Published online before print December 20, 2002, 10.1148/radiol.2262011982
(Radiology 2003;226:387-389.)
© RSNA, 2003
Arthropathy in Behçet Disease: MR Imaging Findings in Two Cases1
Jung-Ah Choi, MD,
Jung Eun Kim, MD,
Sung Hye Koh, MD,
Hye Won Chung, MD and
Heung Sik Kang, MD
1 From the Department of Radiology, Seoul National University College of Medicine, 28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea. Received December 4, 2001; revision requested February 18, 2002; revision received April 10; accepted May 24. Address correspondence to H.S.K. (e-mail: kanghs@radcom.snu.ac.kr).
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ABSTRACT
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Arthropathy is part of the protean manifestations of Behçet disease. Imaging findings reported thus far have been based on those of conventional radiography. Magnetic resonance imaging in two cases of Behçet disease with arthropathy demonstrated synovial thickening and effusion, as well as myositis manifested by high signal intensity on T2-weighted images.
© RSNA, 2003
Index terms: Arthritis, 48.249, 48.252, 48.254 • Behçet disease • Joints, MR, 48.121411, 48.121412, 48.121415, 48.12143
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INTRODUCTION
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The original description of Behçet disease consisted of aphthous ulceration, genital ulceration, and iritis. Since the original description, many other systemic manifestations have been recognized. Among them, joint symptoms are well-recognized features of the syndrome and are described as a usually intermittent, self-limiting, nondestructive arthritis that involves peripheral larger joints such as the knee (1). Authors of previous reports have described variable prevalences of arthropathy, ranging from 5% to 97% (2–7). Radiologic findings have been reported as being none to mild or moderate (1,6,7). Most of the reported radiologic findings consisted of those depicted on conventional radiographs.
To our knowledge, no report about the magnetic resonance (MR) imaging findings of arthropathy in Behçet disease has been published in the English-language literature. Herein, we report the MR imaging findings in two cases of clinically diagnosed Behçet disease with arthropathy. Our institutional review board did not require approval or patient informed consent for this study.
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Case 1
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A 31-year-old man complained of multiple joint pain and swelling, which had started several days prior to admission. Behçet disease had been diagnosed 10 years earlier; at the time, the patient had had swelling of the left knee with heating sensation and pain, genital and oral ulceration, and an eyelid rash. The laboratory examinations were unrevealing, except for an elevated erythrocyte sedimentation rate of 52 mm/h. He was negative for rheumatoid factor and fluorescent antinuclear antibody.
MR examinations of his most painful joint sites, which were his left knee and ankle, were performed (Fig 1). MR examination of the left knee revealed increased signal intensity on T2-weighted images and intermediate signal intensity on T1-weighted images of the distal semimembranosus muscle near the musculotendinous junction. The same area showed enhancement after intravenous administration of gadopentetate dimeglumine (Magnevist; Schering, Berlin, Germany) at a dose of 0.2 mL per kilogram of body weight. Minimal fluid collection was noted in the knee joint cavity. No striking synovial enhancement was observed.
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Figure 1a. Case 1. A 31-year-old man with Behçet disease and left knee and ankle pain. (a) Transverse T1-weighted spin-echo MR image (600/20 [repetition time msec/echo time msec]) of the left knee shows intermediate signal intensity (arrow) in the distal semimembranosus muscle near the musculotendinous junction. (b) Transverse T2-weighted spin-echo MR image (2,000/70) of the same area as in a shows a slightly higher signal intensity (arrow). (c) Gadolinium-enhanced sagittal T1-weighted fat-suppressed spin-echo MR image (800/20) shows diffuse enhancement of the distal portion of the semimembranosus muscle (arrow). (d) Sagittal T2*-weighted gradient-recalled-echo MR image (600/18, 20° flip angle) of the left ankle shows high-signal-intensity fluid collections in the subtalar (solid arrow) and tibiotalar (open arrow) joints.
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Figure 1b. Case 1. A 31-year-old man with Behçet disease and left knee and ankle pain. (a) Transverse T1-weighted spin-echo MR image (600/20 [repetition time msec/echo time msec]) of the left knee shows intermediate signal intensity (arrow) in the distal semimembranosus muscle near the musculotendinous junction. (b) Transverse T2-weighted spin-echo MR image (2,000/70) of the same area as in a shows a slightly higher signal intensity (arrow). (c) Gadolinium-enhanced sagittal T1-weighted fat-suppressed spin-echo MR image (800/20) shows diffuse enhancement of the distal portion of the semimembranosus muscle (arrow). (d) Sagittal T2*-weighted gradient-recalled-echo MR image (600/18, 20° flip angle) of the left ankle shows high-signal-intensity fluid collections in the subtalar (solid arrow) and tibiotalar (open arrow) joints.
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Figure 1c. Case 1. A 31-year-old man with Behçet disease and left knee and ankle pain. (a) Transverse T1-weighted spin-echo MR image (600/20 [repetition time msec/echo time msec]) of the left knee shows intermediate signal intensity (arrow) in the distal semimembranosus muscle near the musculotendinous junction. (b) Transverse T2-weighted spin-echo MR image (2,000/70) of the same area as in a shows a slightly higher signal intensity (arrow). (c) Gadolinium-enhanced sagittal T1-weighted fat-suppressed spin-echo MR image (800/20) shows diffuse enhancement of the distal portion of the semimembranosus muscle (arrow). (d) Sagittal T2*-weighted gradient-recalled-echo MR image (600/18, 20° flip angle) of the left ankle shows high-signal-intensity fluid collections in the subtalar (solid arrow) and tibiotalar (open arrow) joints.
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Figure 1d. Case 1. A 31-year-old man with Behçet disease and left knee and ankle pain. (a) Transverse T1-weighted spin-echo MR image (600/20 [repetition time msec/echo time msec]) of the left knee shows intermediate signal intensity (arrow) in the distal semimembranosus muscle near the musculotendinous junction. (b) Transverse T2-weighted spin-echo MR image (2,000/70) of the same area as in a shows a slightly higher signal intensity (arrow). (c) Gadolinium-enhanced sagittal T1-weighted fat-suppressed spin-echo MR image (800/20) shows diffuse enhancement of the distal portion of the semimembranosus muscle (arrow). (d) Sagittal T2*-weighted gradient-recalled-echo MR image (600/18, 20° flip angle) of the left ankle shows high-signal-intensity fluid collections in the subtalar (solid arrow) and tibiotalar (open arrow) joints.
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MR examination of the left ankle demonstrated fluid in the subtalar and tibiotalar joints, as well as synovial enhancement in the subtalar joint and enhancement in the peroneal tendon sheath. The patient was treated with 5 mg of prednisolone (Delta-Cortef; Pharmacia, Seoul, Korea) administered orally once a day and with 0.6 mg of colchicine (Colchin; Hankook United, Seoul, Korea) administered orally twice a day based on the presumed diagnosis of arthritis associated with Behçet disease, and his clinical symptoms had improved.
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Case 2
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A 35-year-old woman complained of a painful mass on her left wrist that appeared 1 month prior to admission. Behçet disease had been diagnosed 2 years earlier, at which time she had a high fever, a genital ulcer, and a painful erythema nodosum–like lesion in lower areas of both legs. She also underwent surgery 1 year earlier, at which time a right wrist mass located proximal to the flexor digitorum tendon was removed. The pathologic examination revealed giant cell tumor of the tendon sheath. The laboratory data were unremarkable, with negative results for rheumatoid factor, antinuclear antibody, and bacteriologic study.
Ultrasonographic (US) examination of her left wrist demonstrated hypoechoic regions around the flexor digitorum tendon sheaths (Fig 2). MR examination of her left wrist revealed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images (Fig 2). These areas showed enhancement after administration of 0.2 mL/kg of gadodiamide (Omniscan; Nycomed Ireland, Cork, Ireland).
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Figure 2a. Case 2. A 35-year-old woman with Behçet disease and a painful mass on the left wrist. (a) Transverse US scan of the wrist reveals hypoechoic areas (arrows) around the flexor digitorum tendon sheaths. (b) Transverse T1-weighted spin-echo MR image (500/15) at the level of the lunate shows low signal intensities surrounding the flexor digitorum tendons (solid arrows) and convex bowing of the flexor retinaculum (open arrows). (c) Transverse T2-weighted spin-echo MR image (1,800/90) of the same areas as in b shows high signal intensities (arrows). (d) Gadolinium-enhanced T1-weighted spin-echo MR image (500/15) shows enhancement (arrows) of the previously hypointense areas around the flexor digitorum tendon sheaths.
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Figure 2b. Case 2. A 35-year-old woman with Behçet disease and a painful mass on the left wrist. (a) Transverse US scan of the wrist reveals hypoechoic areas (arrows) around the flexor digitorum tendon sheaths. (b) Transverse T1-weighted spin-echo MR image (500/15) at the level of the lunate shows low signal intensities surrounding the flexor digitorum tendons (solid arrows) and convex bowing of the flexor retinaculum (open arrows). (c) Transverse T2-weighted spin-echo MR image (1,800/90) of the same areas as in b shows high signal intensities (arrows). (d) Gadolinium-enhanced T1-weighted spin-echo MR image (500/15) shows enhancement (arrows) of the previously hypointense areas around the flexor digitorum tendon sheaths.
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Figure 2c. Case 2. A 35-year-old woman with Behçet disease and a painful mass on the left wrist. (a) Transverse US scan of the wrist reveals hypoechoic areas (arrows) around the flexor digitorum tendon sheaths. (b) Transverse T1-weighted spin-echo MR image (500/15) at the level of the lunate shows low signal intensities surrounding the flexor digitorum tendons (solid arrows) and convex bowing of the flexor retinaculum (open arrows). (c) Transverse T2-weighted spin-echo MR image (1,800/90) of the same areas as in b shows high signal intensities (arrows). (d) Gadolinium-enhanced T1-weighted spin-echo MR image (500/15) shows enhancement (arrows) of the previously hypointense areas around the flexor digitorum tendon sheaths.
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Figure 2d. Case 2. A 35-year-old woman with Behçet disease and a painful mass on the left wrist. (a) Transverse US scan of the wrist reveals hypoechoic areas (arrows) around the flexor digitorum tendon sheaths. (b) Transverse T1-weighted spin-echo MR image (500/15) at the level of the lunate shows low signal intensities surrounding the flexor digitorum tendons (solid arrows) and convex bowing of the flexor retinaculum (open arrows). (c) Transverse T2-weighted spin-echo MR image (1,800/90) of the same areas as in b shows high signal intensities (arrows). (d) Gadolinium-enhanced T1-weighted spin-echo MR image (500/15) shows enhancement (arrows) of the previously hypointense areas around the flexor digitorum tendon sheaths.
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Convex bowing of the flexor retinaculum was observed. No definite fluid collection or bone change was seen. A small perforation was noted incidentally in the triangular fibrocartilage. She underwent an excisional biopsy of the soft tissue of her left wrist. Pathologic examination revealed proliferative synovitis with severe inflammatory granulation tissue, which was consistent with synovitis in Behçet disease.
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Discussion
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Although joint manifestations have become a well-known part of the spectrum of multisystemic involvement in Behçet disease, previous descriptions have been limited to conventional radiographic findings. These findings have been described as being normal or showing mild abnormalities (6,8) consisting of nonspecific findings such as osteoporosis, and soft-tissue swelling with joint-space narrowing and marginal erosions seen only rarely (6,9,10).
The patient in case 1 showed ankle manifestations of myositis in the musculotendinous junction of the semimembranosus muscle and synovitis, as was suggested by the presence of joint effusion and synovial enhancement at MR imaging. Myositis in patients with Behçet disease has been described as having both diffuse and localized forms (11–13). In a report by Yazici et al (12), findings from muscle biopsy revealed marked muscle fiber degeneration and infiltration by mono- and polymorphonuclear cells, especially in perivascular areas. In another report (13), which included an electron microscopic examination of the muscle specimen subjected to biopsy, myofilamentous disarray and loss with aggregation of mitochondria and lysosomes in addition to cristalike inclusions within the muscle fiber were observed. The increased signal intensities observed on the T2-weighted images in case 1 may have been due to such infiltration by inflammatory cells and aggregation of mitochondria and cristalike inclusions. To our knowledge, radiologic findings of myositis have not been described in literature.
Synovial inflammation has been documented in previous reports in which hypertrophy and hyperplasia of synovial lining cells, hypervascularity, subsynovial accumulation of inflammatory cells, and replacement of the superficial zones of the synovial membrane by dense inflamed granulation tissue composed of lymphocytes, macrophages, fibroblasts, neutrophils, and vascular elements were described (2,9,14). Such pathologic findings are also seen in patients with rheumatoid arthritis (14). Enhancement due to gadolinium uptake in the synovium in our patient may correlate with acute synovitis, as it did in patients with acute rheumatoid arthritis in a previous report (15). Our patient, however, was negative for rheumatoid factor and fluorescent antinuclear antibody.
The patient in case 2 had findings suggestive of synovial inflammation of the flexor digitorum tendon sheath of the wrist at both sonographic and MR examinations. Biopsy helped to confirm the imaging findings of synovial proliferation and inflammation, which are consistent with findings in previous reports (2,9,14).
Our two cases presented with varying degrees and extent of synovial inflammation, ranging from joint effusion to marked synovial thickening and effusion. Such findings are consistent with pathologic findings described in the literature. However, the degree and extent of synovial inflammation were visualized well with MR imaging. Another finding in our study, which has not been described previously as an imaging finding, is myositis, which was visualized at T2-weighted imaging as high signal intensity in the muscle.
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FOOTNOTES
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Author contributions: Guarantors of integrity of entire study, J.A.C., H.S.K.; study concepts, H.S.K.; study design, J.A.C., H.S.K.; literature research, S.H.K.; clinical studies, J.E.K.; data acquisition, J.E.K.; data analysis/interpretation, S.H.K., J.A.C., J.E.K.; manuscript preparation and editing, J.A.C.; manuscript definition of intellectual content, J.A.C., H.W.C.; manuscript revision/review, H.W.C., H.S.K.; manuscript final version approval, H.S.K.
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REFERENCES
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- Caporn N, Higgs ER, Dieppe PA, Watt I. Arthritis in Behçet’s syndrome. Br J Radiol 1983; 56:87-91.[Abstract]
- Yurdakul S, Yazici H, Tuzin Y, et al. The arthritis of Behçet’s disease: a prospective study. Ann Rheum Dis 1983; 42:505-515.[Abstract/Free Full Text]
- Lehner T. Behçet’s syndrome and autoimmunity. Br Med J 1976; 1:465-476.
- Oshima Y, Shimizu T, Yokohan R, et al. Clinical studies on Behçet’s syndrome. Ann Rheum Dis 1963; 22:36-44.[Free Full Text]
- Assaad-Khalil SH. Clinical, genetic, immunological and biochemical features of 180 Egyptian patients with Behçet’s disease. In: O’Duffy JD, Kokmen E, eds. Behçet’s disease: basic and clinical aspects. New York, NY: Marcel Dekker, 1991; 269-277.
- Kim HA, Choi KW, Song YW. Arthropathy in Behçet’s disease. Scand J Rheumatol 1997; 26:125-129.[Medline]
- Park JH. Clinical analysis of Behçet disease: arthritic manifestations in Behçet disease may present as seronegative rheumatoid arthritis or palindromic rheumatism. Korean J Intern Med 1999; 14:66-72.[Medline]
- Rosenberger A, Adler OB, Haim S. Radiological aspects of Behçet’s disease. Radiology 1982; 144:261-264.[Abstract/Free Full Text]
- Vernon-Roberts B, Barnes CG, Revell PA. Synovial pathology in Behçet’s syndrome. Ann Rheum Dis 1978; 37:139-145.[Abstract/Free Full Text]
- Jawad AS, Goodwill CJ. Behçet’s disease with erosive arthritis. Ann Rheum Dis 1986; 45:961-962.[Abstract/Free Full Text]
- Arkin CR, Rothschild BM, Florendo NT, Popoff N. Behçet’s syndrome with myositis: a case report with pathologic findings. Arthritis Rheum 1980; 23:600-604.[Medline]
- Yazici H, Tuzuner N, Tuzun Y, Yurdakul S. Localized myositis in Behçet’s disease (letter). Arthritis Rheum 1981; 24:636.
- Frayha R. Muscle involvement in Behçet’s disease. Arthritis Rheum 1981; 24:636-637.
- Gibson T, Laurent R, Highton J, Wilton M, Dyson M, Millis R. Synovial histopathology of Behçet’s syndrome. Ann Rheum Dis 1981; 40:376-381.[Abstract/Free Full Text]
- Bjorkengren AG, Geborek P, Rydholm U, Holtas S, Petterson H. MR Imaging of the knee in acute rheumatoid arthritis: synovial uptake of gadolinium-DOTA. AJR Am J Roentgenol 1990; 155:329-332.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
