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Imaging of orbital myositis in Crohn's disease
Clinical Imaging 32 (2008) 227 – 229
Imaging of orbital myositis in Crohn's disease Joana Ramalho, Mauricio Castillo⁎ Department of Radiology, University of North Carolina, Chapel Hill, NC 27599, USA Received 25 June 2007; accepted 4 September 2007
Abstract Orbital myositis (OM) is defined as a nonspecific localized orbital inflammatory process in which one or more of the extraocular muscles are involved. Clinically, it is characterized by acute pain exacerbated by eye movements. Diagnosis is based on history, clinical manifestations, and therapeutic response to steroids. Histologically, OM is characterized by polymorphous infiltration of inflammatory cells and varying amounts of fibrosis. Pathogenesis is unknown, but is probably related to underlying immune-mediated processes. Various inflammatory diseases have been associated with OM, and inflammatory bowel disease is an uncommon cause. We describe a patient with recurrent OM and recurrent Crohn's disease. © 2008 Elsevier Inc. All rights reserved. Keywords: Orbits; Extraocular muscles; Inflammation; Crohn's disease; Inflammatory bowel disease
1. Case report A 40-year-old woman with Crohn's disease since 1986, who has been treated over the years with cyclosporine, methotrexate, and 6-mercaptopurine, was admitted with a 2-week history of swelling around the right eye. An ophthalmologist diagnosed the condition as preseptal cellulitis and prescribed antibiotics, moxifloxacin hydrochloride ophthalmic solution, and prednisolone. A computed tomography (CT) study of the orbits was unremarkable. Over the next few days, her symptoms improved, but she had persistent bilateral eye pain with movement, vertical double vision, and blurry vision in the right eye. Similar symptoms had occurred before on multiple occasions, usually accompanying a flare-up of her Crohn's disease. All thyroid function tests yielded negative results. Repeat examination revealed an afebrile patient with slight erythema around the orbits. Visual acuity was 20/50-2 and 20/20 in the right and left eyes, respectively. Motility of ⁎ Corresponding author. Department of Radiology, Chapel Hill, NC 27599-7510, USA. Tel.: +1 919 966 3087; fax: +1 919 966 2884. E-mail address: [email protected] (M. Castillo). 0899-7071/08/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.clinimag.2007.09.012
the right eye on supraduction was present, and all ocular movements were painful. Anterior segment examination yielded unremarkable results, and funduscopy showed only old chorioretinal scars. Orbital MRI showed bilateral enlargement of extraocular muscles, with increased enhancement and with high T2 signal being most prominent in inferior recti muscles (right greater than left) (Fig. 1). Musculotendinous insertions were spared, and the optic nerves and brain were normal. Thyroid ophthalmopathy was excluded as thyroid-stimulating hormone was found to be elevated with suppressed thyroxine (T4). A diagnosis of orbital myositis (OM) was made, and it was treated with prednisone, 60 mg daily, until her symptoms had resolved completely. 2. Discussion OM is defined as a nonspecific localized orbital inflammatory process in which one or more extraocular muscles are involved. Clinically, it is characterized by acute pain exacerbated by eye movements [1]. Diplopia, swelling of the eyelid, conjunctival injection, and exophthalmos may also be present. Diagnosis is based on history, clinical and
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J. Ramalho, M. Castillo / Clinical Imaging 32 (2008) 227–229
Fig. 1. OM due to Crohn's disease. (A) Coronal fat-suppressed postcontrast T1-weighted image shows bilateral thickening of extraocular muscles (right greater than left). The borders of the right superior and lateral recti muscles are somewhat indistinct, but retro-ocular fat has a normal appearance. (B) Axial fat-suppressed postcontrast T1-weighted image shows that the disease causes thickening of extraocular muscle bellies, with sparing of their tendinous insertions.
imaging manifestations, and therapeutic response to steroids. Various inflammatory diseases, such as sarcoidosis, Wegener's granulomatosis, rheumatoid arthritis, and Lyme disease, also produce OM [1]. OM in inflammatory bowel disease is uncommon [2]. Crohn's disease is a chronic granulomatous inflammatory disease of the gastrointestinal tract with a tendency toward remissions and relapses [3]. It may affect any part of the gastrointestinal tract, often involving multiple discontinuous sites. The cause of Crohn's disease is not known. However, several factors—including infections; intestinal mucosal immune system abnormalities; genetic, mesenteric, or vascular alterations; diet; and psychogenic factors—are believed to be involved [3,4]. Crohn's disease can be considered systemic and is often associated with extraintest-
inal manifestations and other autoimmune disorders. Virtually almost every organ system may be involved. Eyes, skin, joints, kidneys, liver, biliary tract, and the vascular system are common sites of involvement. Extraintestinal immunorelated manifestations can be classified into two major groups: (a) reactive manifestations (arthritis, erythema nodosum, pyoderma gangrenosum, aphthous stomatitis, and iritis/uveitis) often associated with intestinal inflammatory activity reflecting a common pathogenic mechanism with the intestinal disease, and (b) other autoimmune diseases independent of bowel disease reflecting a major susceptibility to autoimmunity. Ophthalmological manifestations of inflammatory bowel disease are infrequent, occurring in b10% of patients [5,6]. Episcleritis, scleritis, and uveitis are the most common ones [5]. Ophthalmic complications are independent of the extent of bowel involvement and often occur in the early years of the disease [7]. Ophthalmological symptoms are usually unilateral, but may be bilateral and recurrent. One explanation of the pathogenesis of ocular inflammation implicates an immune-complex-type hypersensitivity reaction to a colonic antigen. This association may explain why patients with colitis or ileocolitis are more likely to have ocular inflammation than those with only small bowel involvement. Other theories suggest that ocular inflammation is due to cytotoxic antibodies or delayed-type hypersensitivity reactions [8]. Ocular complications often coexist with other extraintestinal manifestations, especially arthritis and erythema nodosum. This association may be due to similarities between the microvasculature of the synovium and the microvasculature of the uvea [9]. There are currently two classifications of inflammatory orbital pseudotumors: specific orbital inflammation (SOI) and nonspecific orbital inflammation (NSOI) [1]. SOI is a category designating typical clinical and pathological findings observed in sarcoidosis, Sjögren's syndrome, vasculitis (Wegener's disease and polyarthritis nodosa), and granulomatous and histiocytic diseases. NSOI includes histological forms that are more difficult to distinguish, such as idiopathic granulomatous and idiopathic sclerosing pseudotumors. NSOI appears to arise from an immune reaction in the orbit or to occur as an idiopathic phenomenon, either secondary to a neighboring zone of inflammation or due to distant autoimmune reactions. NSOI includes Crohn's disease, diabetes, rheumatoid arthritis, and Graves' disease [1,5,9]. MRI and CT are frequently used in the evaluation of acute orbital disease. The characteristic imaging finding in OM is diffuse enlargement of extraocular muscles, which exhibit slightly blurred margins. MRI excludes other lesions such as tumor infiltration and may provide additional information such as apical extension, cavernous sinus involvement, and/ or intracranial disease. Inflammatory infiltrates generally show low signal intensity on T1-weighted images, variable intensity on T2, and marked, diffuse, and irregular gadolinium enhancement [1]. In contrast, the sclerosing variant of
J. Ramalho, M. Castillo / Clinical Imaging 32 (2008) 227–229
orbital inflammatory disease typically shows decreased signal intensity on T2-weighted images and may be indistinguishable from neoplasia. OM most commonly affects the superior recti and oblique muscles, as well as the medial recti muscles. The most important differential diagnosis is that of thyroid ophthalmopathy. However, thyroid myopathy is usually painless at onset, symmetrical, slowly progressive, and associated with systemic manifestations of Graves' disease. Lid retraction, limitation of movement opposite to the affected muscle, and deterioration of visual function (including color perception) occur more often in thyroid eye diseases in contrast to OM [10]. It is possible that MRI helps to distinguish between OM and endocrine ophthalmopathy. In thyroid ophthalmopathy, the swelling of extraocular muscles is generally confined to muscle bellies, which remain well defined and spare tendons. In OM, the muscles show irregular contours, inflammation extends to adjacent fat, and there is usually involvement of the tendons. As in our patient, absence of tendon involvement does not exclude OM. A dramatic initial clinical response to corticosteroids may confirm the diagnosis of OM. Steroids must be tapered slowly to prevent exacerbation or recurrence [1]. If the orbital inflammation is steroid resistant, systemic immunosuppressive treatment may be considered [2]. Anti-tumor-necrosis-factor monoclonal antibodies are a new type of biological therapy [1]. Radiation therapy is not a first-line treatment but is a valuable alternative to corticosteroids in some patients. Low-dose radiotherapy is
229
also beneficial in patients with recurrent OM. Radiotherapy is effective for palliation, but not for long-term control [11]. References [1] Gordon LK. Orbital inflammatory disease: a diagnostic and therapeutic challenge. Eye 2006;20:1196–206. [2] Taylor S, McCluskey P, Lightman S. The ocular manifestations of inflammatory bowel disease. Curr Opin Ophthalmol 2006;17:538–44. [3] Danese S, Semeraro S, Papa A, Roberto I, Scaldaferri F, Fedeli G, Gasbarrini G, Gasbarrini A. Extraintestinal manifestations in inflammatory bowel disease. World J Gastroenterol 2005;46:7227–36. [4] Furukawa A, Saotome T, Yamasaki M, Maeda K, Nitta N, Takahashi M, Tsujikawa T, Fujiyama Y, Murata K, Sakamoto T. Cross-sectional imaging in Crohn's disease. Radiographics 2004;24:689–702. [5] Mintz R, Feller E, Bahr R, Shah S. Ocular manifestations of inflammatory bowel disease. Inflamm Bowel Dis 2004;2:135–9. [6] Maalouf T, Angioi R, George JL. Recurrent orbital myositis and Crohn's disease. Orbit 2001;20:75–80. [7] Maurer I, Zierz S. Recurrent orbital myositis—report of a familial incidence. Arch Neurol 1999;56:1407–9. [8] Winguth S, Kurhanewicz J, Wang M, Quivey J, James T, Char D. 31P magnetic resonance spectroscopy (MRS) of experimental orbital myositis. Invest Ophthalmol Vis Sci 1991;8:2417–22. [9] Nabali S, McCarey DW, Browne B, Capell HA. A case of orbital myositis associated with rheumatoid arthritis. Ann Rheum Dis 2002;61:938–9. [10] Troelstra A, Rijneveld WJ, Kooijman AC, Houtman WA. Correlation between NMR scans of extraocular muscles and clinical symptoms in Graves' ophthalmopathy. Doc Ophthalmol 1988;70:243. [11] Isobe K, Uno T, Kawakami H, Ueno N, Kawata T, Abe H, Minowa K, Yamamoto S, Ito H. Radiation therapy for idiopathic orbital myositis: two case reports and literature review. Radiat Med 2004;6:429–31.
Imaging of orbital myositis in Crohn's disease Joana Ramalho, Mauricio Castillo⁎ Department of Radiology, University of North Carolina, Chapel Hill, NC 27599, USA Received 25 June 2007; accepted 4 September 2007
Abstract Orbital myositis (OM) is defined as a nonspecific localized orbital inflammatory process in which one or more of the extraocular muscles are involved. Clinically, it is characterized by acute pain exacerbated by eye movements. Diagnosis is based on history, clinical manifestations, and therapeutic response to steroids. Histologically, OM is characterized by polymorphous infiltration of inflammatory cells and varying amounts of fibrosis. Pathogenesis is unknown, but is probably related to underlying immune-mediated processes. Various inflammatory diseases have been associated with OM, and inflammatory bowel disease is an uncommon cause. We describe a patient with recurrent OM and recurrent Crohn's disease. © 2008 Elsevier Inc. All rights reserved. Keywords: Orbits; Extraocular muscles; Inflammation; Crohn's disease; Inflammatory bowel disease
1. Case report A 40-year-old woman with Crohn's disease since 1986, who has been treated over the years with cyclosporine, methotrexate, and 6-mercaptopurine, was admitted with a 2-week history of swelling around the right eye. An ophthalmologist diagnosed the condition as preseptal cellulitis and prescribed antibiotics, moxifloxacin hydrochloride ophthalmic solution, and prednisolone. A computed tomography (CT) study of the orbits was unremarkable. Over the next few days, her symptoms improved, but she had persistent bilateral eye pain with movement, vertical double vision, and blurry vision in the right eye. Similar symptoms had occurred before on multiple occasions, usually accompanying a flare-up of her Crohn's disease. All thyroid function tests yielded negative results. Repeat examination revealed an afebrile patient with slight erythema around the orbits. Visual acuity was 20/50-2 and 20/20 in the right and left eyes, respectively. Motility of ⁎ Corresponding author. Department of Radiology, Chapel Hill, NC 27599-7510, USA. Tel.: +1 919 966 3087; fax: +1 919 966 2884. E-mail address: [email protected] (M. Castillo). 0899-7071/08/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.clinimag.2007.09.012
the right eye on supraduction was present, and all ocular movements were painful. Anterior segment examination yielded unremarkable results, and funduscopy showed only old chorioretinal scars. Orbital MRI showed bilateral enlargement of extraocular muscles, with increased enhancement and with high T2 signal being most prominent in inferior recti muscles (right greater than left) (Fig. 1). Musculotendinous insertions were spared, and the optic nerves and brain were normal. Thyroid ophthalmopathy was excluded as thyroid-stimulating hormone was found to be elevated with suppressed thyroxine (T4). A diagnosis of orbital myositis (OM) was made, and it was treated with prednisone, 60 mg daily, until her symptoms had resolved completely. 2. Discussion OM is defined as a nonspecific localized orbital inflammatory process in which one or more extraocular muscles are involved. Clinically, it is characterized by acute pain exacerbated by eye movements [1]. Diplopia, swelling of the eyelid, conjunctival injection, and exophthalmos may also be present. Diagnosis is based on history, clinical and
228
J. Ramalho, M. Castillo / Clinical Imaging 32 (2008) 227–229
Fig. 1. OM due to Crohn's disease. (A) Coronal fat-suppressed postcontrast T1-weighted image shows bilateral thickening of extraocular muscles (right greater than left). The borders of the right superior and lateral recti muscles are somewhat indistinct, but retro-ocular fat has a normal appearance. (B) Axial fat-suppressed postcontrast T1-weighted image shows that the disease causes thickening of extraocular muscle bellies, with sparing of their tendinous insertions.
imaging manifestations, and therapeutic response to steroids. Various inflammatory diseases, such as sarcoidosis, Wegener's granulomatosis, rheumatoid arthritis, and Lyme disease, also produce OM [1]. OM in inflammatory bowel disease is uncommon [2]. Crohn's disease is a chronic granulomatous inflammatory disease of the gastrointestinal tract with a tendency toward remissions and relapses [3]. It may affect any part of the gastrointestinal tract, often involving multiple discontinuous sites. The cause of Crohn's disease is not known. However, several factors—including infections; intestinal mucosal immune system abnormalities; genetic, mesenteric, or vascular alterations; diet; and psychogenic factors—are believed to be involved [3,4]. Crohn's disease can be considered systemic and is often associated with extraintest-
inal manifestations and other autoimmune disorders. Virtually almost every organ system may be involved. Eyes, skin, joints, kidneys, liver, biliary tract, and the vascular system are common sites of involvement. Extraintestinal immunorelated manifestations can be classified into two major groups: (a) reactive manifestations (arthritis, erythema nodosum, pyoderma gangrenosum, aphthous stomatitis, and iritis/uveitis) often associated with intestinal inflammatory activity reflecting a common pathogenic mechanism with the intestinal disease, and (b) other autoimmune diseases independent of bowel disease reflecting a major susceptibility to autoimmunity. Ophthalmological manifestations of inflammatory bowel disease are infrequent, occurring in b10% of patients [5,6]. Episcleritis, scleritis, and uveitis are the most common ones [5]. Ophthalmic complications are independent of the extent of bowel involvement and often occur in the early years of the disease [7]. Ophthalmological symptoms are usually unilateral, but may be bilateral and recurrent. One explanation of the pathogenesis of ocular inflammation implicates an immune-complex-type hypersensitivity reaction to a colonic antigen. This association may explain why patients with colitis or ileocolitis are more likely to have ocular inflammation than those with only small bowel involvement. Other theories suggest that ocular inflammation is due to cytotoxic antibodies or delayed-type hypersensitivity reactions [8]. Ocular complications often coexist with other extraintestinal manifestations, especially arthritis and erythema nodosum. This association may be due to similarities between the microvasculature of the synovium and the microvasculature of the uvea [9]. There are currently two classifications of inflammatory orbital pseudotumors: specific orbital inflammation (SOI) and nonspecific orbital inflammation (NSOI) [1]. SOI is a category designating typical clinical and pathological findings observed in sarcoidosis, Sjögren's syndrome, vasculitis (Wegener's disease and polyarthritis nodosa), and granulomatous and histiocytic diseases. NSOI includes histological forms that are more difficult to distinguish, such as idiopathic granulomatous and idiopathic sclerosing pseudotumors. NSOI appears to arise from an immune reaction in the orbit or to occur as an idiopathic phenomenon, either secondary to a neighboring zone of inflammation or due to distant autoimmune reactions. NSOI includes Crohn's disease, diabetes, rheumatoid arthritis, and Graves' disease [1,5,9]. MRI and CT are frequently used in the evaluation of acute orbital disease. The characteristic imaging finding in OM is diffuse enlargement of extraocular muscles, which exhibit slightly blurred margins. MRI excludes other lesions such as tumor infiltration and may provide additional information such as apical extension, cavernous sinus involvement, and/ or intracranial disease. Inflammatory infiltrates generally show low signal intensity on T1-weighted images, variable intensity on T2, and marked, diffuse, and irregular gadolinium enhancement [1]. In contrast, the sclerosing variant of
J. Ramalho, M. Castillo / Clinical Imaging 32 (2008) 227–229
orbital inflammatory disease typically shows decreased signal intensity on T2-weighted images and may be indistinguishable from neoplasia. OM most commonly affects the superior recti and oblique muscles, as well as the medial recti muscles. The most important differential diagnosis is that of thyroid ophthalmopathy. However, thyroid myopathy is usually painless at onset, symmetrical, slowly progressive, and associated with systemic manifestations of Graves' disease. Lid retraction, limitation of movement opposite to the affected muscle, and deterioration of visual function (including color perception) occur more often in thyroid eye diseases in contrast to OM [10]. It is possible that MRI helps to distinguish between OM and endocrine ophthalmopathy. In thyroid ophthalmopathy, the swelling of extraocular muscles is generally confined to muscle bellies, which remain well defined and spare tendons. In OM, the muscles show irregular contours, inflammation extends to adjacent fat, and there is usually involvement of the tendons. As in our patient, absence of tendon involvement does not exclude OM. A dramatic initial clinical response to corticosteroids may confirm the diagnosis of OM. Steroids must be tapered slowly to prevent exacerbation or recurrence [1]. If the orbital inflammation is steroid resistant, systemic immunosuppressive treatment may be considered [2]. Anti-tumor-necrosis-factor monoclonal antibodies are a new type of biological therapy [1]. Radiation therapy is not a first-line treatment but is a valuable alternative to corticosteroids in some patients. Low-dose radiotherapy is
229
also beneficial in patients with recurrent OM. Radiotherapy is effective for palliation, but not for long-term control [11]. References [1] Gordon LK. Orbital inflammatory disease: a diagnostic and therapeutic challenge. Eye 2006;20:1196–206. [2] Taylor S, McCluskey P, Lightman S. The ocular manifestations of inflammatory bowel disease. Curr Opin Ophthalmol 2006;17:538–44. [3] Danese S, Semeraro S, Papa A, Roberto I, Scaldaferri F, Fedeli G, Gasbarrini G, Gasbarrini A. Extraintestinal manifestations in inflammatory bowel disease. World J Gastroenterol 2005;46:7227–36. [4] Furukawa A, Saotome T, Yamasaki M, Maeda K, Nitta N, Takahashi M, Tsujikawa T, Fujiyama Y, Murata K, Sakamoto T. Cross-sectional imaging in Crohn's disease. Radiographics 2004;24:689–702. [5] Mintz R, Feller E, Bahr R, Shah S. Ocular manifestations of inflammatory bowel disease. Inflamm Bowel Dis 2004;2:135–9. [6] Maalouf T, Angioi R, George JL. Recurrent orbital myositis and Crohn's disease. Orbit 2001;20:75–80. [7] Maurer I, Zierz S. Recurrent orbital myositis—report of a familial incidence. Arch Neurol 1999;56:1407–9. [8] Winguth S, Kurhanewicz J, Wang M, Quivey J, James T, Char D. 31P magnetic resonance spectroscopy (MRS) of experimental orbital myositis. Invest Ophthalmol Vis Sci 1991;8:2417–22. [9] Nabali S, McCarey DW, Browne B, Capell HA. A case of orbital myositis associated with rheumatoid arthritis. Ann Rheum Dis 2002;61:938–9. [10] Troelstra A, Rijneveld WJ, Kooijman AC, Houtman WA. Correlation between NMR scans of extraocular muscles and clinical symptoms in Graves' ophthalmopathy. Doc Ophthalmol 1988;70:243. [11] Isobe K, Uno T, Kawakami H, Ueno N, Kawata T, Abe H, Minowa K, Yamamoto S, Ito H. Radiation therapy for idiopathic orbital myositis: two case reports and literature review. Radiat Med 2004;6:429–31.