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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 28  |  Issue : 3  |  Page : 188-191

Concurrence of pigmented villonodular synovitis with calcium pyrophosphate deposition in a postacute stroke patient


1 Department of Physical and Rehabilitation Medicine, MacKay Memorial Hospital, Taipei, Taiwan
2 Department of Physical and Rehabilitation Medicine, Asia University Hospital, Taichung, Taiwan
3 Department of Radiology, Cheng Hsin General Hospital, Taipei, Taiwan
4 Department of Orthopaedics Surgery, MacKay Memorial Hospital, Taipei, Taiwan

Date of Submission07-Nov-2019
Date of Decision15-Dec-2019
Date of Acceptance18-Dec-2019
Date of Web Publication26-Mar-2020

Correspondence Address:
Dr. Shiau-Fu Hsieh
No. 92, Section 2, Chung-Shan North Road, Taipei
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JMU.JMU_107_19

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  Abstract 


Pigmented villonodular synovitis (PVNS) is a rare synovial proliferative disease featuring hemosiderin deposits. Calcium pyrophosphate deposition (CPPD) is a crystal-induced inflammatory arthritis common in the elderly. We reported the case of a 78-year-old male who was under stroke rehabilitation when acute inflammatory and hemorrhagic knee arthritis of his paretic lower limb occurred. CPPD was proven by synovial analysis. Ultrasonography showed widespread synovial nodular lesions in the affected knee and helped guiding difficult arthrocentesis. These led to a rapid diagnosis of PVNS with magnetic resonance imaging. In elderly stroke patients, knee pain, being a common complaint, warrants a careful diagnosis including adequate imaging. This case demonstrates that ultrasonography is an accessible and useful diagnostic tool.

Keywords: Calcium pyrophosphate deposition, knee, magnetic resonance imaging, pigmented villonodular synovitis, ultrasonography


How to cite this article:
Hsieh SF, Wu SY, Hung YC, Wang GS. Concurrence of pigmented villonodular synovitis with calcium pyrophosphate deposition in a postacute stroke patient. J Med Ultrasound 2020;28:188-91

How to cite this URL:
Hsieh SF, Wu SY, Hung YC, Wang GS. Concurrence of pigmented villonodular synovitis with calcium pyrophosphate deposition in a postacute stroke patient. J Med Ultrasound [serial online] 2020 [cited 2020 Dec 5];28:188-91. Available from: http://www.jmuonline.org/text.asp?2020/28/3/188/281448




  Introduction Top


Pigmented villonodular synovitis (PVNS) is a benign rare disease involving the proliferation of joint synovium.[1] It typically presents as joint swelling, pain, and hemarthrosis. Calcium pyrophosphate deposition (CPPD) is a common inflammatory arthritis in the elderly due to calcium pyrophosphate (CPP) dihydrate crystal deposits.[2] Like PVNS, its most frequently involved joint is the knee.[3],[4]

We present the case of a 78-year-old male, who was taking dual-antiplatelet treatment after stroke. He presented with acute severe knee pain at his paretic leg during inpatient rehabilitation. Through evidences from arthrocentesis, well-correlated musculoskeletal ultrasound (US), and magnetic resonance images, a rare diagnosis, coexistence of PVNS and CPPD, is made apart from other common causes of knee pain in this time frame.


  Case Report Top


A 78-year-old male had acute pain, swelling, and heat at the knee of his paralyzed left lower limb after 1 month of inpatient rehabilitation for a new ischemic stroke at his right periventricular region. He also started dual-antiplatelet (aspirin and clopidogrel) treatment because of stroke recurrence. The patient had a history of hypertension, dyslipidemia, diabetes, gout, and chronic kidney disease but denied previous joint problems or recent trauma.

Acute severe pain, swelling, redness, and heat without ecchymosis of his left paretic knee led to the discontinuation of stroke rehabilitation. The passive and active ranges of the knee were limited. No particular point tenderness was identified. Neither valgus nor varus stress test was positive. Anterior and posterior drawer tests were not performed due to pain. No sign of new neurologic deficit was noticed. Laboratory tests showed mild leukocytosis (leukocyte = 11.90 × 103/μL) and high C-reactive protein (=12.91 mg/dL) level. A 0.2 ng/mL of procalcitonin level was within the 0.1–0.25 ng/mL cutoff level. The levels of antinuclear antibody, rheumatoid factor, and uric acid were within the normal range. Repeated US-guided knee arthrocentesis yielded massive bloody joint fluid. These two synovial fluid analyses had similar profiles. The second analysis showed that leukocyte was 17,600/mm3 (neutrophil = 80% and lymphocyte = 4%). Synovial fluid cultures were negative. CPP crystal was found in one of the two samples.

Knee radiography [Figure 1] identified soft-tissue swelling, osteophytes, joint space narrowing, and chondrocalcinosis at medial joint space. Furthermore, bone erosions at lateral femur and lateral border of the medial femur condyle were worth noticing. US examinations [Figure 2] provided important diagnostic clues including interspersed fluid and proliferative synovium of multiple, villus-like, heterogeneous projections with hyperemia at the suprapatellar bursa and knee joint. A thin layer of hyperechoic signal within the intercondylar cartilage correlated with chondrocalcinosis on radiography. US allowed for evaluation of major knee structures, including unremarkable patella tendon, menisci, and collateral ligaments, aside from a synovial plica.
Figure 1: X-ray of the patient's affected left knee, (a) Coronal view. Joint space narrowing at the lateral aspect of the knee. Chondrocalcinosis at the medial joint space (arrowhead). Bone erosions were discovered at the lateral femur and the lateral border of medial femur condyle. (arrow), (b) Sagittal view. Increased suprapatellar bursal effusions and periarticular soft-tissue and subcutaneous swelling were seen. Calcification in popliteal artery identified

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Figure 2: Ultrasound and magnetic resonance images of the patient's affected left knee at the similar level. (a) Magnetic resonance imaging proton density fat-suppressed sequence (sagittal) with high-signal intensity synovial proliferations with obscured internal contents (asterisk), (b) Ultrasound scan showing villus-like heterogeneous nodules. Septum-like structure separating the nodules (asterisk), possibly an embryologic remnant, a synovial plicae, (c) T2-weighted fat-suppressed gradient echo sequence (coronal) showing exaggerated low-signal intensity contents of the proliferated synovial nodules. The signal change from Figure 2a-c is characteristic of the hemosiderin components of pigmented villonodular synovitis in magnetic resonance imaging and is termed as “Blooming effect,” (d) Ultrasound scan at suprapatellar region along the short axis (almost corresponding to the yellow dash box) in (c) also reveals proliferated synovial tissue (asterisk) with fluid collection. Chondrocalcinosis (arrowhead) of the calcium pyrophosphate deposition is noted at the femoral condyle and in the X-ray. The arrow indicates the “cartilage interface sign” or “the double-contour sign of gouty arthritis” as one of the patient's past history

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Magnetic resonance imaging (MRI) [Figure 2] confirmed PVNS as the most possible cause of proliferative synovium and hemarthrosis. Proton density fat-suppressed sequence outlined multifocal low-signal intensity nodules in the knee joint, suprapatellar bursa, and popliteal fossa with bone erosions. These structures showed further decreased signal intensity under T2-weighted fat-suppressed gradient echo sequence, compatible with a characteristic MRI “blooming artifact” of hemosiderin.

Antibiotics were temporarily used early in the course to cover possible septic arthritis. Colchicine and oral prednisolone were started for acute CPP arthritis later. Symptoms and laboratory findings improved dramatically thereafter. Repeated arthrocentesis also helped relieving knee swelling. The patient refused surgical excision or adjuvant intra-articular instillation of radioactive isotopes or adjuvant external beam radiotherapy for diffuse PVNS of the knee due to his high anesthetic risk and the possibility of recurrence. Watchful waiting with periodic follow-up was decided as a patient-centered treatment plan. His knee remained symptom free 9 months later while he remained wheelchair bound due to poor stroke recovery.


  Discussion Top


We reported the coexistence of PVNS and CPPD causing acute knee arthritis with hemarthrosis in a postacute stroke patient. Poststroke knee pain is common and osteoarthritis is the most frequent diagnosis.[5] However, in this case, disproportionate inflammation and atraumatic hemarthrosis raised our suspicions for atypical causes. Blood and joint fluid analyses confirmed CPP existence and excluded septic arthritis, gouty arthritis, rheumatoid arthritis, or seronegative arthritis. Radiography revealed characteristic CPPD and excluded late-stage rheumatoid arthritis and gouty arthritis. Musculoskeletal ultrasonography showed finding of hemarthrosis and synovial proliferation and led to the early confirmative diagnosis of PVNS by MRI which is the gold standard diagnostic tool. Differential diagnoses of acute atraumatic knee effusions are summarized in [Table 1].
Table 1: Diagnostic features of acute atraumatic knee effusions

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PVNS is a rare benign proliferative disease of the synovium with hemosiderin deposition. Knee joint is most frequently affected, followed by hip, ankle, shoulder, and elbow.[3],[4] Diagnosis may be difficult and late as clinical manifestations are nonspecific, including joint pain, warmth, swelling, and limitation of mobility. PVNS is classified into localized type and diffuse type.[1] Hemarthrosis is a feature of PVNS, especially in its diffuse type. The multinodular thickening of synovium is infiltrated with synovial cells, macrophages, histiocytes, giant cells, and plasma cells with characteristic granular hemosiderin deposits in phagocytic cells microscopically.[6] Bone erosion is possible in the advanced stage.

The US and MRI findings of PVNS in this case correlate well. In PVNS, US can be used to (1) provide clues to support advanced imaging such as MRI, (2) exclude other conditions, (3) monitor disease activity through change of synovium and effusion, (4) detect early osteochondral change, and (5) guide knee arthrocentesis and focus on invasive treatment in complicated cases with indications. US could demonstrate joint effusion and proliferative synovium of PVNS, but MRI remains the gold standard of imaging diagnosis. In MRI, the proliferative synovium has low signal in T1 and T2 sequences. In gradient echo sequence, scattered hemosiderin granules in the synovium show further decrease in signal intensity due to the magnetic susceptibility of hemosiderin.[4] This is called the “blooming effect” and aids in the diagnosis of PVNS in MRI. Imaging features of PVNS and a summary of other causes of atraumatic hemarthrosis are listed [Table 2]. Another common diagnosis of hemarthrosis is hemophilia. Recent studies had advocated US scoring systems be used as a part of comprehensive periodic monitoring.[7] The essential US screening items are similar for hemophilia and PVNS. Both diseases would cause proliferating synovial tissue, but currently, we are not certain if there is any specific US sign to differentiate these two diseases.
Table 2: Ultrasonography and magnetic resonance imaging findings of pigmented villonodular synovitis and differential diagnosis of atraumatic hemarthrosis

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CPPD features intra-articular CPP dihydrate deposits in cartilage, synovium, joint capsule, and ligaments.[2] It is the third most common inflammatory arthritis and a major cause of acute monoarticular arthritis in the elderly. Synovial fluid CPPD crystal showing weak-positive birefringence is diagnostic. Characteristic chondrocalcinosis may be demonstrated by US and radiography but is mostly nonspecific [Figure 2].[8]

Both PVNS and CPPD may attribute to the clinical manifestations of this case. Dual-antiplatelet treatment may be another possible contributor of hemarthrosis. To our understanding, this is the first case reported in the English literature of concurrent PVNS and CPPD and the first report discussing the image features of PVNS under US and MRI.

Ethical statement

This study was approved by IRB of Mackay Memorial Hospital (approved no. 19MMHIS150e obtained on Sep. 16th, 2019) and informed consent was waived by IRB.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Myers BW, Masi AT. Pigmented villonodular synovitis and tenosynovitis: A clinical epidemiologic study of 166 cases and literature review. Medicine (Baltimore) 1980;59:223-38.  Back to cited text no. 1
    
2.
Ferrone C, Andracco R, Cimmino MA. Calcium pyrophosphate deposition disease: Clinical manifestations. Reumatismo 2012;63:246-52.  Back to cited text no. 2
    
3.
Al-Nakshabandi NA, Ryan AG, Choudur H, Torreggiani W, Nicoloau S, Munk PL, et al. Pigmented villonodular synovitis. Clin Radiol 2004;59:414-20.  Back to cited text no. 3
    
4.
Bravo SM, Winalski CS, Weissman BN. Pigmented villonodular synovitis. Radiol Clin North Am 1996;34:311-26, x-xi.  Back to cited text no. 4
    
5.
Doruk P. The impact of knee osteoarthritis on rehabilitation outcomes in hemiparetic stroke patients. J Back Musculoskelet Rehabil 2013;26:207-11.  Back to cited text no. 5
    
6.
Botez P, Sirbu PD, Grierosu C, Mihailescu D, Savin L, Scarlat MM. Adult multifocal pigmented villonodular synovitis-clinical review. Int Orthop 2013;37:729-33.  Back to cited text no. 6
    
7.
Di Minno MND, Pasta G, Airaldi S, Zaottini F, Storino A, Cimino E, et al. Ultrasound for early detection of joint disease in patients with hemophilic arthropathy. J Clin Med 2017;6. pii: E77.  Back to cited text no. 7
    
8.
Paparo F, Fabbro E, Ferrero G, Piccazzo R, Revelli M, Camellino D, et al. Imaging studies of crystalline arthritides. Reumatismo 2012;63:263-75.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]


This article has been cited by
1 Aspirin/clopidogrel
Reactions Weekly. 2020; 1832(1): 62
[Pubmed] | [DOI]



 

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