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Clinical profile of serpiginous choroiditis

by | May 1, 2021 | Manuscripts | 0 comments

Nandhini Elango, Bachelor’s in Optometry

Junior Research Fellow, Sankara Nethralaya, Chennai, India

 

Introduction

 Serpiginous choroiditis (SC) is a recurrent, asymmetrically bilateral inflammation of the choroid that leads to loss of choriocapillaris, atrophy, or loss of overlying retinal pigment epithelium (RPE), degeneration, and loss of photoreceptor cells with adhesion of the degenerated retina with the choroid (Refer figure 1). (1)

 

Figure 1: Various morphological variant of SC: (a) peripapillary classic serpiginous choroiditis, (b) macular classic serpiginous choroiditis, (c) multifocal serpiginoid choroiditis, and (d) serpiginous-like choroiditis involving peripheral retina.

Image courtesy:  Majumder, P. D., Biswas, J., & Gupta, A. (2019). Enigma of serpiginous choroiditis. Indian journal of ophthalmology, 67(3), 325.

Epidemiology

SC is a relatively rare clinical entity, prevalence ranging from 0.2% to 5% of all uveitis patients. It has a higher prevalence in men and affects young to middle aged adults. (1, 3)

Aetiology

  • A majority of SC remains idiopathic.
  • An intraocular inflammation with an autoimmune response specific to the ocular tissue has been observed in Classic serpiginous choroiditis (CSC). (4)
  • Various infectious aetiologies has been suggested by many authors such as Mycobacterium tuberculosis is the most common aetiology in serpiginous like choroiditis (SLC) (6). Other microorganisms like Toxoplasma gondii, candida sp, Treponema pallidum, and virus were seen in SC. (4, 5)

Clinical features

Patient with SC may present with

  • Blurred vision,
  • Metamorphosia,
  • Scotoma,
  • Floaters,
  • Visual field defects (3)

The word serpiginous from Latin “serpere – to creep” is characterized by wavy or amoeboid – like lesions in choroid. The lesions are characterized by ill-defined patches of grayish-white or creamy yellow colour discoloration at the level of the deep retina and retinal pigment epithelium (RPE). (2)

 

Investigation

Fundus autofluorescence

  • Active choroiditis – Hyperautofluorescence patches with hypoautofluorescent margin or granular Hyperautofluorescence.
  • Healed choroiditis – uniform hypoautofluorescent patch with sharp margins (Refer figure: 2).

  Fundus fluorescein angiography

  • Active choroiditis – Late phase angiogram: borders show hyperfluorescence may spread centrally to form a uniform or spotty appearance in the entire lesion.
  • Healed choroiditis – Late phase angiogram: hypofluorescent patch with sharp staining of the margins (Refer figure 3).

Indocyanine green angiography

  • Active choroiditis – Early and late hypoautofluorescence with ill-defined margins or early hypoautofluorescence with increased fluorescence towards the late phase with faint edges.
  • Healed choroiditis – Early and late hypofluorescence with well-defined margins.   

Optical coherence tomography (OCT)

  • Active choroiditis – slightly increased retinal thickness (retinal edema).
  • Healed choroiditis – Thin retina and Loss of outer retinal architecture.

 

Figure 2: (a) Fundus photograph of the left eye showing multifocal choroidal lesions of varying shape and size and at various stages of resolution in a patient with serpiginous-like choroiditis and (b) autofluorescence of the left eye showing variegated pattern of hypoautofluorescence and hyperautofluorescence (yellow arrow). Note the healed choroiditis lesions (white arrows) characterized by total hypoautofluorescence area with sharp borders.

Image courtesy:  Majumder, P. D., Biswas, J., & Gupta, A. (2019). Enigma of serpiginous choroiditis. Indian journal of ophthalmology, 67(3), 325.

 

Figure 3: Color fundus photograph (a) and early (b and d) and late-phase (c and e) fundus fluorescein angiography and indocyanine green angiography pictures of serpiginous-like choroiditis. Active choroiditis appears as hypofluorescence with fuzzy, irregular borders in early phase (b), followed by profuse leakage of the dye leading to hyperfluorescence in late phase of fundus fluorescein angiography (c). Active lesions in indocyanine green angiography show blockage of the dye beginning from the early phase (d) to the late phase (e).

Image courtesy:  Majumder, P. D., Biswas, J., & Gupta, A. (2019). Enigma of serpiginous choroiditis. Indian journal of ophthalmology, 67(3), 325.

Complications

The commonest complications associated with SC is choroidal neovascularization (CNV). It ranges from 10% to 25%.(7) The other complications are retinal vasculitis, vascular occlusions, secondary neovascularization, vitreous haemorrhage, serous retinal detachment, and cystoid macular edema (CME) which are less commonly seen. (1, 8)

Differential diagnosis

The main differential diagnoses are conditions that affect the choriocapillaris, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), tuberculosis, outer retinal toxoplasmosis, and choroidal ischemia (13).

Treatment

  • Treatment may involve corticosteroid, immunosuppressive agents, intravitreal corticosteroid injection and biologicals. (9-11)
  • Anti-tubercular treatment (ATT) along with oral corticosteroids or immunosuppressive agents has been proven to control active inflammation as well as future recurrence in patients with characteristic clinical lesions and suggestive history such as contact with TB patients or origin from TB endemic region. (12)

 

Conclusion 

SC is the recurrent, bilateral, and progressive inflammation involving the outer retina and inner choroid. Spectral domain OCT and novel imaging techniques such as FAF have improved diagnostic accuracy, management, follow-up, and determination of the visual prognosis. The main treatment goal being to suppress active inflammation and reduce the number of recurrences, one must exercise sufficient caution prior administering local immunomodulatory therapy and the need for periodic follow-up and self-examination of central vision by Amsler chart should be emphasized.

 

Reference

  1. Majumder, P. D., Biswas, J., & Gupta, A. (2019). Enigma of serpiginous choroiditis. Indian journal of ophthalmology, 67(3), 325.
  1. Khanamiri, H. N., & Rao, N. A. (2013). Serpiginous choroiditis and infectious multifocal serpiginoid choroiditis. Survey of ophthalmology, 58(3), 203-232.                        
  1. Abrez, H., Biswas, J., & Sudharshan, S. (2007). Clinical profile, treatment, and visual outcome of serpiginous choroiditis. Ocular immunology and inflammation, 15(4), 325-335.
  1. Erkkilä, H., Laatikainen, L., & Jokinen, E. (1982). Immunological studies on serpiginous choroiditis. Graefe’s Archive for Clinical and Experimental Ophthalmology, 219(3), 131-134.
  1. Maumenee, A. E. (1970). Clinical Entities in “Uveitis”: An Approach to the Study of Intraocular Inflammation: XXVI Edward Jackson Memorial Lecture. American journal of ophthalmology, 69(1), 1-27.
  1. Gupta, A., Bansal, R., Gupta, V., Sharma, A., & Bambery, P. (2010). Ocular signs predictive of tubercular uveitis. American journal of ophthalmology, 149(4), 562-570.
  1. Kuo, I. C., & Cunningham Jr, E. T. (2000). Ocular neovascularization in patients with uveitis. International ophthalmology clinics, 40(2), 111-126.
  1. Gupta, V., Gupta, A., & Rao, N. A. (2007). Intraocular tuberculosis—an update. Survey of ophthalmology, 52(6), 561-587.
  1. Akpek, E. K., Baltatzis, S., Yang, J., & Stephen Foster, C. (2001). Long-term immunosuppressive treatment of serpiginous choroiditis. Ocular immunology and inflammation, 9(3), 153-167.
  1. Adıgüzel, U., Sarı, A., Özmen, C., & Öz, Ö. (2006). Intravitreal triamcinolone acetonide treatment for serpiginous choroiditis. Ocular immunology and inflammation, 14(6), 375-378.
  1. Chinchurreta Capote, A., Requena Jimenez, J. M., Lorenzo Soto, M., Romero Gomez, C., & García de Lucas, M. D. (2014). Effectiveness of adalimumab for refractory serpiginous choroiditis. Ocular immunology and inflammation, 22(5), 405-408.
  1. Bansal, R., Gupta, A., Gupta, V., Dogra, M. R., Bambery, P., & Arora, S. K. (2008). Role of anti-tubercular therapy in uveitis with latent/manifest tuberculosis. American journal of ophthalmology, 146(5), 772-779.
  2. Lim, W. K., Buggage, R. R., & Nussenblatt, R. B. (2005). Serpiginous choroiditis. Survey of ophthalmology, 50(3), 231-244.

 

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