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Multifocal Choroiditis with Panuveitis along with Subretinal Fibrosis and Uveitis Syndrome

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Title: Multifocal Choroiditis with Panuveitis along with Subretinal Fibrosis and Uveitis Syndrome
Author (s): Samuel Levant, BS, MS-4 at Emory University; Joseph Simonett, MD; Wen Fan Hu, MD; Albert Vitale, MD
Photographer: Jim Gilman, CRA, FOPS
Date: 07/2021

Keywords/Main Subjects: Multifocal Choroiditis, Panuveitis, Subretinal Fibrosis, Uveitis, White Dot Syndromes

Diagnosis: Multifocal Choroiditis with Panuveitis as well as Subretinal Fibrosis and Uveitis Syndrome

Description of Case:

A 37-year-old woman carrying a diagnosis of multifocal choroiditis with panuveitis (MCP) along with subretinal fibrosis and uveitis syndrome (SFU) returned to clinic in June 2021. The patient initially presented in 2010 with asymmetric blurry and decreased vision, scotomas, and an enlarged blind spot in her left eye. Within one month of initial presentation, she had bilateral anterior inflammation and round pale-yellow choroidal lesions in the posterior pole and periphery in her left eye on examination. On OCT in the left eye, the patient had growing macular lesions in the IS/OS junction with choroidal hyperreflectivity, and on FAF, the patient had large and small areas of hypoautofluorescence correlating with areas of chorioretinal scarring and punched out chorioretinal lesions, respectively. In her right eye, the patient had only subtle pigmentary changes. Systemic evaluation with laboratory testing and chest imaging was unremarkable.

The disease progressed rapidly in the patient’s left eye before stabilizing with oral and topical steroids and immunomodulatory therapy (IMT), specifically methotrexate. For the decade after her initial flare, she remained stable and with only in the left eye affected. She used methotrexate for less than one year before stopping IMT due to pregnancy. By late 2020, the patient’s left eye had significant scarring and fibrosis on fundus exam. Her left eye had large areas of hypoautofluorescence centrally and numerous spotted areas peripherally on FAF along with subretinal hyperreflective material on OCT and large areas of hypofluorescence around the macula and arcades on ICG. At this time, the patient developed new inflammatory lesions in her right eye with sub-RPE deposits threatening the fovea on OCT and new hyperautofluorescent lesions parafoveally and in the macula on FAF. Repeat systemic work-up for infectious and inflammatory conditions was performed and again found to be unrevealing. She was treated aggressively due to her history of rapid progression in the left eye: over the next few months she received high dose oral steroids, topical steroids, a dexamethasone intravitreal implant, mycophenolate mofetil, adalimumab, and four injections of intravitreal aflibercept due to presence of secondary CNV. Eventually, the inflammatory lesions stabilized and vision started to improve with these interventions. The patient was seen in June 2021 as she was tapering off her oral prednisone.

Discussion:

Multifocal choroiditis with panuveitis (MCP) is a bilateral and progressive chronic, recurrent multifocal chorioretinal inflammatory disease that features prominent intraocular inflammation including anterior segment cell and vitritis. The disorder lacks a clear etiology with possible viral, genetic, and autoimmune associations. It is characterized by numerous round or oval yellow to white choroidal lesions ranging from 50-350 μm at the posterior pole or midperipheral fundus that occur individually or in clusters or streaks. These active posterior lesions can be associated with optic nerve inflammation and optic neuropathy, cystoid macular edema (CME), and choroidal neovascular membranes (CNVM). Occasionally, rapidly progressive subretinal fibrosis localized to the posterior pole can occur over weeks to months in association with the intraocular inflammation, so called subretinal fibrosis and uveitis syndrome. This rare subtype of the disease can be particularly visually devastating and requires aggressive treatment in its active stage. The inactive lesions appear as punched-out areas of pigmented chorioretinal atrophy. Anterior inflammation – presenting as AC cell and flare, nongranulomatous keratic precipitates, posterior synechiae, and cataracts – helps differentiate MCP from other chorioretinal diseases.

The disease has a predilection for myopic women, at three times the rate of men, typically in their third decade of life (though a range from 9-69 is reported). Neither race nor past medical or ocular history appear to affect the risk of MCP, but patients with MCP are more likely to have a personal or familial history of autoimmune disease.

A patient may present with complaints of asymmetric blurry vision, scotomas, photopsia, floaters, and enlarged blind spots.  There are no specific tests to establish a diagnosis of MCP, so diagnosis is made clinically with a thorough history, ocular exam, and testing to rule out other potential inflammatory and infectious diagnoses. Included in the differential is sarcoidosis, birdshot retinochoroidopathy, multiple evanescent white dot syndrome, presumed ocular histoplasmosis syndrome, punctate inner choroidopathy, acute posterior multifocal placoid pigment epitheliopathy, primary CNS lymphoma, VKH, syphilis, tuberculosis, Lyme disease, and viral retinitis.

MCP tends to recur in one or both eyes without treatment with nearly half of all patients developing CNVMs and 60-75% resulting in permanent vision loss. The prognosis is mixed and appears dependent on the early diagnosis, initiation, and maintenance of treatment to prevent the chronic and recurrent inflammation that leads to permanent vision loss. Both systemic and local corticosteroids are often used to treat the acute inflammation in MCP, but their effect is temporary. Immunomodulatory therapy (IMT) is the primary modality for longterm treatment and is indicated with foveal threatening lesions, recurrent disease, or new inflammatory lesions in a previously unaffected eye in situations where the fellow eye has had poor outcomes. Commonly employed first-line agents include the antimetabolites (methotrexate, azathioprine, and mycophenolate mofetil) with low threshold for the use of TNF-α inhibitors (adalimumab or infliximab) as second line therapy for recurrent disease. With IMT, there is an 82% reduced risk of posterior pole complications and a 92% reduced risk of development of visual acuity worsen than 20/200. Laser photocoagulation, intravitreal anti-VEGF, and photodynamic therapy are all effective in treating existing CNVMs.

 Images or video:

Levant, Simonett, Vitale- 5.4.10 FAF OS

Figure 1. FAF OS 05/2010 with numerous small hyperautofluorescent spots

Levant, Simonett, Vitale- 6.26.10 FAF OS

Figure 2. FAF OS on 06/2010 with larger central hyperautofluorescent area and many smaller lesions

Levant, Simonett, Vitale- 6.15.21 FAF OS

Figure 3. FAF OS on 6/2021 showing large area of central hypoautofluorescence with numerous peripheral lesions

Levant, Simonett, Vitale- 5.4.10 pic OS

Figure 4. initial fundus photograph OS on 5/2010 with pale-yellow round lesions centrally and peripherally

Levant, Simonett, Vitale- 7.26.10 pic OS
Figure 5. fundus photograph OS on 07/2010 showing rapid progression of lesions from 5/2010

Levant, Simonett, Vitale- 4.6.21 pic OS
Figure 6. fundus photograph OS on 4/2021 with central subretinal fibrosis and numerous punched-out atrophic chorioretinal lesions

Levant, Simonett, Vitale- 3.25.21 FA OS

Figure 7. FA transit OD on 3/2021 with staining of lesions OS including large area of submacular fibrosis

 

Summary of the Case:

This 37-year-old female diagnosed with multifocal choroiditis with panuveitis as well as subretinal fibrosis and uveitis syndrome presented in 2010 and rapidly progressed in her left eye with numerous round yellow choroidal lesions at the posterior pole and midperipheral fundus along with prominent anterior inflammation and unremarkable lab work-up. She remained relatively quiet over the next decade until the disease recurred in her other eye and required very aggressive intervention to stabilize.

Format: Case report

References:

  1. Standardization of Uveitis Nomenclature (SUN) Working Group. Classification Criteria for Multifocal Choroiditis With Panuveitis. Am J Ophthalmol. 2021;228:152-158.
  2. Haen SP, Spaide RF. Fundus autofluorescence in multifocal choroiditis and panuveitis. Am J Ophthalmol. 2008;145(5):847-853.
  3. Thorne JE, Wittenberg S, Jabs DA, et al. Multifocal choroiditis with panuveitis incidence of ocular complications and of loss of visual acuity. Ophthalmology. 2006;113(12):2310-2316.
  4. Kedhar SR, Thorne JE, Wittenberg S, Dunn JP, Jabs DA. Multifocal choroiditis with panuveitis and punctate inner choroidopathy: comparison of clinical characteristics at presentation. Retina. 2007;27(9):1174-1179.
  5. Moses KN. Multifocal Choroiditis and Panuveitis. In: Uveitis: A Quick Guide to Essential Diagnosis. Cham, Switzerland: SPRINGER; 2020:81-84.
  6. Foster CS, Vitale AT, Vitale AT, Shakoor A. Multifocal Choroiditis and Panuveitis. In: Diagnosis and Treatment of Uveitis. Second ed. New Delhi: Jaypee Brothers Medical; 2013:1033-1049.

Faculty Approval by: Albert Vitale, MD

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