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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 929-931

A case of bilateral cystoid macular edema after rituximab infusion


2nd Department of Ophthalmology, University of Athens Medical School, 'Attikon' General Hospital Athens, Haidari, Athens, Greece

Date of Submission13-Apr-2022
Date of Acceptance14-Jun-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Nikolaos Gouliopoulos
2nd Department of Ophthalmology, University of Athens Medical School, 'Attikon' General Hospital Athens, Greece, 1 Rimini Str, Haidari, Haidari, Athens 12462
Greece
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_951_22

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  Abstract 


A 65-year-old man experienced sudden bilateral vision loss after rituximab infusion. Best corrected visual acuity was 1/10, and cystoid macular edema was identified. A bilateral rituximab-induced cystoid macular edema was diagnosed, and an intravitreal dexamethasone implant was applied. The exudative phenomena resolved, and the visual acuity rose to 8/10. Another rituximab infusion was followed by vision loss and recurrence of macular edema. A dexamethasone implant was reapplied, which showed great results. This is a rare case of rituximab-induced bilateral macular edema, which recurred after a second infusion. We also present an alternative treatment consisting of intravitreal dexamethasone implant.

Keywords: Dexamethasone Implant, IgG4-related disease, macular edema, rituximab


How to cite this article:
Rouvas A, Bouratzis N, Gouliopoulos N. A case of bilateral cystoid macular edema after rituximab infusion. Indian J Ophthalmol Case Rep 2022;2:929-31

How to cite this URL:
Rouvas A, Bouratzis N, Gouliopoulos N. A case of bilateral cystoid macular edema after rituximab infusion. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Nov 27];2:929-31. Available from: https://www.ijoreports.in/text.asp?2022/2/4/929/358199



IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disorder which affects multiple organs.[1] Rituximab (RTX), an anti-CD20 monoclonal B-cell depleting antibody, has been identified as an effective treatment of IgG4-RD.[2] Several side effects have been attributed to RTX, including fever, hives, rashes during infusions, breathing difficulties, and susceptibility to infections.[3]

Herein, we describe a case of bilateral cystoid macular edema due to RTX infusion, which was successfully resolved after the application of intravitreal dexamethasone (DEX) implant (Ozurdex; Allergan, Inc., Irvine, CA, USA). None of the previous cases of RTX-induced macular edema were treated with the application of a DEX implant.


  Case Report Top


A 65-year-old man referred to our department 10 days after the initiation of bilateral visual deterioration and metamorphopsia. His medical history revealed that the symptoms started 1 day after RTX infusion, which was applied for the treatment of IgG4-RD. The rest of his systemic/ocular history was unremarkable, while his laboratory evaluation values were within the normal range for the patient's age.

At presentation, in both eyes, best corrected visual acuity (BCVA) was 1/10 and intraocular pressure was 16 mmHg, while no significant abnormalities of the anterior segment were detected, except for a grade 1 nuclear opacity of the lens. Fundoscopy revealed the presence of macular edema, while ocular ultrasonography did not identify any abnormalities. Optical coherence tomography (OCT) scans showed almost symmetrical intraretinal fluid (IRF) and subretinal fluid (SRF) bilaterally [Figure 1]a and [Figure 1]b. Fluorescein angiography (FA) demonstrated bilateral cystoid macular edema during the late phases [Figure 1]c,[Figure 1]d,[Figure 1]e,[Figure 1]f. Indocyanine green angiography (ICGA) was not helpful regarding the fluid origin [Figure 1]g and [Figure 1]h.
Figure 1: At presentation, optical coherence tomography scans revealed the presence of intraretinal and subretinal fluid in the right (OD) (a) and left (OS) (b) eyes. Fluorescein angiography identified the presence of cystoid macular edema during the late phases in OD (c and e) and OS (d and f). Indocyanine green angiography could not identify the fluid origin (g and h)

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No common causes of macular edema could be recognized by the ocular and clinical examination. More specifically, our patient did not suffer from diabetes mellitus or malignant hypertension; his clinical examination and laboratory work up were unremarkable, which included complete blood count, angiotensin-converting enzyme (ACE), lysozyme, anti-neutrophil cytoplasmic antibodies (ANCA), and antinuclear antibodies (ANA) levels. No cases of macular edema associated with IgG4-RD have been detected previously. However, it has been suggested that RTX infusion may be followed by macular edema development, as it has been shown in four cases.[3],[4],[5] Taking into account the short interval between RTX infusion and the initiation of macular disorders, a causative link is plausible. Thus, a diagnosis of bilateral RTX-induced cystoid macular edema was made.

Treatment consisted of intravitreal DEX implant application in both eyes. Two months later, our patient was re-examined. Bilaterally, BCVA rose to 8/10, intraocular pressure was 18 mmHg, the lens opacities remained stable, while OCT scans revealed resolution of IRF and SRF.

The situation remained stable for 3 months, when our patient underwent another RTX infusion. He experienced the same visual disturbances and referred to our department 1 week later. New BCVA was 3/10 and the OCT examination revealed the recurrence of IRF and SRF bilaterally. A DEX implant was reapplied, and the treatment resulted in complete regression of the exudative phenomena 1 month later [Figure 2] and [Figure 3]. BCVA was 6/10 bilaterally because of retinal pigment epithelium metaplasia and absence of inner/outer segment layer in central fovea. The patient has been monitored monthly; 8 months later, the situation remains unchanged.
Figure 2: Infrared fundus photography and optical coherence tomography scans of the right eye at the initial presentation (a) and 1 month after the second application of intravitreal dexamethasone implant (b). Initial intraretinal and subretinal fluid completely resolved, while metaplasia of retinal pigment epithelium and absence of inner/outer segment layer in the region of the central fovea were also detected (b)

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Figure 3: Infrared fundus photography and optical coherence tomography scans of the left eye at the initial presentation (a) and 1 month after the second application of intravitreal dexamethasone implant (b). Initial intraretinal and subretinal fluid completely resolved, while metaplasia of retinal pigment epithelium and absence of inner/outer segment layer in the region of the central fovea were also detected (b)

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  Discussion Top


Herein, we describe a rare case of bilateral IRF and SRF development following RTX infusion. Moreover, this is the first time in literature that this type of edema was resolved with the application of an intravitreal DEX implant.

Cystoid macular edema may develop following an intraocular surgery or due to multiple pathological conditions, including diabetic retinopathy, central/branch retinal vein occlusion, intraocular inflammation, infectious, infiltrative, medication-induced, idiopathic, and hereditary causes.[5] Our patient had not undergone an ocular surgery, while he did not suffer from diabetes mellitus or any other systemic disease, except for IgG4-RD. Furthermore, the clinical examination ruled out the presence of any ocular cause that could result in macular edema. There is one previous case of a patient suffering from IgG4-RD, in whom unilateral SRF was present due to orbital infiltration by IgG4-predominant plasma cells.[6] However, in our patient, no signs of orbital involvement were detected.

In the literature, four cases of macular edema due to RTX infusion have been reported;[3],[4],[5] two patients suffered from granulomatosis with polyangiitis,[3] one from IgG4-RD,[5] and one from antibody-mediated rejection in a kidney transplant recipient.[4] It is worthy to note that in one case, a second RTX infusion was accompanied by a recurrence of cystoid macular edema,[3] an adverse effect that our patient also encountered. Although the exact pathophysiology of RTX-induced cystoid macular edema is yet to be elucidated, it has been suggested that local cytokine release and accumulation may be the possible linking mechanisms.[5]

RTX-induced macular edema was successfully treated in the previously described cases. In one case, subtenon injection of triamcinolone (40 mg) resulted in BCVA improvement and macular edema resolution.[3] In another case, intravenous injections of methylprednisolone (500 mg for 3 days) were administered and oral intake of corticosteroids and azathioprine was recommended due to scleral thinning. Although macular edema persisted for 10 months, it finally receded and the BCVA rose to 10/10.[3] Two other patients were treated with intravitreal injections of triamcinolone,[4],[5] which showed excellent results. Our patient was treated with intravitreal DEX implant in both eyes and experienced significant visual and anatomical gains.


  Conclusion Top


In conclusion, we document that RTX infusion may be accompanied by macular edema development and the clinicians should be alerted about this uncommon adverse effect, underlying the importance of early recognition and prompt treatment in cases of RTX-induced macular edema. We also presented an alternative treatment, consisting of intravitreal DEX implant, in order to achieve complete edema regression.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181-92.  Back to cited text no. 1
    
2.
Ebbo M, Grados A, Samson M, Gro M, Loundou A, Rigolet A, et al. Long-term efficacy and safety of rituximab in IgG4-related disease: Data from a French nationwide study of thirty-three patients. PLoS One 2017;12:e0183844.  Back to cited text no. 2
    
3.
Bussone G, Kaswin G, de Menthon M, Delair E, Brezin AP, Guillevin L. Macular oedema following rituximab infusion in two patients with Wegener's granulomatosis. Clin Exp Rheumatol 2010;28:90-92.  Back to cited text no. 3
    
4.
Juric I, Juric Tomasevic B, Basic-Jukic N. Unique case of macular edema following rituximab treatment for antibody-mediated rejection in kidney transplant recipient. Ther Apher Dial 2021;25:361-2.  Back to cited text no. 4
    
5.
Gilca M, Merrill PT. Cystoid macular edema secondary to rituximab. Retin Cases Brief Rep 2019;13:111-4.  Back to cited text no. 5
    
6.
Karamchandani JR, Younes SF, Warnke RA, Natkunam Y. IgG4-related systemic sclerosing disease of the ocular adnexa: A potential mimic of ocular lymphoma. Am J Clin Pathol 2012;137:699-711.  Back to cited text no. 6
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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