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Show Dermatomyositis-Related Nonischemic Central Retinal Vein Occlusion Yvonne Wang, BA, Michael L. Morgan, MD, PhD, Angelina Espino Barros Palau, MD, Andrew G. Lee, MD, Rod Foroozan, MD Abstract: A 25-year-old woman with dermatomyositis suf-fered a right central retinal vein occlusion (CRVO) with visual acuity of 20/40. Examination of the right eye showed vitreous cells, suggesting inflammation of the central retinal vein leading to a CRVO as the presumed mechanism. She was admitted to hospital, and extensive evaluation was negative. She was maintained on corticosteroids to manage her dermatomyositis. One month later, she had macular edema and elevated intraocular pressure. Both resolved with dorzolamide, timolol, and intravitreal bevacizumab, and vision returned to 20/20 in the right eye. Journal of Neuro-Ophthalmology 2015;35:289-292 doi: 10.1097/WNO.0000000000000235 © 2015 by North American Neuro-Ophthalmology Society Dermatomyositis (DMS) is an idiopathic inflammatory myositis with characteristic cutaneous findings. The exact mechanism is unknown but is hypothesized to be immune-mediated microangiopathic disease causing ische-mia and inflammation of muscles (1, 2). DMS often presents with typical skin findings, including Gottron pap-ules on the digits, heliotrope eruption of the eyelids, and pigment changes in light exposed areas (3). These skin findings typically precede the muscle findings, which present as symmetric, proximal myalgias, most commonly affecting the deltoids and hip flexors without muscle atrophy. Other systemic findings include interstitial lung disease (4, 5), esophageal involvement (6), cardiomyopathy (7), and malig-nancy (3). Ocular findings in DMS are less common. We present a patient with DMS who presented with vision loss from nonischemic central retinal vein occlusion (CRVO). CASE REPORT A 25-year-old woman with a history of DMS and latent tuberculosis (TB) presented with 2 weeks of painless monocular vision loss of the right eye. Eight months before, she underwent muscle biopsy after 5 months of progressive proximal muscle weakness. The biopsy established the diagnosis of DMS. The patient did not have any skin findings, which likely contributed to the delay in diagnosis. Before starting corticosteroids, a cutaneous purified protein derivative test was positive, but a chest x-ray was negative. The patient had no pulmonary symptoms. For presumed latent TB, she was treated with isoniazid (INH) and pyridoxine for 9 months. For her DMS, she was prescribed prednisone 80 mg daily and slowly tapered to 17.5 mg and 15 mg on alternate days. She had no other medical illnesses, had no history of surgery, and was taking no other medications. She had no known drug allergies. She had worked as a teacher but was unemployed. She denied any history of smoking, drinking, or recreational drugs. Family history and review of systems were noncontributory. After 8 months of steroid therapy, the patient awoke with decreased vision in the right eye. On examination, her right fundus showed diffuse intraretinal hemorrhages and optic disc and macular edema. Brain magnetic resonance imaging was normal, and she was referred for neuro-ophthalmic evaluation. Visual acuity was 20/40, right eye and 20/20, left eye. Color vision was slightly reduced in the right eye (10/11 Ishihara plates) and normal in the left eye (11/11 plates). Pupils were 5 mm in the dark to 3 mm in the light with a right relative afferent pupillary defect. Weill Cornell Medical College Departments of Ophthalmology, Neurology, and Neurosurgery (YW, AGL), Weill Cornell Medical College, New York, New York; Department of Ophthalmology (MLM, AEBP, AGL), Houston Methodist Hospital, Houston, Texas; Weill Cornell Medical College Departments of Ophthalmology (AGL), Neurology, and Neurosurgery, Weill Cornell Medical College, Houston, Texas; Department of Ophthalmology (AGL, RF), Baylor College of Medicine, Houston, Texas; Department of Ophthalmology and Visual Sciences (AGL), The University of Texas Medical Branch, Galveston, Texas; and Department of Head & Neck Surgery (AGL), The University of Texas MD Anderson Cancer Center, Houston, Texas. The authors report no conflicts of interest. Address correspondence to Rod Foroozan, MD, Department of Ophthalmology, Baylor College of Medicine, 1977 Butler Boulevard, Houston, TX 77030; E-mail: foroozan@bcm.edu Wang et al: J Neuro-Ophthalmol 2015; 35: 289-292 289 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Ocular movements were intact, and alignment was ortho-tropic. Visual fields were full to confrontation. Slit-lamp examination was normal with intraocular pressure (IOP) of 13 mm Hg, right eye and 17 mm Hg, left eye. Funduscopy of the right eye showed disc edema, macular edema, dilated retinal vessels, and retinal hemorrhages in all 4 quadrants as well as vitreous cells (Fig. 1). The left fundus was normal. The patient was diagnosed with a right CRVO secondary to DMS and admitted to the hospital. Complete blood count showed a normal white blood cell count, mild anemia, and a normal platelet count. C-reactive protein, anticardiolipin antibodies, antinuclear antibodies, cyto-plasmic and perinuclear antineutrophilic cytoplasmic antibodies and angiotensin-converting enzyme were negative. Quantiferon gold testing for TB, syphilis serology, HIV, lysozyme, and urine histoplasma tests were all negative. Lumbar puncture revealed an elevated opening pressure of 27.5 cm H2O with protein 25 mg/dL (normal: 15-45 mg/dL), glucose 42 mg/dL (normal: 50- 80 mg/dL), 1 WBC/mL, 3 RBCs/mL, cytology negative for malignancy, and negative acid-fast bacillus stain and culture. At one-month follow-up, the patient's right visual acuity had declined to 20/50. Her funduscopic examination showed the evolution of CRVO with retinal hemorrhages and macular edema (Fig. 2). Because her IOP was 36 mm Hg, right eye and 30 mm Hg, left eye, she was treated with dorzolamide and timolol. Intravitreal bevacizumab was in-jected in the right eye 3 times over the subsequent 2 months. At 3-month follow-up, her vision had improved to 20/20 in the right eye, and her IOP was 13 mm Hg, right eye and 15 mm Hg, left eye. Her right fundus showed resolving scattered intraretinal hemorrhages and improve-ment of macular edema. DISCUSSION Complement-mediated microvasculopathy is the primary hypothesized mechanism for DMS (2, 8). Systemic small-vessel vasculitis involving nearly every major organ sys-tem, but particularly of the skin and lungs, has been reported in DMS (4, 5). Ocular involvement in DMS is less common. Case reports have documented retinopathy (9-11) ocular myositis (12), frosted branch angiitis (13), and optic neuropathy (14). Fong and Schatz (15) conducted a review of CRVO in adults younger than 50 years and found that they were less likely to be asso-ciated with systemic atherosclerotic disease and more likely to be inflammatory in nature compared with older patients. Nonischemic CRVO has been reported as a sequela of a number of autoinflammatory and autoimmune diseases, including sarcoidosis (16), systemic lupus erythematosus (17), Crohn disease (18), ulcerative colitis (19), and post-streptococcal uveitis syndrome (20). In DMS, an auto-antibody- mediated complement activation results in destruction of the small capillary beds (8). The resulting ischemic damage of the vasculature leads to chronic inflammatory changes that occlude the vessels causing myopathy, rash, and other DMS manifestations (1). Our patient had vitreous cells in her right eye, supporting that phlebitis of the central retinal vein was the cause of her CRVO. She was admitted for evaluation of other causes of vasculitis, including lupus and sarcoidosis, which was unrevealing. An alternative hypothesis is that DMS causes a systemic thrombotic microangiopathy resulting in CRVO. Sugimoto et al (21) reported a case of thrombotic microangiopathy associated with DMS that caused cutaneous vasculitis, renal hypertension, and occlusive retinopathy with cotton-wool spots and capillary obstruction. Similar findings also were reported in a child with juvenile DMS (22). However, in both cases, thrombocytopenia was a diagnostic sign of thrombotic microangiopathy. Our patient had a normal platelet count. CRVO is associated with hypercoagulable states, such as pregnancy (23, 24), homocysteinemia, and antiphos-pholipid syndrome (25, 26). There is some evidence that patients with DMS have an increased rate of deep venous thrombosis, reported to be as high as 20%, although the mechanism is uncertain (27). Although CRVO can be FIG. 1. Appearance of the right fundus is consistent with a central retinal vein occlusion, and the left fundus is normal. 290 Wang et al: J Neuro-Ophthalmol 2015; 35: 289-292 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. a complication of hypercoagulable state with DMS, we believe that in our patient, thrombosis of the central retinal veins was due to phlebitis, supported by the finding of vitreous cells. Our patient had the comorbid diagnosis of latent TB for which she had almost completed 8 months of INH. Given that TB has several known ocular complications including optic neuropathy (28), she had repeat QuantiFERON testing and cerebrospinal fluid acid-fast bacilli culture, both negative. We did not suspect her CRVO to be related to latent TB because she had no evidence of active disease and she was almost fully treated at the time of visual loss. We found no evidence of other causes for CRVO, including syphilis and HIV. Thus, we concluded that our patient's most likely cause of her CRVO was a complication of DMS. Because of the patient's relatively good visual function, she most likely had a nonischemic CRVO. This conclusion is consistent with previously reported cases of CRVO in young patients (71%-73% nonischemic) (15). There are no standardized treatment protocols for nonischemic CRVO in young patients, although steroids have been sug-gested as a treatment option to suppress the proposed inflammatory mechanism (15). Because our patient was already taking prednisone, we deferred altering her cortico-steroid therapy to her rheumatologist. The patient was observed for resolution of inflammation and management of the sequelae of CRVO. Macular edema secondary to CRVO was treated successfully with intravitreal bevacizu-mab injections. The elevation in IOP was most likely sec-ondary to steroid use. Steroid-induced glaucoma is well characterized and typically presents within weeks of starting ophthalmic steroids but may present months after starting systemic steroids (29). Elevated IOP was first noted in our patient a month after she presented with the CRVO after 9 months of systemic steroid use. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: Y. Wang, M. L. Morgan, R. Foroozan; b. Acquisition of data: M. L. Morgan, R. Foroozan; c. Analysis and interpretation of data: Y. Wang, M. L. Morgan, A. Espino Barros Palau, A. G. Lee, R. Foroozan. Category 2: a. Drafting the manuscript: Y. Wang, M. Morgan, A. G. Lee, R. Foroozan; b. Revising it for intellectual content: Y. Wang, M. L. Morgan, A. Espino Barros Palau, A. G. Lee, R. Foroozan. Category 3: a. Final approval of the completed manuscript: Y. Wang, M. L. Morgan, A. Espino Barros Palau, A. G. Lee, R. Foroozan. REFERENCES 1. Luo YB, Mastaglia FL. Dermatomyositis, polymyositis and immune-mediated necrotising myopathies. Biochim Biophys Acta. 2014;S0925-4439:00162-00168. 2. Kissel JT, Halterman RK, Rammohan KW, Mendell JR. The relationship of complement-mediated microvasculopathy to the histologic features and clinical duration of disease in dermatomyositis. Arch Neurol. 1991;48:26-30. 3. Femia AN, Vleugels RA, Callen JP. Cutaneous dermatomyositis: an updated review of treatment options and internal associations. Am J Clin Dermatol. 2013;14:291-313. 4. Kawakami T, Mizoguchi M, Saito R, Soma Y. Histopathological evidence of small-vessel vasculitis within the skin and lungs associated with interstitial pneumonia in an adult patient with dermatomyositis. Clin Exp Dermatol. 2008;33:415-417. 5. Verstraeten AS, Verbrugghe W, Wuyts W. A patient with interstitial lung disease secondary to dermatomyositis: a case report and review of the literature. Acta Clin Belg. 2013;68:240-244. 6. Dagan A, Markovits D, Braun-Moscovici Y, Rozin A, Toledano K, Balbir-Gurman A. Life-threatening oropharyngeal aphagia as the major manifestation of dermatomyositis. Isr Med Assoc J. 2013;15:453-455. 7. Lu Z, Wei Q, Ning Z, Qian-Zi Z, Xiao-Ming S, Guo-Chun W. Left ventricular diastolic dysfunction-early cardiac FIG. 2. Appearance of macular edema with optical coherence tomography at presentation (A), with improvement at 2 weeks (B) and 4 weeks (C) following intravitreal bevacizumab. D. The appearance of the right macula at 5 months after 2 additional bevacizumab injections. Wang et al: J Neuro-Ophthalmol 2015; 35: 289-292 291 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. impairment in patients with polymyositis/dermatomyositis: a tissue doppler imaging study. J Rheumatol. 2013;40:1572-1577. 8. Emslie-Smith AM, Engel AG. Microvascular changes in early and advanced dermatomyositis: a quantitative study. Ann Neurol. 1990;27:343-356. 9. Yeo LM, Swaby DS, Situnayake RD, Murray PI. Irreversible visual loss in dermatomyositis. Br J Rheumatol. 1995;34:1179-1181. 10. Backhouse O, Griffiths B, Henderson T, Emery P. Ophthalmic manifestations of dermatomyositis. Ann Rheum Dis. 1998;57:447-449. 11. Yan Y, Shen X. Purtscher-like retinopathy associated with dermatomyositis. BMC Ophthalmol. 2013;13:36. 12. Kokotis P, Theodossiadis P, Bouros C, Sfikakis PP. Bilateral ocular myositis as a late complication of dermatomyositis. J Rheumatol. 2005;32:379-381. 13. Lee MY, Kim HH, Kim KS. Frosted branch angiitis, presumably related to dermatomyositis. Ocul Immunol Inflamm. 2011;9:129-131. 14. Foroozan R. Visual loss from optic neuropathy in dermatomyositis. Rheumatology (Oxford). 2004;43:391-393. 15. Fong AC, Schatz H. Central retinal vein occlusion in young adults. Surv Ophthalmol. 1993;37:393-417. 16. Adema AY, ten Kate RW, Plaisier MB. Central retinal vein occlusion as an uncommon complication of sarcoidosis. Neth J Med. 2013;71:134-136. 17. Yen YC, Weng SF, Chen HS, Lin YS. Risk of retinal vein occlusion in patients with systemic lupus erythematosus: a population-based cohort study. Br J Ophthalmol. 2013;97:1192-1196. 18. Yamane Ide S, Reis Rda S, Vieira de Moraes H Jr. Retinal central vein occlusion in remission of Crohn's disease: case report. Arq Bras Oftalmol. 2007;70:1034-1036. 19. Doi M, Nakaseko Y, Uji Y, Fujioka C. Central retinal vein occlusion during remission of ulcerative colitis. Jpn J Ophthalmol. 1999;43:213-216. 20. De Smet MD. Papillophlebitis and uveitis as a manifestation of post-streptococcal uveitis syndrome. Eye (Lond). 2009;23:985-987. 21. Sugimoto T, Hajiro T, Fujimoto T, Kojyo N, Horie M, Kashiwagi A. Thrombotic microangiopathy in an adult patient with clinically amyopathic dermatomyositis complicated with interstitial lung disease. Lupus. 2007;16:1004-1005. 22. Bader-Meunier B, Monnet D, Barnerias C, Halphen I, Lambot- Juhan K, Chalumeau M, Costedoat-Chalumeau N, Ribeil JA, Bodemer C, Gherardi R. Thrombotic microangiopathy and Purtscher-like retinopathy as a rare presentation of juvenile dermatomyositis. Pediatrics. 2012;129:e821-e824. 23. Humayun M, Kattah J, Cupps TR, Limaye SR, Chrousos GA. Papillophlebitis and arteriolar occlusion in a pregnant woman. J Clin Neuroophthalmol. 1992;12:226-229. 24. McLoone EM, Best RM. Pregnancy-related papillophlebitis. Eur J Ophthalmol. 2004;14:65-66. 25. Fegan CD. Central retinal vein occlusion and thrombophilia. Eye (Lond). 2002;16:98-106. 26. Lahey JM, Tunç M, Kearney J, Modlinski B, Koo H, Johnson RN, Tanaka S. Laboratory evaluation of hypercoagulable states in patients with central retinal vein occlusion who are less than 56 years of age. Ophthalmology. 2002;109:126-131. 27. Gaitonde SD, Ballou SP. Deep venous thrombosis in dermatomyositis. J Rheumatol. 2008;35:2288. 28. Ooi YL, Tai LY, Subrayan V, Tajunisah I. Combined optic neuropathy and central retinal artery occlusion in presumed ocular tuberculosis without detectable systemic infection. Ocul Immunol Inflamm. 2011;19:370-372. 29. Kersey JP, Broadway DC. Corticosteroid-induced glaucoma: a review of the literature. Eye (Lond). 2006;20:407-416. 292 Wang et al: J Neuro-Ophthalmol 2015; 35: 289-292 Clinical Observation Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
