Remitting seronegative symmetrical synovitis with pitting edema. It dramatically responds to low doses of glucocorticoids. Syndrome of remitting seronegative symmetrical synovitis with. Paraneoplastic remitting seronegative symmetrical synovitis. The syndrome was reported to be associated with hla b7 3 and hla a2 haplotypes. Deletions and duplications of the pafah1b1 and ywhae genes in 17p. It commonly has a benign course leading to remission but in some cases has been associated with neoplastic, other rheumatic, or comorbid diseases. Dear editor, a 48yearold female presented with pain and swelling in the bilateral proximal interphalangeal pip, metacarpophalangeal mcp and wrist joints for the past 20 days. Ruben queiro, mercedes alperi, jose luis riestra and javier ballina. Disease bioinformatics research of rs3pe syndrome has been linked to edema, synovitis, pitting edema, remitting seronegative symmetrical synovitis with, arthritis. However, the patients with pure rs3pe syndrome were characterised by shorter duration of treatment, lower cumulative corticosteroid dose and lower frequency. Rs3pe is a definite syndrome, subset of polyarthritis with favourable outcome and has a good prognosis in the older patients.
Pdf remitting seronegative symmetrical synovitis with. Paraneoplastic remitting seronegative symmetrical synovitis with pitting edema syndrome should be treated with lowdose prednisolone during pembrolizumab therapy. It was acute in onset, severe in intensity to such an extent that she was unable to make a. Remitting seronegative symmetrical synovitis with pitting oedema rs 3 pe syndrome is a rare inflammatory syndrome affecting the elderly, characterized by. Rs3pe can be associated with neoplasia and various rheumatic conditions. Rs3pe can be a disease and not a syndrome has been more and more evaluated. If no underlying disorder can be identified, this entity has an excellent prognosis and responds well to treatment.
The search terms included remitting seronegative symmetrical synovitis with pitting edema, remitting seronegative syndrome with pitting edema and rs3pe. In patients whose disease follows the expected course, an intensive search for underlying occult infections or cancers is. Another important characteristic of rs3pe syndrome. Remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome. Even if the adenocarcinoma relapses with time, the symptoms of rs3pe led our patient to an early diagnosis and successful treatment and to freedom from the disease for. A case of remitting seronegative symmetrical synovitis. Rs3pe syndrome, pembrolizumab, immune checkpoint inhibitor. Unilateral rs3pe in a patient of seronegative rheumatoid. The name of this condition comes from the initials of three researchers who first identified it.
Rs3pe is a type of benign seronegative polyarthritis that affects older people and is characterised by sudden onset of oedema and swelling of the dorsum of the hands with. Antiinflammatory treatment with corticosteroids leads to prompt improvement. Rs3pe is listed in the worlds largest and most authoritative dictionary database of abbreviations and acronyms. In older people it is important to distinguish this syndrome from pmr polymyalgia rheumatica in view of the duration of treatment with steroids. Pdf to determine the clinical characteristics of patients with pure remitting. Remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome. The name 3m is derived from the initials of the three researchers who first identified it. Here, we describe a 67yearold man with mds with refractory anemia who developed rs3pe syndrome 3 months after the diagnosis of mds.
Mas is a severe, potentially fatal disease characterized by severe systemic inflammation. S hort stature, h yperextensibility, o cular depression deeply set eyes, r ieger anomaly, and t eething delay. It may present, as a paraneoplastic manifestation especially in older people who show a poor response to steroids. Fordnafiberlengthmeasurement,cellsweresynchronized withtheadditionof100nm nocodazoleinculturemedia. Remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome is a very rare condition incorporating a tenosynovitis of the hands and wrists, as well as the feet, ankles and. Indeed, reported here, tocilizumab treatment resulted in a remarkable suppression of clinical symptoms, accompanied by a reduction in mmp3 levels as well. Weakness of the deep abdominal muscles ventrally crosses with weakness of the gluteus maximus and medius.
Lowercrossed syndrome lcs is also referred to as distal or pelvic crossed syndrome. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Download pdf 250k download meta ris compatible with endnote, reference manager, procite, refworks. Magnetic resonance imaging mri was used to describe the anatomical structures affected by inflammation in pure rs3pe syndrome. It is very suggestive of a subset of the seronegative polyarthritides of the elderly, the rs3pe syndrome or remitting seronegative symmetrical synovitis with pitting edema, as initially described by mccarty in 1985. It can present as an acute onset polyarthritis with associated pitting oedema of the extremities. The cause of sapho syndrome is unknown and treatment is focused on managing symptoms. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare syndrome consisting of acute symmetrical tenosynovitis of the hands and wrists associated with pain and marked pitting edema of the dorsum of the hands or the feet. Remitting seronegative symmetrical synovitis with pitting edema rs3pe, first described in 1985 by mccarty et al, is a rare syndrome that is a subset of the seronegative symmetrical polyarthritis of older people. Rs3pe is a type of benign seronegative polyarthritis that affects older people and is characterised by sudden onset of oedema and swelling of the dorsum of the hands with symmetrical polysynovitis involving wrists, metacarpophalangeal, interphalangeal, tarsal and metatarsophalangeal joints, tenosynovitis of the flexors and extensors of the hands, morning stiffness with negative rheumatoid. Remitting seronegative symmetrical synovitis with pitting oedema 73. Pd1 inhibitors have revolutionized the treatment of advanced. Rs3pe always has pitting edema, negative rf, absence of bony erosions, generally has an excellent therapeutic response to a small dose of glucocorticoid, and lacks the hladrb1 genotype that is present in both early and lateonset ra. Rs3pe in association with dipeptidyl peptidase4 inhibitor.
Aug 29, 2008 remitting seronegative symmetrical synovitis with pitting oedema syndrome, a rare inflammatory arthritis, commonly affects people in the older age group. This syndrome could be mistaken by other polyarthritis, which normally affects the elderly. Rs3pe syndrome is a rare but well defined syndrome known to be. Is remitting seronegative symmetrical synovitis with pitting. Remitting seronegative symmetrical synovitis with pitting oedema syndrome, a rare inflammatory arthritis, commonly affects people in the older age group. Treatment of a patient with remitting seronegative.
Background remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome is an acute inflammatory polyartritis and palpable pitting edema of the elderly patients. It usually presents as an acute, symmetric polysynovitis with edema of the dorsum of the hands and feet. The clinical features of the disease are characterized by bilatera we use cookies to enhance your experience on our website. The term short syndrome was first created to reflect several of the features of the original reported cases.
It is characterized by symmetrical distal pitting edema with synovitis of the hands and feet that is generally very responsive to lowdose glucocorticoids. Pulmonary infiltrations were detected concomitantly with the development of rs3pe syndrome, and prednisolone improved both the pulmonary and extrapulmonary lesions. Ab1034 the treatment approach for the management of. About europe pmc funders joining europe pmc governance roadmap outreach. Remitting seronegative symmetrical synovitis with pitting edema abbreviated rs3pe or sometimes rs3pe is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema swelling of the back of the hands andor feet, and a negative serum rheumatoid factor.
Myelodysplastic syndrome precedes the onset of remitting. We describe a case of rs3pe and explore the literature surrounding this arthritis. Sir, rs3pe is an inflammatory disorder of unknown aetiology that affects elderly persons 1. Rs3 pe syndrome definition of rs3 pe syndrome by medical. Considering the global picture, the diagnosis of rs3pe syndrome was. Recognition and management of polymyalgia rheumatica and. So it is now thought that the key features of 3p25 microdeletion syndrome are due to absence of setd5.
Syndrome of remitting seronegative symmetrical synovitis. Crps represents inhomogeneous chronic pain conditions. Oct 31, 2003 we report two cases of paraneoplastic remitting seronegative symmetrical synovitis with pitting edema rs3pe associated with prostatic adenocarcinoma. This hemitransection of the cord produces a characteristic set of clinical signs and symptoms. Remitting seronegative symmetrical synovitis with pitting edema a subgroup of pts with rheumatoid arthritis with sudden onset, seronegativity for rheumatoid factors management aspirin, nsaids prognosis excellent.
In lcs, tightness of the thoracolumbar extensors on the dorsal side crosses with tightness of the iliopsoas and rectus femoris. Myelodysplastic syndrome mds is characterized by infective hematopoiesis with possible transformation to leukemia. Polyarthritis and pitting oedema annals of the rheumatic. It is actually a combination of two forms of dystonia, blepharospasm and oromandibular dystonia omd. Rs3pe is a distinct syndrome entity rather than a subset of ra. Read paraneoplastic remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome. However, the patients with pure rs3pe syndrome were. Though the nature of the disease still remains illusive, the outcome is excellent.
By continuing to use our website, you are agreeing to our use of cookies. In particular, deletion of pafah1b1 causes isolated lissencephaly while deletions involving both pafah1b1 and ywhae cause millerdieker syndrome. The clinical features of the disease are characterized by bilateral pitting oedema of the hands and sudden onset of polyarthritis. Remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome is a definite subset of the seronegative symmetrical polyarthritis of the aged, characterized by dramatic pitting edema of the hands, male predominance, old age and exquisite response to. The paraneoplastic meaning of r3spe remitting seronegative simmetrical synovitis with pitting edema syndrome introduction. Over the years, several types of tumors have been described associated with the syndrome and. The differential diagnosis of rs3pe syndrome includes rheumatoid arthritis ra, complex regional pain syndrome, gout arthritis, systemic lupus erythematosus sle, systemic. Mar 07, 2016 there is no formal criteria for diagnosis yet. We have recently encountered two cases of rs3pe that developed shortly after the initiation of a dipeptidyl peptidase4 dpp4 inhibitor and resolved markedly upon cessation of it. Rs3pe is a syndrome first described in 1985 as a distinct type of seronegative rheumatoid arthritis. Rs3pe syndrome as paraneoplastic phenomenon since 1985, tumors have been descripted in association with rs3pe 1. Synovitis inflammation of the joints, acne, pustulosis thick yellow blisters containing pus often on the palms and soles, hyperostosis increase in bone substance and osteitis inflammation of the bones. Rs3pe as abbreviation means remitting seronegative symmetrical synovitis with pitting edema. Case report open access rs3pe syndrome developing during the course of probable toxic shock syndrome.
Meige syndrome belongs to a group of disorders known as dystonia. Rs3pe syndrome is a rare but welldefined syndrome known to be. In the latter, the clinical picture of rs3pe developed after surgical intervention for the primary lesion. The aetiology of the disease is still not well known but we can classify it as a disease with characteristic clinical features. On the other hand, rs3pe syndrome was diagnosed in 3 men 76, 76, and 81 years old.
Rs3pe could be in some cases a paraneoplastic syndrome. Rs3pe syndrome developing during the course of probable toxic. A new articular syndrome described as immunrelated side effect of immunotherapy. Rheumaknowledgy macrophage activation syndrome mas. It can also occur as a paraneoplastic syndrome and can sometimes be a harbinger of malignancy. The patient started treatment with rifampicin 300 mgmonth. Although the pathogenesis of rs3pe remains unknown, overproduction of il6 has been demonstrated to contribute to its development 2, 3. Remitting seronegative symmetrical synovitis with pitting. Remitting seronegative symmetrical synovitis with pitting oedema. This group of neurological conditions is generally characterized by involuntary muscle contractions that force the body into abnormal, sometimes painful, movements and. We hereby report a case of unilateral rs3pe in a patient of seronegative rheumatoid arthritis which was. Daniel mccarty and coworkers in 1985, it was felt to be a distinct form of elderly or lateonset rheumatoid arthritis lora characterized by symmetrical synovitis of the hands and ankles with pitting edema, elevated acute phase reactants, negative rheumatoid factor rf.
Although there are overlapping clinical manifestations between rs3pe, elderlyonset rheumatoid arthritis, and polymyalgia rheumatica, rs3pe has distinct characteristics. Remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome is a rare inflammatory arthritis, first described by mccarty et al in 1985 characterised by abrupt onset of symmetrical distal synovitis, marked pitting edema of the dorsum of the hands andor feet, absence of rheumatoid factor rf, and favorable response to corticosteroids. Nivolumab induced remitting seronegative symmetrical. S ir, rs3pe is an inflammatory disorder of unknown aetiology that affects elderly persons.
One of the patients was positive for helicobacter pylori and the other had secondary bone metastases. A dose study was performed of mri intravenous contrast medium enhancement of rheumatoid synovium in the knee, comparing the enhancement with gadoteridol at doses of 0. Remitting seronegative symmetrical synovitis with pitting edema is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema of the back of the hands andor feet, and a negative serum rheumatoid factor. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare syndrome. It is also known as brueghels syndrome and oral facial dystonia. Patients with idiopathic rs3pe showed an excellent response to low doses of corticosteroids compared to the poor response to rs3pe in association with neoplasia. To investigate whether the remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome may represent a paraneoplastic disorder in a significant percentage of cases. Therefore, we started treatment with vancomycin 1 gday and clindamycin 600 mgday, after which his fever rapidly remitted. Rs3pe syndrome developing during the course of probable. Remitting seronegative symmetrical synovitis with pitting edema or sometimes rs 3 pe is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema swelling of the back of the hands andor feet, and a negative serum rheumatoid factor. Meige syndrome nord national organization for rare. Rs3pe is the short form of remitting seronegative symmetrical synovitis with pitting edema.
What is shorthand of remitting seronegative symmetrical synovitis with pitting edema. Pdf remitting seronegative symmetrical synovitis with pitting. The exact pathophysiology of rs3pe remains unknown. Patients diagnosed with rs3pe syndrome at the medical college of wisconsin before 1995 were telephoned and asked about their rheumatologic course since initial diagnosis of rs3pe. Syndrome of remitting seronegative symmetrical synovitis with pitting oedema rs 3 pe ak kundu abstract rs 3 pe syndrome, often mimicking rheumatoid arthritis ra or polymyalgia rheumatica pmr, has puzzled the rheumatologists until latee80s. Frequent features frequent features the most common features in children with setd5 syndrome are also found in many other genetic. Asymmetrical involvement had also been reported, but unilateral presentation is very rare. If no underlying disorder can be identified idiopathic rs3pe, this entity has an excellent prognosis and responds well to treatment. Is remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome associated with hlaa2.
Brownsequard syndrome is caused by damage to one half of the spinal cord either left or right. It has not been reported as an association with rs3pe or lung cancer. Fault tolerant circuit for syndrome measurements for the 7,1,3 qec code using steane ancilla. Leprosy presenting as remitting seronegative symmetrical synovitis. Due to poor ergonomics and the sedentary nature of most jobs these days, upper crossed syndrome is amongst the most common musculoskeletal imbalances of the 21st century. Dystonia is a group of movement disorders that vary in their symptoms, causes, progression, and treatments.
Treatment with prednisone 15 mg daily was instituted. Patients show excellent response to low dose steroids with complete and sustained remissions. This unfortunate postural deviation is also known as. Hemophagocytic syndrome, hemophagocytic lymphohistiocytosis hlh, histiocytic erythrophagocytosis. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare inflammatory arthritis characterized by. Increased serum concentration of il6 has been observed in patients with rs3pe 2, 3, and therefore il6 inhibition with tocilizumab might constitute a novel strategy for treatment of rs3pe. The most common shorthand of remitting seronegative symmetrical synovitis with pitting edema is rs3pe. Intravenous mri contrast enhancement of inflammatory synovium. Eight elderly men and two elderly women presented with symmetrical polysynovitis of acute onset involving most of their appendicular joints and flexor digitorum tendons associated with pitting edema of the dorsum of both hands and both feet.
T he u ni ve rs i t y o f n e bra s ka m e di c a l c e nt e r, c e nt e r f or c ont i nui ng e duc a t i on d e s i gna t e s t hi s e nduri ng m a t e ri a l f or a. Rs3pe syndrome can be associated with both solid tumours like gastric, pancreatic and haematological malignancies like nonhodgkins lymphoma. Remitting seronegative symmetrical synovitis with pitting oedema rs3pe is a rare rheumatologic condition frequently missed but easily treated. Cells were then washed with pbs and incubated with 100 m cldu for 15 min.
Is remitting seronegative symmetrical synovitis with. Fault tolerant circuit for syndrome measurements for the 7,1,3 qec code using shor states. Remitting seronegative symmetrical synovitis with pitting edema rs3pe. Remitting seronegative symmetrical synovitis with pitting edema or rs3pe is a rare elderlyonset rheumatic syndrome. Upper crossed syndrome is an imbalance that occurs in the soft tissues of the thoracic and cervical areas of the body. When omd is combined with blepharospasm, it may be referred to as meiges syndrome named after henri meige, the french neurologist who first described the symptoms in detail in 1910. To investigate clinical differences in warm and cold complex regional pain syndrome crps phenotypes.
Out of all patients aged over 50 n 3,347, the rate of pmr was 0. The study of rs3pe syndrome has been mentioned in research publications which can be found using our bioinformatics tool below. Maroteaux lamy syndrome is an autosomal recessive disease caused by deficiency of the lysosomal enzyme nacetylgalactosamine 4sulfatase arylsulfatase b which is involved in glycosaminoglycan gag degradation 1, 2. To investigate whether the remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome may represent a pdf remitting seronegative symmetrical synovitis with pitting oedema syndrome, a rare inflammatory arthritis, commonly affects people in the older age group remitting seronegative symmetrical synovitis with pitting edema. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare. Pdf syndrome of remitting seronegative symmetrical. Sapho syndrome genetic and rare diseases information. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is an uncommon elderlyonset rheumatic disease characterized by tenosynovitis of both flexor and extensors tendons at the wrist and at the feet. Remitting seronegative symmetrical synovitis with pitting edema rs3pe is a rare but wellreported clinical entity. Another important characteristic of rs3pe syndrome is its association with. It is classically described as symmetrical involvement of both upper extremities. Rs3pe syndrome remitting symmetrical seronegative synovitis with pitting edema was first described by mc carthy in 1985. Bir olgu sunumu abst ract rs3pe remitting seronegative, symmetric synovitis with pitting edema is a benign, seronegative syndrome, with an.
Case 1 was a 74yearold woman with a 30year history of type 2 diabetes who presented with edema of the hands, low grade fever, and malaise, which developed 5 weeks. Objectiveto determine the clinical characteristics of patients with pure remitting seronegative symmetrical synovitis with pitting oedema rs3pe syndrome, and to investigate its relation with polymyalgia rheumatica pmr. The case presented is unique, combining a presenting picture of rs3pe syndrome with a. We included case reports and clinical trials which providing details regarding the clinical presentation, management and prognosis of patient s with newly or previously diagnosed rs3pe. Rs3pe typically involves the joints of the extremities. Rs3pe syndrome remitting seronegative symmetrical synovitis with pitting oedema occurs mostly in elderly people over 60 years of age and is demonstrable on the dorsum of the hands. The rs3pe syndrome remitting seronegative symmetrical synovitis with pitting edema is a manifestation of rheumatoid arthritis in the elderly with a good prognosis. Neurology is the official journal of the american academy of. Remitting seronegative symmetrical synovitis with pitting edema rs3pe syndrome was first reported by mccarty et al. Us was performed with a linear array transducer operating at 12 mhz.
The diagram opposite shows the effects of hemitransection of the cord on the right side of the individual. Miller, mckusick, and malvaux and report their findings in the medical literature in 1972. Isolated duplications of pafah1b1 have been associated with mild developmental delay and hypotonia, while isolated duplications. Mergers offer an example of such changes, when subgroups merger partners merge into a common, superordinate group post.
751 640 502 840 1420 860 259 1159 578 837 1101 875 231 164 918 783 267 1153 1337 132 1185 461 648 216 1158 74 190 865 1412 888 792 1480 1395 1367 808 975 1382 1405 709