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Remitting Seronegative Symmetrical Synovitis With Pitting Edema

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Remitting seronegative symmetrical synovitis with pitting edema is also known as puffy edematous hand syndrome. The below article outlines this rare disease.

Written by

Dr. Chandhni. S

Medically reviewed by

Dr. Anuj Gupta

Published At April 27, 2023
Reviewed AtApril 27, 2023

Introduction

Remitting seronegative symmetrical synovitis with pitting edema, abbreviated RS3PE is a rare rheumatological disease which was earlier thought to be a variant of rheumatoid arthritis but was later recognized as a separate clinical entity. It is a condition which is difficult to diagnose and is often a topic of debate between rheumatologists if it should be called a disease or a syndrome.

Rheumatic disease refers to a bunch of conditions like arthritis and conditions affecting the muscles, tendons, bones and joints. Degeneration of the protective cartilage lining in the joint leads to severe joint pain in arthritis. Rheumatoid arthritis, on the other hand, is the most common type of arthritis which is an autoimmune disorder where the body’s immune system attacks the body’s own healthy cells leading to inflammation and pain in the joints. It was Mc Carty and his colleagues in 1985 who described 10 cases of a distinct form of rheumatoid arthritis that first appeared during old age and exhibited excellent response and prognosis following treatment.

What Is Remitting Seronegative Symmetrical Synovitis With Pitting Edema?

The name of this disorder encompasses its most striking features. Let us consider each of these terms independently to decode its definition.

The term remitting refers to decreasing or subsiding symptoms. Usually the blood of individuals with rheumatoid arthritis contains proteins called the rheumatoid factor (RF) and anti-CCP (autoantibodies) which aid in the diagnosis. Anti-CCP antibodies appear earlier and the anti-CCP test is more sensitive.

In RS3PE, these proteins are absent and hence the term seronegative is used. Seropositivity is generally associated with more severity of the disease. The synovial membrane consists of a thin layer of connective tissue that lines a joint; an inflammation of this layer is termed synovitis.

Pitting edema refers to a swelling typically in the legs or arms characterized by a dimple or depression known as pitting, following the application of finger pressure. To summarize, RS3PE is a benign condition with subsiding symptoms, is seronegative with symmetrical synovitis and pitting edema.

Who Is Affected by RS3PE?

This disease affects the geriatric age group. This does not automatically mean that the young are not affected; the originally reported cases of the disease showed a male predisposition with the affected male is to female ratio at 4:1, but an increasing number of affected females have been reported, challenging this belief.

What Are the Causes of RS3PE?

The exact cause remains unknown with no identifiable triggers in most cases. Many idiopathic (without an apparent cause) cases of RS3PE have been reported.

Some factors such as environmental factors and infection with certain microbes have been postulated to be of relevance in the etiopathogenesis of RS3PE. Infectious agents like Mycoplasma pneumoniae, Streptobacillus moniliformis, parvovirus, etc have been implicated as associated factors.

Frequent association of RS3PE with neoplasms have led some researchers to theorize that it is part of a paraneoplastic syndrome. RS3PE has been described in association with various cancers such as prostate, ovary, breast, lung, endometrium, bladder, liver, gastrointestinal cancer, etc. Some other cases in relation with T-cell lymphoma, non-Hodgkin lymphoma, ankylosing spondylitis, polymyalgia rheumatica and myelodysplasia syndrome, etc. have also been reported. Hence it is advisable to check for an underlying cancer as a precaution in RS3PE.

Additionally, factors such as vascular endothelial growth factor (VEGF) thought to increase vascular permeability; elevated IL-6 (interleukin 6) in the synovial fluid; and an established association with Human Leukocyte Antigen (HLA) - HLA-B7, HLA-CW7, HLA-A2, HLA-DQW2 are all considered to be contributing factors in the pathogenesis of RS3PE.

What Are the Symptoms Of RS3PE?

RS3PE is characterized by a sudden/acute onset of inflammation of joints and synovitis of a number of small joints in the wrists and hands. There is symmetrical pitting edema (swelling) affecting the dorsum of the hands (giving a ‘boxing glove’ appearance), feet, wrists and ankles. Skin over the involved joints is stretched and warm to touch, loss of wrinkles is noted in the elderly. The duration of the illness is short and usually remitting within 18 months following the onset. The classical description of the disease describes bilateral involvement, however, some cases with unilateral involvement have been reported and presently unilateral RS3PE is well recognized. Distal joints such as wrists and ankles are predominantly involved, additionally, stiffness in joints like the metacarpophalangeal, proximal interphalangeal joints, shoulders, knees, and elbows may also be present. Inflammation and stiffness in joints causes mild-to-severe joint pain and limitation of joint movement. The paraneoplastic form of RS3PE may show systemic symptoms such as fever, loss of appetite and weight loss.

How Is RS3PE Diagnosed?

Diagnosis is largely based on the history and clinical presentation. Diagnostic criteria used a guide that includes:

  • Sudden onset

  • Age over 50 years

  • Pitting edema involving all four limbs

  • Seronegativity

Investigations that can be performed include:

  1. Radiological examination reveals no erosive changes typical of arthritis in the joints.

  2. Imaging with ultrasound scan reveals symmetrical synovitis. It demonstrates subcutaneous edema with dilated lymphatics, peritendinous thickening with hypervascularization that is more prominent in the extensor tendons, etc. Contrast-enhanced Doppler ultrasound and color Doppler ultrasound are more sensitive than conventional ultrasonography.

  3. MRI scan also reveals subcutaneous edema maximal at the mid-dorsum. There is enhancement of tendon sheaths and joint effusions.

  4. Serology is negative for RF and anti-CCP antibodies. Matrix Metalloproteinase-3 (MMP-3) is a potential positive serum marker especially in paraneoplastic cases.

  5. Acute elevation of C-reactive protein which is a marker of inflammation.

  6. ESR (erythrocyte sedimentation rate), also a marker of inflammation, is elevated.

What Are the Possible Differential Diagnoses and Complications of RS3PE?

RS3PE may be confused with a number of other diseases like polymyalgia rheumatica (also seronegative, presents is elderly and responds to steroids), rheumatoid arthritis (can be differentiated by seropositive test results), severe hypothyroidism with pretibial myxedema, spondyloarthropathy, reflex sympathetic dystrophy, etc. due to similarities in presentation.

Possible complications though rare may include carpal tunnel syndrome, pleural effusions and cardiac complications.

What Is the Treatment for RS3PE?

Treatment is pharmacological, RS3PE responds dramatically to steroid medication such as prednisolone at low doses (5 mg to 20 mg) and in a short time. Non-steroidal anti-inflammatory drugs (NSAIDs) when prescribed may be advantageous. In individuals with an underlying cancer, treatment of the cancer can lead to remission. Paraneoplastic RS3PE does not respond to steroid medication.

Conclusion

The syndrome of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare elderly-onset arthritis variant, now recognized as an individual disorder,which is acute and involves complete resolution of symptoms following appropriate treatment with steroid and NSAID medication. It is missed due to a lack of knowledge and distinct diagnostic criteria since a wide variety of diseases show similarity in signs and symptoms. About 50 % of RS3PE cases can be associated with some types of cancers and hence it is wise to screen for cancer when a diagnosis of RS3PE is made.

Frequently Asked Questions

1.

What Is the Outlook for rs3pe Syndrome?

The prognosis for RS3PE syndrome varies depending on individual cases, with some individuals experiencing improvement and others facing more prolonged challenges. Seeking guidance from a healthcare professional is crucial to understanding the specific outlook for this condition.

2.

Is Spontaneous Resolution Common in Seronegative Arthritis?

While spontaneous resolution is observed in certain instances of seronegative arthritis, the course of the disease can differ significantly among affected individuals. Factors such as the underlying cause and the body's response to treatment play a role in determining outcomes.

3.

Is RSSS Considered an Autoimmune Disorder?

RSSS is regarded by some as an autoimmune disorder, where the immune system mistakenly attacks healthy tissues, leading to inflammation. This classification underscores the importance of appropriate medical management and interventions to address the immune system's role in the condition.

4.

What Conditions Should Be Considered in the Differential Diagnosis of Rsss?

In the differential diagnosis of RSSS, healthcare professionals consider and rule out other inflammatory conditions that may present with similar symptoms. This process is essential to accurately identifying and addressing the specific underlying cause of the symptoms.

5.

Can Remitting Seronegative Symmetrical Synovitis Become a Chronic Condition?

Remitting Seronegative Symmetrical Synovitis has the potential to become a chronic condition in certain cases, highlighting the importance of ongoing medical monitoring and management to mitigate the long-term impact on joint health.

6.

Can Remitting Seronegative Symmetrical Synovitis Become a Chronic Condition?

Remitting Seronegative Symmetrical Synovitis has the potential to become a chronic condition in certain cases, highlighting the importance of ongoing medical monitoring and management to mitigate the long-term impact on joint health.

7.

What Are the Four Classifications of Seronegative Arthritis?

The four main classifications of seronegative arthritis include ankylosing spondylitis, psoriatic arthritis, reactive arthritis, and enteropathic arthritis. Each of these conditions presents unique challenges and symptoms, requiring tailored approaches to treatment.

8.

How Prevalent Is Remitting Seronegative Symmetrical Synovitis in the Population?

The prevalence of Remitting Seronegative Symmetrical Synovitis in the population is not extensively documented, emphasizing the need for further research to understand the frequency and distribution of this condition among different demographic groups.

9.

Which Dietary Choices Are Recommended for Managing Remitting Seronegative Symmetrical Synovitis?

Dietary recommendations for managing Remitting Seronegative Symmetrical Synovitis may involve incorporating anti-inflammatory foods into one's diet. However, individual dietary plans should be discussed with healthcare professionals to ensure they align with the overall treatment strategy.

10.

Is There a Cure for Inflammation of the Synovial Membrane?

There is no cure for inflammation of the synovial membrane. Treatment focuses on managing symptoms, reducing inflammation, and improving joint function to enhance the individual's quality of life.

11.

Is It Possible to Engage in Normal Activities, Such as Walking, With Synovitis?

While engaging in normal activities like walking may be possible with appropriate management of synovitis, it is essential to work closely with healthcare professionals to develop a personalized care plan that addresses the specific needs and limitations of the individual.

12.

Can Synovitis Develop at Any Point in a Person’s Life?

Synovitis can potentially develop at any point in a person's life, triggered by various factors such as injury, infection, or underlying inflammatory conditions. The onset of synovitis is not necessarily restricted to a specific age range.

13.

Are There Specific Fruits That Promote the Production of Synovial Fluid?

While specific fruits are not known to directly promote the production of synovial fluid, maintaining a well-balanced and anti-inflammatory diet can contribute to overall joint health. Fruits rich in antioxidants, such as berries and citrus fruits, may indirectly support joint function.

14.

What Are Effective Methods for Alleviating Pain Associated With Synovitis?

Effective methods for alleviating pain associated with synovitis often include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroid injections, physical therapy, and rest. Consultation with a healthcare professional is essential to determine the most appropriate pain management approach based on the individual's specific situation.

15.

Does Synovitis Typically Resolve Without Intervention?

Synovitis may not always resolve without intervention, particularly if it is associated with an underlying condition such as rheumatoid arthritis or other inflammatory joint disorders. Timely medical intervention is crucial to managing symptoms and preventing potential complications.

16.

Is Synovitis Considered a Rare Medical Condition?

Synovitis is not considered a rare medical condition. It can occur in various joint-related issues, ranging from acute injuries to chronic inflammatory conditions. The prevalence depends on the underlying causes, and it is relatively common in the context of inflammatory arthritis and other joint disorders.
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Dr. Anuj Gupta
Dr. Anuj Gupta

Spine Surgery

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