Skip Navigation LinksHome > January 2013 - Volume 19 - Issue 1 > Development of Rice Bodies in 2 Children Younger Than 3 Year...
JCR: Journal of Clinical Rheumatology:
doi: 10.1097/RHU.0b013e31826d6b5e
Case Reports

Development of Rice Bodies in 2 Children Younger Than 3 Years

Druschel, Claudia; Funk, Julia F.; Kallinich, Tilmann; Lieb, Angelika; Placzek, Richard P.

Free Access
Article Outline
Collapse Box

Author Information

From the Charite University Medicine Berlin, Berlin, Germany.

The authors declare no conflict of interest.

Correspondence: Claudia Druschel, MD, Center for Musculoskeletal Surgery, Charité, University Medicine Berlin, Campus Virchow Augustenburger Platz 1, 13353, Berlin, Germany 13353. E-mail: Claudia.druschel@charite.de.

Collapse Box

Abstract

Abstract: Rice bodies are synovial fluid nodules macroscopically resembling shiny white rice beans. They have been seen in synovial fluid from several types of inflammatory arthritis including tuberculosis, pyogenic arthritis, and juvenile idiopathic arthritis and adult rheumatoid arthritis. Microscopically, they consist of amorphous material, fibrin, and collagen. We report the rare cases of 2 children younger than 3 years with multiple rice body formations in the knee joints.

Rice bodies were initially described by Riese in 1896 to occur in joints affected by tuberculosis.1 The biochemical and ultrastructural composition of rice bodies, which are named after their macroscopic appearance, was reported by Albrecht et al2 in 1965 in cases of adults with rheumatoid arthritis. Generally involving knee and shoulder, rice bodies arise from the synovium and enter the fluid.3 Some investigators suggest that ischemia and microinfarctions after intra-articular synovial inflammation are causing the formation of rice bodies.4 Then, the rice bodies arise from infarcted synovium and are shed into articular fluid. Microinfarctions are generally caused by rheumatologic diseases such as rheumatoid arthritis, systemic lupus erythematosus, seronegative arthritis, or infectious pathologies such as nonspecific septic arthritis or tuberculosis.5 The association of rice bodies with chronic inflammatory disease is readily known for adult patients but to date rarely reported for young children.2 We report clinical, radiologic, and histopathologic findings and the surgical therapy for 2 children younger than 3 years with juvenile idiopathic arthritis (JIA) complicated by rice body formation in the knee joint. The children were treated in 2 pediatric orthopedic university departments.

Back to Top | Article Outline
Case 1

A 2-year-old girl was referred to our clinic with painless swelling of both knees initially noticed by her foster parents 7 months earlier. The medical history of the otherwise healthy and normally developed girl was negative regarding trauma, tuberculosis, or other infectious diseases. At first presentation in our department, physical examination revealed severe effusion of both knees, which is more pronounced on the left side than on the right side. No other signs of infection, for example, erythema or local hyperthermia, were observed. An elastic soft mass was palpable at the left lateral recess. The range of motion was only slightly decreased. Inflammation parameters were persistently mildly elevated (C-reactive protein, 14.3 mg/L; erythrocyte sedimentation rate, 34 mm in the first hour; immunoglobulin G, 1400 mg/dL). Furthermore, antinuclear antibodies were detected at a low titer (1:160), whereas rheumatoid factor, HLA-B27, and the tuberculin skin test results were negative. Radiography of both knees showed no apparent calcification, degenerative, or tumorous changes (Fig. 1). Magnetic resonance imaging (MRI) of the involved area revealed massive effusion in both knees containing multiple nodules of varying size that were isointense on T1-weighted and slightly hyperintense on T2-weighted spin echo images relative to the hypointense skeletal muscle (Fig. 1). Records from another hospital reported a failed knee joint aspiration, which revealed only small amounts of bloody fluid. Because of these findings, we decided to perform an open surgical exploration with biopsy of the more affected left knee joint for diagnostic and therapeutic purposes via longitudinal incision over the lateral recess. The subcutaneous fat and fascia were found to be not affected. When the joint capsule was incised, it revealed a joint filled with clear fluid containing numerous tiny, white “rice bodies,” each measuring approximately 3 mm in diameter (Fig. 2). After a thorough lavage and debridement of the remaining rice bodies, a synovia biopsy specimen was taken. The histopathologic examination confirmed the macroscopic diagnosis of rice bodies. Most of them were covered with fibrin and isolated macrophages. In addition, a severe chronic nonspecific inflammation of the synovial tissue with small fibrin islets emerging from the synovial surface was observed. There was no apparent granuloma suggesting sarcoidosis. Microbacterial cultures revealed no growth in the aerobic, anaerobic, or mycobacterial cultures. Biopsy and surgical lavage without synovectomy were performed on the right knee 1 week later. Both knees received a therapeutic intra-articular injection with corticosteroids. The patient regained good function of both knee joints 2 weeks after operation. Because of the complicated course of the underlying oligoarticular JIA, a systemic methotrexate therapy was initiated.

Figure 1
Figure 1
Image Tools
Figure 2
Figure 2
Image Tools
Back to Top | Article Outline
Case 2

An almost 3-year-old girl was referred to the other institution with swelling of the right knee. Medical history revealed a gastroenteritis 3 months before the swelling and was negative regarding trauma or any other diseases. The physical examination showed an effusion of the right knee with mild hyperthermia but without erythema. The range of motion was limited especially in flexion. The ultrasound examination of the right knee joint showed an extensive effusion with synovial hypertrophy and hyperechoic signals. Laboratory investigation results including C-reactive protein and rheumatoid factor were negative. Radiographs of the right knee joint revealed no osseous changes but a noncalcified soft tissue mass. Magnetic resonance imaging showed an extensive effusion with numerous nodules inside the joint that were not observable on T1-weighted sequences but clearly observable on T2-weighted images (Fig. 3). For diagnostic and therapeutic purposes, an arthroscopic intervention with complete synovectomy was performed. Cannulation of the knee joint through the anterolateral port during arthroscopy caused spillage of numerous tiny, white bodies (Fig. 4). In addition, extensive villous hypertrophy of the synovium was present. Histopathologic evaluation showed that the rice bodies were composed mainly of organized fibrin and collagen surrounded by a thin fibrin layer (Fig. 4). Full range of motion was established within 6 weeks. Subsequent MRI showed no evidence of further rice bodies. An antinuclear antibodies-positive juvenile oligoarthritis type I was diagnosed and treated with nonsteroidal anti-inflammatory drugs.

Figure 3
Figure 3
Image Tools
Figure 4
Figure 4
Image Tools
Back to Top | Article Outline

DISCUSSION

Development of rice bodies in childhood is a possibly overlooked and rarely reported complication of JIA. Rice bodies, resembling polished white rice, have been detected in synovial fluid in several types of inflammatory arthritis including tuberculosis, pyogenic arthritis, and both juvenile and adult rheumatoid arthritis.2 They can be encountered in both early and late periods during the course of rheumatoid arthritis, but their appearance is not related to the duration or clinical and radiologic findings of the disease.6 The origin of rice bodies is almost certainly from the synovium, and their clinical significance has always been obscure.7 It has most frequently been proposed that microvascular disease within the rheumatoid synovial tissue leads to synovial microinfarcts. Subsequent sloughing of the infarcted tissue into the joint cavity with the surface then covered by fibrin layers may lead to the classic appearance of rice bodies.8 They are generally regarded as end products of synovial inflammation, proliferation, and degeneration.9 Berg et al10 also found blood vessels in some of the rice bodies, suggesting a previous attachment to the synovial membrane. The nucleus consists of type I (40%), type II (40%), and type V (20%) collagen, which has the same configuration as the synovium in tuberculosis infection.3 Whether repeated local corticosteroid injections induce the formation of rice bodies is a current matter of debate.7 To our knowledge, the 2 presented patients are the first published case reports of children younger than 3 years with the diagnosis of development of rice bodies. In the literature, a small number of reports of this disease in older children and adolescents are found with a variety of treatment regimens. Asik et al3 reported an 11-year-old boy with rice bodies of synovial origin in the knee joint. Their patient had complete recovery of the symptoms after arthroscopic drainage and partial synovectomy. Wynne-Roberts and Cassidy2 detected rice bodies in the left knee of a 15-year-old boy with a known arthritis. He received a synovectomy with removal of numerous rice bodies. Under local injection of intra-articular corticosteroid, he developed recurrent effusions. Li-Yu et al7 included in their study a 5-year-old boy with camptodactyly of the left knee. The patient received recurrent intra-articular corticosteroid injections. The formation of rice bodies on the basis of JIA is a rare complication, and data concerning incidence are lacking.

Rice body arthritis can be detected with MRI that can reveal the intra-articular free bodies, differentiate among various soft tissue masses, and may show the extent of articular involvement.4 Rice bodies appear on MRI as nonattenuating homogenous soft tissue nodules, isointense in the T1-weighted sequences, and minimally hyperintense when compared with muscle in the T2-weighted series.11 Conventional radiographs mostly present soft tissue swelling. If a calcified mass can be detected, the differential diagnosis of synovial chondromatosis has to be excluded.3 A careful histopathologic evaluation of the debrided free bodies and synovial tissue is necessary to look for granulomatous involvement.3 Further differential diagnoses are pigmented villonodular synovitis and tumoral calcinosis by scleroderma or Sjögren syndrome.12

Regarding the therapeutic options, different approaches can be found in the literature. Operation, open arthrotomy, or arthroscopy is recommended for the evacuation of the rice bodies. There is no consensus if a synovectomy is essential or not. Because rice body formation in rheumatoid arthritis is a result of chronic inflammation and its complications, for example, blood vessel congestion, a consequent control of disease activity seems to be a reasonable approach to prevent reoccurrence. The pharmacologic therapy depends on the activity and the exact form of inflammatory disease and ranges from nonsteroidal anti-inflammatory drugs to biologicals. If nonrheumatologic conditions identified by means of microbiological and histologic examination are responsible for the disorder, a differentiated immunosuppressive or antimicrobial regimen has to be applied.

Back to Top | Article Outline

REFERENCES

1. Suso S, Piedro L, Ramon R. Tuberculous synovitis with “rice bodies” presenting as carpal tunnel syndrome. J Hand Surg Am. 1988; 13: 574–576.

2. Wynne-Roberts CR, Cassidy JT. Juvenile rheumatoid arthritis with rice bodies: light and electron microscopic studies. Ann Rheum Dis. 1979; 38: 8–13.

3. Asik M, Eralp L, Cetik Ö, et al.. Rice bodies of synovial origin in the knee joint. Arthroscopy. 2001; 17: 1–4.

4. Steinfeld R, Rock MG, Younge DA, et al.. Massive subacromial bursitis with rice bodies. Clin Orthop. 1994; 30: 185–190.

5. Cheung HS, Ryan LM, Kozin F, et al.. Synovial origins of rice bodies in joint fluid. Arthritis Rheum. 1980; 23: 72–76.

6. Popert AJ, Scott DL, Wainwright AC, et al.. Frequency of occurrence, mode of development and significance of rice bodies in rheumatoid joints. Ann Rheum Dis. 1982; 41: 109–117.

7. Li-Yu J, Clayburne GM, Sieck MS, et al.. Calcium apatite crystals in synovial fluid rice bodies. Ann Rheum Dis. 2006; 61: 387–390.

8. McCarty DJ, Cheung HS. Origin and significance of rice bodies in synovial fluid. Lancet. 1982; i: 715–716.

9. Albrecht M, Marinette GV, Jacox RF, et al.. A biochemical and electron microscopy study of rice bodies from rheumatoid patients. Arthritis Rheum. 1965; 8: 1053–1063.

10. Berg E, Wainwright R, Barton B, et al.. On the nature of rheumatoid rice bodies. Arthritis Rheum. 1977; 20: 1343–1349.

11. Chen A, Wong LY, Sheu CY, et al.. Distinguishing multiple rice body formation in chronic subacromial-subdeltoid bursitis from synovial chondromatosis. Skeletal Radiol. 2004; 33: 531–533.

12. Katayama I, Higashi K, Mukai H, et al.. Tumoral calcinosis in scleroderma. J Dermatol. 1989; 16: 82–85.

Keywords:

juvenile rheumatoid arthritis; development of rice bodies

© 2013 Lippincott Williams & Wilkins, Inc.

Follow Us!

Login

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.