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A Case of Pseudo Rheumatoid Arthritis Mimicking Remitting Seronegative Symmetrical Synovitis With Pitting Edema Rs3pe Syndrome

This case report discusses a 79-year-old woman diagnosed with pseudo-rheumatoid arthritis (pseudo-RA) type Calcium Pyrophosphate Deposition Disease (CPPD), which mimicked Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) syndrome. Initial treatment with corticosteroids showed an incomplete response, leading to further investigation that revealed chondrocalcinosis in multiple joints. The findings highlight the importance of considering pseudo-RA type CPPD in the differential diagnosis of RS3PE syndrome and the value of radiographic assessment in such cases.
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0% found this document useful (0 votes)
40 views4 pages

A Case of Pseudo Rheumatoid Arthritis Mimicking Remitting Seronegative Symmetrical Synovitis With Pitting Edema Rs3pe Syndrome

This case report discusses a 79-year-old woman diagnosed with pseudo-rheumatoid arthritis (pseudo-RA) type Calcium Pyrophosphate Deposition Disease (CPPD), which mimicked Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) syndrome. Initial treatment with corticosteroids showed an incomplete response, leading to further investigation that revealed chondrocalcinosis in multiple joints. The findings highlight the importance of considering pseudo-RA type CPPD in the differential diagnosis of RS3PE syndrome and the value of radiographic assessment in such cases.
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Kato K, et al.

, HSOA J Gerontol Geriatr Med 2025, 11: 245


DOI: 10.24966/GGM-8662/100245

HSOA Journal of
Gerontology and Geriatric Medicine
Case report

A Case of Pseudo-Rheumatoid Keywords: Chondrocalcinosis in multiple joints; Pseudo-rheumatoid


arthritis type CPPD; RS3PE syndrome
Arthritis Mimicking Remitting
Seronegative Symmetrical Introduction
RS3PE syndrome, first described by McCarty et al., in 1985, pres-
Synovitis with Pitting Edema ents with dramatic symmetrical distal synovitis and pitting edema of
the hands and/or feet [1]. The condition characteristically demon-
(RS3PE) Syndrome strates a rapid and sustained response to low-dose corticosteroids.
Our case initially presented with clinical features highly suggestive
Kokoro Kato1, Yoshiki Umezawa2, Yasushi Imai3, Kanichi Asai3, of RS3PE syndrome, but the incomplete response to corticosteroids
Yoshinori Noguchi3* and Yasuhiro Osugi4 prompted further investigation.
Department of General Practice, Fujita Health University Okazaki Medical
Case Presentation
1

Center, Okazaki City, Aichi, Japan


2
Dozen Hospital, Taito City, Tokyo, Japan A 79-year-old woman presented to an outpatient clinic with a
Department of General and Family Medicine, Toyota Regional Medical
3 1-week history of generalized pain. She had a 2-month history of
Center, Toyota City, Aichi, Japan symmetrical digital edema involving the MCP and PIP joints of both
hands and a 1-month history of bilateral ankle arthritis. Her medical
4
Department of Community based Medicine, Fujita Health University School
of Medicine, Toyoake City, Aichi, Japan history included left breast cancer status post resection and demen-
tia. Her family physician detected anemia and elevated inflammatory
markers and referred her to our outpatient clinic in the general med-
Abstract icine department. On examination, her vital signs were as follows:
We report a Calcium Pyrophosphate Deposition Disease (CPPD) blood pressure 128/78 mmHg, pulse rate 91 beats/min, respiratory
case confusingly similar to Remitting Seronegative Symmetrical Sy- rate 20 breaths/min, and oxygen saturation 99% (room air). She was
novitis with Pitting Edema (RS3PE) syndrome. An elderly woman afebrile, awake, and alert. Physical examination revealed bilateral pit-
presented with generalized pain, symmetrical digital edema of Meta- ting edema of the MCP and PIP joints and the dorsal surface of both
carpophalangeal (MCP) and Proximal Interphalangeal (PIP) joints, hands, along with swelling of both ankle joints. Tenderness was nota-
and bilateral ankle arthritis. Initial diagnosis of RS3PE syndrome ble on the right index finger involving the MCP joint, the right elbow
was reconsidered due to atypical steroid response. Radiographic joint, and both ankle joints. There was no evidence of thoracoabdom-
examination revealed chondrocalcinosis in multiple joints, leading to
inal abnormalities, spinal tenderness, or skin findings suggestive of
a final diagnosis of pseudo-rheumatoid arthritis (pseudo-RA) type
psoriasis or other dermatological conditions.
CPPD. This case highlights that pseudo-RA type CPPD can pres-
ent with clinical features remarkably similar to RS3PE syndrome, Investigations
underscoring the importance of considering pseudo-RA type CPPD
in the differential diagnosis of RS3PE syndrome and emphasizing Laboratory results were as follows: white blood cells, 5.3 × 10³/µL
the value of radiographic assessment when evaluating RS3PE-like
(reference range: 4.0-10.0 × 10³/µL); hemoglobin, 8.0 g/dL (reference
presentations.
range: 12.0-16.0 g/dL); mean corpuscular volume, 85 fL (reference
range: 80-100 fL), suggesting normocytic anemia; platelets, 54.8 ×
10⁴/µL (reference range: 15.0-35.0 × 10⁴/µL); blood urea nitrogen,
21.8 mg/dL (reference range: 8.0-20.0 mg/dL); creatinine, 0.84 mg/
*Corresponding author: Yoshinori Noguchi, Department of General and Family
Medicine, Toyota Regional Medical Center, 3-30-1 Nishiyama-cho, Toyota City,
dL (reference range: 0.5-1.0 mg/dL); and C-Reactive Protein (CRP)
Aichi, 471-0062, Japan, E-mail: [email protected] level, 5.83 mg/dL (reference range: <0.3 mg/dL). The Erythrocyte
Sedimentation Rate (ESR) was 143 mm/hr (reference range: 3.0-15.0
Citation: Kato K, Umezawa Y, Imai Y, Asai K, Noguchi Y, et al. (2025) A Case mm/hr), and Antinuclear Antibody (ANA) titer was 1:40 (homoge-
of Pseudo-Rheumatoid Arthritis Mimicking Remitting Seronegative Symmetrical
neous and speckled patterns). The Rheumatoid Factor (RF), anti-cy-
Synovitis with Pitting Edema (RS3PE) Syndrome. HSOA J Gerontol Geriatr Med
11: 245. clic citrullinated peptide antibodies, Proteinase-3-Antineutrophil
Cytoplasmic Antibodies (PR3-ANCA), Myeloperoxidase-Antineu-
Received: March 11, 2025; Accepted: March 13, 2025; Published: March 20, trophil Cytoplasmic Antibodies (MPO-ANCA), and blood culture
2025 were negative. Her thyroid function and serum ferritin levels were
Copyright: © 2025 Kato K, et al. This is an open-access article distributed under
also normal.
the terms of the Creative Commons Attribution License, which permits unrestrict-
ed use, distribution, and reproduction in any medium, provided the original author The findings of symmetrical pitting edema of predominantly pe-
and source are credited. ripheral joints with elevated acute phase reactants (ESR, CRP) in an
Citation: Kato K, Umezawa Y, Imai Y, Asai K, Noguchi Y, et al. (2025) A Case of Pseudo-Rheumatoid Arthritis Mimicking Remitting Seronegative Symmetrical Synovitis
with Pitting Edema (RS3PE) Syndrome. HSOA J Gerontol Geriatr Med 11: 245.

• Page 2 of 4 •

older adult led to an initial impression of RS3PE syndrome, based on Differential Diagnosis
the McCarty criteria [1] which include: 1) bilateral pitting edema of
the hands, 2) sudden onset of polyarthritis, 3) age >50 years and 4) Differential diagnoses of RS3PE syndrome
seronegative RF. We tested for ANCA due to the multisystem nature RS3PE syndrome is a rare inflammatory arthritis marked by sym-
of her presentation and to rule out vasculitis. We then began treating metrical distal synovitis, pitting edema of the hands and feet, and ab-
her with oral prednisolone (10 mg/day) with immediate improvement sence of RF. This condition is relatively common in older men. Treat-
of the bilateral hand edema. However, arthritis symptoms in the right ment with 10-20 mg/day of prednisolone generally results in rapid
MCP joint, right wrist, and both ankle joints became more prominent improvement and a good prognosis [2]. Diseases with a similar pre-
after a 10-day treatment course. sentation to RS3PE syndrome include polymyalgia rheumatica, El-
Because the response to low-dose steroids was not typical for derly-Onset Rheumatoid Arthritis (EORA), Calcium Pyrophosphate
RS3PE syndrome, we reviewed the diagnosis. A plain radiograph of Deposition Disease (CPPD), vasculitis, infectious diseases such as
the right hand revealed chondrocalcinosis at the MCP joint of the sec- infective endocarditis, paraneoplastic syndromes, fibromyalgia, pso-
ond and third fingers and the wrist joint, with no erosive changes sug- riatic arthritis, ankylosing spondylitis and hypothyroidism. In these
gestive of Rheumatoid Arthritis (RA) (Figure 1). These radiographic differential diagnoses, the characteristics of high acute phase reac-
findings were not obtained prior to steroid initiation as the initial clin- tants, RF negativity, and systemic manifestations resembling RS3PE
ical presentation strongly suggested RS3PE syndrome, which typi- led us to focus on EORA.
cally responds dramatically to low-dose steroids. These symptomatic
RA generally develops at 30-50 years of age, but if it develops at
characteristics, laboratory and imaging findings, and clinical course
an older age, it is called EORA. EORA has a lower frequency of pos-
were consistent with pseudo-RA CPPD, which was our final diagno-
itive RF rate than younger-onset RA (80%), and both small and large
sis. Although synovial fluid analysis would have been the gold stan-
joints are commonly affected in EORA. This disease often resembles
dard for confirming CPPD diagnosis, the characteristic radiographic
RS3PE syndrome, with peripheral edema and relatively high levels of
findings of chondrocalcinosis in conjunction with the clinical presen-
acute phase reactants. As a feature of the imaging examination, plain
tation were considered sufficient for diagnosis in this case.
radiographs can confirm bone erosion of affected joints [3]. While
evaluating for bone erosion of EORA, a plain radiograph of the right
hand revealed synovial calcification in multiple joints, which were
distinctive of pseudo-RA CPPD (Type B CPPD) [4].

CPPD can be classified into six types, from Type A to Type F.


Type A is the so-called pseudogout, which is the most common pat-
tern in symptomatic CPPD. This feature is marked by acute or sub-
acute arthritic attacks lasting approximately 1 day to 4 weeks. One
or a few arthritis symptoms may occur, but many of these symptoms
are self-limiting, and the pain is somewhat less severe than that in
gout. Knee joints are most commonly affected, and medical disease
or trauma may be the triggering factor. Type B CPPD has clinical
features that are completely different from pseudogout; instead, it
more closely resembles RA, leading to it being termed pseudo-RA.
Approximately 5% of patients with Type B CPPD have multiple joint
involvement with subacute attacks lasting 4 weeks to several months.
Nonspecific symptoms, such as morning stiffness and general fatigue,
are common. Clinically, it closely resembles RA, with synovial thick-
ening, localized edema, arthritis, and abnormal CRP or ESR [5].

Psoriatic arthritis was considered in the differential diagnosis due


to the presence of dactylitis-like findings. However, the absence of
psoriatic skin lesions, nail changes, and the characteristic radiograph-
ic findings of CPPD instead of the pencil-in-cup deformities typically
seen in psoriatic arthritis made this diagnosis less likely.

Outcome and Follow-Up


Oral celecoxib (200 mg/day) treatment was initiated. As a result,
the arthritic findings in the right fingers, wrist, and ankle joints, and
the inflammatory response in blood tests improved within 1 week.
No relapse was observed after the discontinuation of celecoxib. The
Figure 1: Plain radiography of the right hand showing calcified lesions at normocytic anemia was thoroughly evaluated, with no evidence of
the MP joint of the second and third fingers, and the wrist joint (arrows),
with no supportive findings of rheumatoid arthritis. Additionally, mild os- hemolysis, gastrointestinal bleeding, or nutritional deficiencies, sug-
teoarthritis changes are observed at the DIP and PIP joints of the third, gesting it was anemia of chronic inflammation related to the inflam-
fourth, and fifth fingers as age-related changes. matory arthritis. The good response to Nonsteroidal Anti-Inflamma-
tory Drugs (NSAIDs) without relapse was compatible with CPPD.

HSOA J Gerontol Geriatr Med ISSN: 2381-8662, Open Access Journal Volume 11 • Issue 1• 100245
DOI: 10.24966/GGM-8662/100245
Citation: Kato K, Umezawa Y, Imai Y, Asai K, Noguchi Y, et al. (2025) A Case of Pseudo-Rheumatoid Arthritis Mimicking Remitting Seronegative Symmetrical Synovitis
with Pitting Edema (RS3PE) Syndrome. HSOA J Gerontol Geriatr Med 11: 245.

• Page 3 of 4 •

NSAIDs were chosen as the treatment for pseudo-RA CPPD based on While synovial fluid analysis remains the gold standard for di-
evidence suggesting that they are effective for symptomatic relief in agnosing CPPD, characteristic radiographic findings of chondro-
CPPD, particularly for chronic forms of the disease [6]. calcinosis in conjunction with appropriate clinical presentation can
support the diagnosis. NSAIDs are typically effective in managing
Discussion pseudo-RA type CPPD, whereas RS3PE syndrome generally demon-
strates a dramatic and sustained response to low-dose corticosteroids.
The literature describes several cases where CPPD has mimicked
various inflammatory rheumatic diseases. Kano et al. reported a case Ethics
of pseudo-RA CPPD that exhibited polyarthritis and calcified lesions
in multiple joints similar to our case, but peripheral pitting edema Written informed consent was obtained from the patient for publi-
was absent [7]. Thus, the prominent presence of peripheral edema in cation of this case report and accompanying images.
our case represents an unusual clinical presentation for pseudo-RA
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