin. Exp. Rheumatol. 18 (4 Suppl 20): S53–55. PMID   10948764.
  3. ↑ Queiro R (March 2004). "RS3PE syndrome: a clinical and immunogenetical study". Rheumatol. Int. 24 (2): 103–05. doi:10.1007/s00296-003-0330-3. PMID   12750942. S2CID   22516577.
  4. 1 2 3 Olivé A, del Blanco J, Pons M, Vaquero M, Tena X (February 1997). "The clinical spectrum of remitting seronegative symmetrical synovitis with pitting edema. The Catalán Group for the Study of RS3PE". J. Rheumatol. 24 (2): 333–36. PMID   9034993.
  5. 1 2 3 4 Cantini F, Salvarani C, Olivieri I, et al. (April 1999). "Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome: a prospective follow up and magnetic resonance imaging study". Ann. Rheum. Dis. 58 (4): 230–36. doi:10.1136/ard.58.4.230. PMC   1752869 . PMID   10364902.
  6. 1 2 3 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.
  7. ↑ Hartley AJ, Manson J, Jawad AS (2010). "Remitting seronegative symmetrical synovitis with pitting oedema". Grand Rounds. 10: 71–73. doi:10.1102/1470-5206.2010.0015 (inactive 1 November 2024). Archived from the original on 2011-07-11. Retrieved 2010-12-20.{{cite journal}}: CS1 maint: DOI inactive as of November 2024 (link)
  8. 1 2 3 4 5 Kardes S, Karagulle M, Erdogan N (May 2015). "Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) associated with psoriatic arthritis". Scand J Rheumatol. 44 (4): 339–40. doi:10.3109/03009742.2015.1020069. PMID   25958968. S2CID   2298418.
  9. ↑ Díez-Porres L, Muñoz-Fernández S, Aguado P, Alonso M, Martín-Mola E (November 2002). "Remitting seronegative symmetrical synovitis with pitting oedema as the first manifestation of psoriatic arthropathy". Rheumatology (Oxford). 41 (11): 1333–35. doi: 10.1093/rheumatology/41.11.1333-a . PMID   12422012.
  10. ↑ Russell EB (September 2005). "Remitting seronegative symmetrical synovitis with pitting edema syndrome: followup for neoplasia". J. Rheumatol. 32 (9): 1760–61. PMID   16142875. Archived from the original on 2009-04-29. Retrieved 2009-01-29.
  11. ↑ Fietta P, Manganelli P (November 2006). "Remitting seronegative symmetrical synovitis with pitting edema syndrome: followup for neoplasia". J. Rheumatol. 33 (11): 2365–66, author reply 2366. PMID   17086622.[ dead link ]
  12. ↑ Bucaloiu ID, Olenginski TP, Harrington TM (December 2007). "Remitting seronegative symmetrical synovitis with pitting edema syndrome in a rural tertiary care practice: a retrospective analysis". Mayo Clin. Proc. 82 (12): 1510–15. doi:10.4065/82.12.1510. PMID   18053459. Archived from the original on 2007-07-01.
  13. ↑ Arima K, Origuchi T, Tamai M, et al. (November 2005). "RS3PE syndrome presenting as vascular endothelial growth factor associated disorder". Ann. Rheum. Dis. 64 (11): 1653–55. doi:10.1136/ard.2004.032995. PMC   1755286 . PMID   16227418.
  14. ↑ Olivieri I, Salvarani C, Cantini F (February 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.
  15. ↑ Agarwal V, Dabra AK, Kaur R, Sachdev A, Singh R (September 2005). "Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome: ultrasonography as a diagnostic tool". Clin. Rheumatol. 24 (5): 476–79. doi:10.1007/s10067-004-1061-x. PMID   15856369. S2CID   20352825.
  16. ↑ McCarty DJ, O'Duffy JD, Pearson L, Hunter JB (November 1985). "Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome". JAMA. 254 (19): 2763–67. doi:10.1001/jama.254.19.2763. PMID   4057484.
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