International Journal of Rheumatic Diseases 2014; 17: 333–335
POSTGRADUATE QUIZ
The orbital mass A 24-year old man, a known case of juvenile-onset systemic lupus erythematosus (SLE), presented with 1 month of painful eye movement. On his ophtalmologic examination, the funduscopic examination, pupillary response, gaze and visual acuity were normal. With the exception of mild right medical canthus tenderness, his examination was otherwise normal. He was under treatment with prednisolone, hydroxychloroquine and calcium supplement since 12 years ago. On laboratory study, the erythrocyte sedimentation rate (ESR) was 65 mm/h (Westergren), C-reactive protein 14 mg/L (normal 0–10 mg/L), mild anemia (hemoglobin 10 mg/dL) (normal 13.5–17.5), positive anti-double stranded DNA (1 : 80) (normal < 1: 10), and rim (peripheral) pattern fluorescent antinuclear antibody (ANA) (titer 1 : 320) (normal < 1 : 40). Orbital computed tomography (CT) scan was performed. It showed an isodense mass in the medial canthus of right orbit in close contact to the globe and sclera extending to the lower eyelid (Fig. 1a–c, arrow). She underwent incisional biopsy of the orbital mass. On histologic examination, there was vasculitis with occlusion and thickening of the vessel walls and diffuse ischemic necrosis (Fig. 1d).
WHAT IS YOUR DIAGNOSIS? Orbital presentation of systemic lupus erythematosus Systemic lupus erythematosus is a chronic multisystemic autoimmune disease with unknown exact etiology. Ocular involvement due to SLE is seen in up to one-third of patients.1 It is accepted that the majority of signs and symptoms, including ocular manifestations have a vascular etiology, the nature of which varies from vasculopathy secondary to immune complex deposits, hyaline changes, fibrinoid necrosis with or without thrombosis, fibrosis and perivascular mononuclear cell infiltration. True vasculitis with inflammatory cell infiltrate in the wall of the vessel is relatively uncommon. Ocular changes occur more frequently in patients with active SLE.2 The most common
association is keratoconjunctivitis sicca, while the most visually devastating sequelae occurs secondary to optic nerve involvement and retinal vaso-occlusion.1 SLE can involve any orbital structure, including the anterior segment (e.g., blepharitis, keratitis, episcleritis, scleritis2,3), uvea, choroid, retina,2–5 ocular adenexa (e.g., dacryoadenitis) and rarely orbital soft tissue (e.g., myositis, panniculitis, pseudotumor, orbital mass2,4–6). There are also neuro-ophthalmic manifestations, such as optic neuritis, ischemic optic neuropathy, ocular motor abnormalities, internuclear ophthalmoplegia, one and a half syndrome and abnormal pupillary reflex.2 The most significant and characteristic, although not pathognomic, ophthalmologic features of SLE are retinopathy and choroidopathy. The retinal lesions on fundoscopy and gross examination consist of fluffy irregular whitish spots of the ‘cotton wool’ type located on the superficial layer of the retina, usually in the posterior part of the fundus. They are often associated with small superficial retinal hemorrhages at the margins of the lesions, papilledema and generalized blurring of the choroidal reflex. On microscopic sections the ‘cotton wool’ spots are microinfarcts of the nerve fiber layer. These swollen degenerated nerve fibers appear as pink areas, each containing a central globular body, resembling a nucleus and hence are called ‘cytoid bodies’. The arteries of the choroid may show hyaline or fibrinoid changes associated with mononuclear cell infiltration.1–6 SLE in the eye rarely presents as non-specific inflammation with mixed inflammatory infiltrates and varying degrees of fibrosis known as orbital pseudotumor or non-specific orbital inflammation.7 Selectively or diffusely orbital structures are involved by inflammatory processes and lead to dacryoadenitis, myositis, optic perinuritis, scleritis, and superior orbital fissure and cavernous sinus inflammation with dramatic response to steroids.7 This patient had orbital soft tissue mass without intraocular or other systemic involvement and biopsy revealed vasculitis and vascular occlusion with ischemic necrosis, rather than non-specific
© 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd
H. Mirfazaelian et al.
(a)
(c)
inflammatory processes. With positive serologic markers, the recurrence of SLE as an orbital mass was diagnosed. This presentation of SLE with true vasculitis is rare. Systemic corticosteroid therapy is the cornerstone of managing orbital mass, for which dramatic improvement has diagnostic value in favor of orbital pseudotumor.8 Other therapies with promise include cytotoxic agents, immunosuppressants, intravenous immunoglobulins, tumor necrosis factor (TNF)-alpha inhibitor, monoclonal antibody and mycophenolate moftil, which inhibit de novo purine synthesis and prevent B and T lymphocyte replication.9 Intraorbital injection of corticosteroid has been found to be useful and effective treatment of orbital pseudotumor and may be considered as a first-line treatment in some patients.10 The patient was referred to the rheumatology unit and underwent cotricosteroid treatment with prompt response over 3 days and gradual tumor regression and symptom resolution with uneventful follow-up. Hadi MIRFAZAELIAN,1 Behzad KHADEMI,2 Babak BAGHERI3 and Yahya DANESHBOD4 1
Department of Emergency Medicine, Tehran University of Medical Sciences, Tehran, 2Department of Ophthalmology, Shiraz University of Medical Sciences, 3Department of Ophthalmology, Bagheri Optic Clinic, and 4Department of
334
(b)
(d)
Figure 1 (a–c) Spiral computed tomography scan of both orbits shows an isodense mass in the medial canthus of the right orbit in close contact to globe and sclera (arrow) extending to lower eyelid. (d) Histology showing diffuse ischemic necrosis with thickening and occlusion of the vessel walls.
Molecular pathology, Dr. Daneshbod Pathology Laboratory, Shiraz, Iran Correspondence: Dr Hadi Mirfazaelian, email: [email protected]
REFERENCES 1 Palejwala NV, Walia HS, Yeh S (2012) Ocular manifestations of systemic lupus erythematosus: a review of the literature. Autoimmune Dis 2012, 290898. 2 Davies JB, Rao PK (2008) Ocular manifestations of systemic lupus erythematosus. Curr Opin Ophthalmol 19, 512–8. 3 Frith P, Burge SM, Millard PR, Wojnarowska F (1990) External ocular findings in lupus erythematosus: a clinical and immunopathological study. Br J Ophthalmol 74, 163–7. 4 Amirlak I, Narchi H (2008) Isolated orbital pseudotumor as the presenting sign of systemic lupus erythematosus. J Pediatr Ophthalmol Strabismus 45 (1), 51–4. 5 Read RW (2004) Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm 12, 87–99. 6 Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C (2007) Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford) 46, 1757–62. 7 Yuen SJ, Rubin PA (2002) Idiopathic orbital inflammation: ocular mechanisms and clinicopathology. Ophthalmol Clin North Am 15 (1), 121–6.
International Journal of Rheumatic Diseases 2014; 17: 333–335
The orbital mass
8 Yuen SJA, Rubin PA (2003) Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol 121, 491–9. 9 Chaudhry IA, Shamsi FA, Arat YO, Riley FC (2008) Orbital pseudotumor: distinct diagnostic features and management. Middle East Afr J Ophthalmol 15 (1), 17–27.
International Journal of Rheumatic Diseases 2014; 17: 333–335
10 Leibovitch I, Prabhakaran VC, Davis G, Selva D (2007) Intraorbital injection of triamcinolone acetonide in patients with idiopathic orbital inflammation. Arch Ophthalmol 125, 1647–51.
335
Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
POSTGRADUATE QUIZ
The orbital mass A 24-year old man, a known case of juvenile-onset systemic lupus erythematosus (SLE), presented with 1 month of painful eye movement. On his ophtalmologic examination, the funduscopic examination, pupillary response, gaze and visual acuity were normal. With the exception of mild right medical canthus tenderness, his examination was otherwise normal. He was under treatment with prednisolone, hydroxychloroquine and calcium supplement since 12 years ago. On laboratory study, the erythrocyte sedimentation rate (ESR) was 65 mm/h (Westergren), C-reactive protein 14 mg/L (normal 0–10 mg/L), mild anemia (hemoglobin 10 mg/dL) (normal 13.5–17.5), positive anti-double stranded DNA (1 : 80) (normal < 1: 10), and rim (peripheral) pattern fluorescent antinuclear antibody (ANA) (titer 1 : 320) (normal < 1 : 40). Orbital computed tomography (CT) scan was performed. It showed an isodense mass in the medial canthus of right orbit in close contact to the globe and sclera extending to the lower eyelid (Fig. 1a–c, arrow). She underwent incisional biopsy of the orbital mass. On histologic examination, there was vasculitis with occlusion and thickening of the vessel walls and diffuse ischemic necrosis (Fig. 1d).
WHAT IS YOUR DIAGNOSIS? Orbital presentation of systemic lupus erythematosus Systemic lupus erythematosus is a chronic multisystemic autoimmune disease with unknown exact etiology. Ocular involvement due to SLE is seen in up to one-third of patients.1 It is accepted that the majority of signs and symptoms, including ocular manifestations have a vascular etiology, the nature of which varies from vasculopathy secondary to immune complex deposits, hyaline changes, fibrinoid necrosis with or without thrombosis, fibrosis and perivascular mononuclear cell infiltration. True vasculitis with inflammatory cell infiltrate in the wall of the vessel is relatively uncommon. Ocular changes occur more frequently in patients with active SLE.2 The most common
association is keratoconjunctivitis sicca, while the most visually devastating sequelae occurs secondary to optic nerve involvement and retinal vaso-occlusion.1 SLE can involve any orbital structure, including the anterior segment (e.g., blepharitis, keratitis, episcleritis, scleritis2,3), uvea, choroid, retina,2–5 ocular adenexa (e.g., dacryoadenitis) and rarely orbital soft tissue (e.g., myositis, panniculitis, pseudotumor, orbital mass2,4–6). There are also neuro-ophthalmic manifestations, such as optic neuritis, ischemic optic neuropathy, ocular motor abnormalities, internuclear ophthalmoplegia, one and a half syndrome and abnormal pupillary reflex.2 The most significant and characteristic, although not pathognomic, ophthalmologic features of SLE are retinopathy and choroidopathy. The retinal lesions on fundoscopy and gross examination consist of fluffy irregular whitish spots of the ‘cotton wool’ type located on the superficial layer of the retina, usually in the posterior part of the fundus. They are often associated with small superficial retinal hemorrhages at the margins of the lesions, papilledema and generalized blurring of the choroidal reflex. On microscopic sections the ‘cotton wool’ spots are microinfarcts of the nerve fiber layer. These swollen degenerated nerve fibers appear as pink areas, each containing a central globular body, resembling a nucleus and hence are called ‘cytoid bodies’. The arteries of the choroid may show hyaline or fibrinoid changes associated with mononuclear cell infiltration.1–6 SLE in the eye rarely presents as non-specific inflammation with mixed inflammatory infiltrates and varying degrees of fibrosis known as orbital pseudotumor or non-specific orbital inflammation.7 Selectively or diffusely orbital structures are involved by inflammatory processes and lead to dacryoadenitis, myositis, optic perinuritis, scleritis, and superior orbital fissure and cavernous sinus inflammation with dramatic response to steroids.7 This patient had orbital soft tissue mass without intraocular or other systemic involvement and biopsy revealed vasculitis and vascular occlusion with ischemic necrosis, rather than non-specific
© 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd
H. Mirfazaelian et al.
(a)
(c)
inflammatory processes. With positive serologic markers, the recurrence of SLE as an orbital mass was diagnosed. This presentation of SLE with true vasculitis is rare. Systemic corticosteroid therapy is the cornerstone of managing orbital mass, for which dramatic improvement has diagnostic value in favor of orbital pseudotumor.8 Other therapies with promise include cytotoxic agents, immunosuppressants, intravenous immunoglobulins, tumor necrosis factor (TNF)-alpha inhibitor, monoclonal antibody and mycophenolate moftil, which inhibit de novo purine synthesis and prevent B and T lymphocyte replication.9 Intraorbital injection of corticosteroid has been found to be useful and effective treatment of orbital pseudotumor and may be considered as a first-line treatment in some patients.10 The patient was referred to the rheumatology unit and underwent cotricosteroid treatment with prompt response over 3 days and gradual tumor regression and symptom resolution with uneventful follow-up. Hadi MIRFAZAELIAN,1 Behzad KHADEMI,2 Babak BAGHERI3 and Yahya DANESHBOD4 1
Department of Emergency Medicine, Tehran University of Medical Sciences, Tehran, 2Department of Ophthalmology, Shiraz University of Medical Sciences, 3Department of Ophthalmology, Bagheri Optic Clinic, and 4Department of
334
(b)
(d)
Figure 1 (a–c) Spiral computed tomography scan of both orbits shows an isodense mass in the medial canthus of the right orbit in close contact to globe and sclera (arrow) extending to lower eyelid. (d) Histology showing diffuse ischemic necrosis with thickening and occlusion of the vessel walls.
Molecular pathology, Dr. Daneshbod Pathology Laboratory, Shiraz, Iran Correspondence: Dr Hadi Mirfazaelian, email: [email protected]
REFERENCES 1 Palejwala NV, Walia HS, Yeh S (2012) Ocular manifestations of systemic lupus erythematosus: a review of the literature. Autoimmune Dis 2012, 290898. 2 Davies JB, Rao PK (2008) Ocular manifestations of systemic lupus erythematosus. Curr Opin Ophthalmol 19, 512–8. 3 Frith P, Burge SM, Millard PR, Wojnarowska F (1990) External ocular findings in lupus erythematosus: a clinical and immunopathological study. Br J Ophthalmol 74, 163–7. 4 Amirlak I, Narchi H (2008) Isolated orbital pseudotumor as the presenting sign of systemic lupus erythematosus. J Pediatr Ophthalmol Strabismus 45 (1), 51–4. 5 Read RW (2004) Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm 12, 87–99. 6 Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C (2007) Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford) 46, 1757–62. 7 Yuen SJ, Rubin PA (2002) Idiopathic orbital inflammation: ocular mechanisms and clinicopathology. Ophthalmol Clin North Am 15 (1), 121–6.
International Journal of Rheumatic Diseases 2014; 17: 333–335
The orbital mass
8 Yuen SJA, Rubin PA (2003) Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol 121, 491–9. 9 Chaudhry IA, Shamsi FA, Arat YO, Riley FC (2008) Orbital pseudotumor: distinct diagnostic features and management. Middle East Afr J Ophthalmol 15 (1), 17–27.
International Journal of Rheumatic Diseases 2014; 17: 333–335
10 Leibovitch I, Prabhakaran VC, Davis G, Selva D (2007) Intraorbital injection of triamcinolone acetonide in patients with idiopathic orbital inflammation. Arch Ophthalmol 125, 1647–51.
335
Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
![[Unusual cause of orbital mass].](/assets/img/hold.png)
