Letter Bilateral Serous Macular Elevation Associated with Lupus Protein‑losing Enteropathy Angela Hiu Yan Wong, FCOphth HK, FHKAM; Stephen Tak Lun Li, FCOphth HK, FHKAM Chi Kin Ho, FCOphth HK, FHKAM; Po Fat Yiu, FCOphth HK, FHKAM Department of Ophthalmology, Tuen Mun Hospital, Hong Kong SAR, China
J Ophthalmic Vis Res 2017; 12(2): 245-246
Dear Editor, We describe two clinical cases of patients who first presented to ophthalmologists with bilateral serous macular elevation and generalized edema. They were later diagnosed with lupus protein‑losing enteropathy complicated with life‑threatening conditions and were treated with immunosuppressants. As ophthalmic symptoms could be the initial presentation of this disease, our report highlights the importance of careful systemic review and early referral to reduce the morbidity and mortality. Two middle‑aged Chinese men presented with subacute blurred vision, periorbital swelling, chemosis, and bilateral ankle edema for one week. Their best corrected visual acuity ranged from 0.3 to 0.5 LogMAR. Examination revealed bilateral serous macular elevation. Optical coherent tomography showed macular elevation ranged from 726 to 800 µm with significant subretinal fluid accumulation. Fluorescein angiography revealed multiple leakage points at the level of the retinal pigment epithelium, without any vascular leakage. Both patients had severe hypoalbuminemia with albumin levels of 13 g/L and 29 g/L, respectively (normal value, 35–50 g/L). Liver and renal function tests were normal. Autoimmune markers including anti‑nuclear antibody, rheumatoid factor, erythrocyte sedimentation rate, and C‑reactive protein levels were all markedly elevated. Albumin scan using Tc‑99m human serum albumin revealed increased tracer activity in the proximal small bowel. Both patients were diagnosed with lupus protein‑losing enteropathy. Clinical investigations showed that both patients had ascites and pleural effusion. The condition of the first patient was further complicated with deep venous
thrombosis requiring bilateral lower limb amputation and long‑term warfarin therapy. Both patients were started on oral prednisolone (1 mg/kg/day) and immunosuppressants, including oral cyclophosphamide (100 mg/day) for the first patient and oral azathioprine (100 mg/day) for the second patient. Steroid dose was then tapered down. Periorbital swelling and macular elevation resolved in three months [Figure 1a and b], and the best corrected visual acuity improved to the range of 0.6 to 0.7 LogMAR. Both patients were kept on low dose oral prednisolone (5 mg/day) and immunosuppressants for maintenance. To our knowledge, this is the first report discussing the relationship between bilateral serous macular elevation and lupus protein‑losing enteropathy. Lupus protein‑losing enteropathy is a rare and life‑threatening manifestation of systemic lupus erythematosus. It is characterized by leakage of serum proteins from the gastrointestinal tract with profound generalized edema and hypoalbuminemia, without proteinuria. Prognosis is good for early treatment with systemic immunosuppressants.[1] Altered immunity and changes in osmotic pressure are proposed as mechanisms for development of serous retinal detachment. Negi and Marmor[2] emphasized the importance of osmotic pressure in maintaining fluid dynamics for retinal attachment to the retinal pigment epithelium. Severe hypoalbuminemia causes sustained
Correspondence to:
Angela Hiu Yan Wong, FCOphth HK, FHKAM. Tuen Mun Eye Centre 6/F, 4 Tuen Lee Street, Tuen Mun, Hong Kong SAR, China. E‑mail: [email protected] Received: 18-06-2016
Accepted: 07-12-2016
a
b
Figure 1. Optical coherence tomography showing subretinal fluid accumulation before treatment (a) and its resolution after treatment (b).
© 2017 Journal of Ophthalmic and Vision Research | Published by Wolters Kluwer ‑ Medknow
245
Letter; Wong et al
reduction of osmotic pressure in the choriocapillaries, leading to decreased vitreoretinal to choroidal fluid outflow and subsequent bilateral serous macular elevation. A complex immune reaction resulting in hyperpermeability of the choroidal vasculature also plays a role in disease development, as reported in a case of graft‑versus‑host disease.[3] Our cases highlighted the importance of systemic workup in patients with bilateral serous macular elevation and generalized edema. Ophthalmic symptoms could be the initial presentation of lupus protein‑losing enteropathy. Careful systemic review and early referral may avoid life‑threatening complications.
Financial Support and Sponsorship
2.
3.
Negi A, Marmor MF. Effects of subretinal and systemic osmolality on the rate of subretinal fluid resorption. Invest Ophthalmol Vis Sci 1984;25:616‑620. Cheng LL, Kwok AK, Wat NM, Neoh EL, Jon HC, Lam DS. Graft‑vs‑host‑disease‑associated conjunctival chemosis and central serous chorioretinopathy after bone marrow transplant. Am J Ophthalmol 2002;134:293‑295.
This is an open access article distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as the author is credited and the new creations are licensed under the identical terms.
Access this article online Quick Response Code:
Website: www.jovr.org
Nil. DOI: 10.4103/jovr.jovr_117_16
Conflicts of Interest There are no conflicts of interest.
REFERENCES 1.
246
Al‑Mogairen SM. Lupus protein‑losing enteropathy (LUPLE): A systemic review. Rheumatol Int 2011;31:995‑1001.
How to cite this article: Wong HYA, Li TLS, Ho CK, Yiu PF. Bilateral Serous macular elevation associated with lupus protein-losing enteropathy. J Ophthalmic Vis Res 2017;12:245-6. © 2017 Journal of Ophthalmic and Vision Research | Published by Wolters Kluwer ‑ Medknow
Journal of Ophthalmic and Vision Research Volume 12, Issue 2, April-June 2017
J Ophthalmic Vis Res 2017; 12(2): 245-246
Dear Editor, We describe two clinical cases of patients who first presented to ophthalmologists with bilateral serous macular elevation and generalized edema. They were later diagnosed with lupus protein‑losing enteropathy complicated with life‑threatening conditions and were treated with immunosuppressants. As ophthalmic symptoms could be the initial presentation of this disease, our report highlights the importance of careful systemic review and early referral to reduce the morbidity and mortality. Two middle‑aged Chinese men presented with subacute blurred vision, periorbital swelling, chemosis, and bilateral ankle edema for one week. Their best corrected visual acuity ranged from 0.3 to 0.5 LogMAR. Examination revealed bilateral serous macular elevation. Optical coherent tomography showed macular elevation ranged from 726 to 800 µm with significant subretinal fluid accumulation. Fluorescein angiography revealed multiple leakage points at the level of the retinal pigment epithelium, without any vascular leakage. Both patients had severe hypoalbuminemia with albumin levels of 13 g/L and 29 g/L, respectively (normal value, 35–50 g/L). Liver and renal function tests were normal. Autoimmune markers including anti‑nuclear antibody, rheumatoid factor, erythrocyte sedimentation rate, and C‑reactive protein levels were all markedly elevated. Albumin scan using Tc‑99m human serum albumin revealed increased tracer activity in the proximal small bowel. Both patients were diagnosed with lupus protein‑losing enteropathy. Clinical investigations showed that both patients had ascites and pleural effusion. The condition of the first patient was further complicated with deep venous
thrombosis requiring bilateral lower limb amputation and long‑term warfarin therapy. Both patients were started on oral prednisolone (1 mg/kg/day) and immunosuppressants, including oral cyclophosphamide (100 mg/day) for the first patient and oral azathioprine (100 mg/day) for the second patient. Steroid dose was then tapered down. Periorbital swelling and macular elevation resolved in three months [Figure 1a and b], and the best corrected visual acuity improved to the range of 0.6 to 0.7 LogMAR. Both patients were kept on low dose oral prednisolone (5 mg/day) and immunosuppressants for maintenance. To our knowledge, this is the first report discussing the relationship between bilateral serous macular elevation and lupus protein‑losing enteropathy. Lupus protein‑losing enteropathy is a rare and life‑threatening manifestation of systemic lupus erythematosus. It is characterized by leakage of serum proteins from the gastrointestinal tract with profound generalized edema and hypoalbuminemia, without proteinuria. Prognosis is good for early treatment with systemic immunosuppressants.[1] Altered immunity and changes in osmotic pressure are proposed as mechanisms for development of serous retinal detachment. Negi and Marmor[2] emphasized the importance of osmotic pressure in maintaining fluid dynamics for retinal attachment to the retinal pigment epithelium. Severe hypoalbuminemia causes sustained
Correspondence to:
Angela Hiu Yan Wong, FCOphth HK, FHKAM. Tuen Mun Eye Centre 6/F, 4 Tuen Lee Street, Tuen Mun, Hong Kong SAR, China. E‑mail: [email protected] Received: 18-06-2016
Accepted: 07-12-2016
a
b
Figure 1. Optical coherence tomography showing subretinal fluid accumulation before treatment (a) and its resolution after treatment (b).
© 2017 Journal of Ophthalmic and Vision Research | Published by Wolters Kluwer ‑ Medknow
245
Letter; Wong et al
reduction of osmotic pressure in the choriocapillaries, leading to decreased vitreoretinal to choroidal fluid outflow and subsequent bilateral serous macular elevation. A complex immune reaction resulting in hyperpermeability of the choroidal vasculature also plays a role in disease development, as reported in a case of graft‑versus‑host disease.[3] Our cases highlighted the importance of systemic workup in patients with bilateral serous macular elevation and generalized edema. Ophthalmic symptoms could be the initial presentation of lupus protein‑losing enteropathy. Careful systemic review and early referral may avoid life‑threatening complications.
Financial Support and Sponsorship
2.
3.
Negi A, Marmor MF. Effects of subretinal and systemic osmolality on the rate of subretinal fluid resorption. Invest Ophthalmol Vis Sci 1984;25:616‑620. Cheng LL, Kwok AK, Wat NM, Neoh EL, Jon HC, Lam DS. Graft‑vs‑host‑disease‑associated conjunctival chemosis and central serous chorioretinopathy after bone marrow transplant. Am J Ophthalmol 2002;134:293‑295.
This is an open access article distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as the author is credited and the new creations are licensed under the identical terms.
Access this article online Quick Response Code:
Website: www.jovr.org
Nil. DOI: 10.4103/jovr.jovr_117_16
Conflicts of Interest There are no conflicts of interest.
REFERENCES 1.
246
Al‑Mogairen SM. Lupus protein‑losing enteropathy (LUPLE): A systemic review. Rheumatol Int 2011;31:995‑1001.
How to cite this article: Wong HYA, Li TLS, Ho CK, Yiu PF. Bilateral Serous macular elevation associated with lupus protein-losing enteropathy. J Ophthalmic Vis Res 2017;12:245-6. © 2017 Journal of Ophthalmic and Vision Research | Published by Wolters Kluwer ‑ Medknow
Journal of Ophthalmic and Vision Research Volume 12, Issue 2, April-June 2017
