Serpiginous choroidopathy

From EyeWiki


Serpiginous choroidopathy (SC) is a rare, bilateral, chronic, progressive, recurrent inflammatory disease of the retinal pigment epithelium (RPE), choriocapillaris and choroid of unknown etiology.

Etiology

SC is a rare clinical entity causing less than 5% of posterior uveitis cases. It has a higher prevalence in men and affects young to middle-aged adults. No systemic disease associations have been identified.

Pathophysiology

Although of unknown etiology, its origin is probably immunogenic since it seems to respond to treatment with cortisteroids and other immunossupressants. Moreover, affected patients also show an increased frequency of HLA-B7 and retinal S-antigen associations. Other pathogenic mechanisms have been proposed and several authors link SC to infectious agents like Mycobacterium tuberculosis and herpes viruses but this remains to be proven.

Symptoms

Patients present with a painless unilateral vision loss, metamorphopsia or central scotoma.

Diagnosis

SC presents with gray-yellowish subretinal infiltrates that usually spread centrifugally from the peripapillary region in a serpiginous (snake-like) manner. Active lesions show a leading edge and resolve with subsequent RPE and choriocapillary atrophy. Consecutive recurrences cause further atrophy leaving hypo and hyperpigmented lesions that spread irregularly over the posterior fundus. Although bilateral, the disease is often asymmetric with multiple lesions in different stages of resolution in both eyes. Recurrences have variable intervals that range from months to years. Anterior chamber and vitritis are minimal. Others forms of SC include Macular serpiginous choroidopathy and Ampiginous choroidopathy (also known as relentless placoid choriorretinitis). The former begins as a macular lesion that spares the peripapillary region with a higher risk of choroidal neovascularization (CNV) and poor visual outcome. Ampiginous choroidopathy is an atypical form of SC characterized by multifocal plaque-like lesions scattered over the posterior pole that resemble acute posterior multifocal placoid pigment epiteliopathy (APMPPE) but without the spontaneous resolution typical of this white-dot syndrome showing multiple areas of atrophy that become contiguous over time.

Diagnostic procedures

Ancillary testing in SC include fluorescein angiography (FA), indocyanine green angiography (ICG) and fundus autofluorescence (FAF). In active lesions occurring at the margins of an atrophic lesion, FA typically shows early hypofluorescence with late leakage. As in other inflammatory choroidopathies, ICG is characterized by hypofluorescent areas from early to late frames. FAF has become a very useful and minimally invasive tool for monitoring disease progression in SC. Active lesions show a hypoautofluorescent halo surrounding the edges of a hyperautofluorescent lesion. Semi-active or transitional lesions show a linear hypoautofluorescence surrounding all edges of the hyperautofluorescent lesion. Quiet or inactive lesions are dark and uniformly hypoautofluorescent.

Differential diagnosis

The differential diagnosis includes other causes for posterior uveitis that course with multifocal or placoid choriorretinitis like Multifocal Choriorretinitis, APMPPE, Toxoplasmosis and Tuberculosis (TB). TB has recently been associated with the development of a choroidopathy that mimics SC commonly named serpiginous-like choroidopathy (SLC). This disease probably represents a hypersensitivity reaction to Mycobacterium tuberculosis infection, which leads to the development of a chorioretinitis that is similar to SC. This poses a diagnostic challenge since these two disease entities have a very different therapeutic approach and antibacillary treatment associated with oral corticosteroids is mandatory for SLC in order to avoid future recurrences. Recent works claim that SLC can be distinguished from SC because the lesions are noncontiguous to the optic disc, are frequently multifocal with a more peripheral involvement, tend to spare the fovea even in eyes with macular involvement and commonly course with vitritis. SLC may not respond to sole treatment with steroids and/or other immunossupressants making TB testing (with tuberculin skin testing or QuantiFERON-TB Gold) mandatory, especially when in endemic regions.

Medical therapy

Treatment of SC aims to stop chorioretinal inflammation especially when the advancing lesions threaten the fovea. Systemic or periocular corticosteroids are frequently used but recurrence prevention usually requires long term anti-inflammatory treatment with a steroid-sparing agent like cyclosporine A and/or azathioprine. Long term management can be challenging and up to 25% of the eyes have a final visual acuity less than 20/200.

Complications

The most common complication of SC is choroidal neovascularization affecting up to 35% of patients. Other reported complications are subretinal fibrosis, cystoid macular edema, branch vein occlusion, serous retinal detachment, optic disk neovascularization and anterior uveitis.

Prognosis

SC is a challenging disease, probably autoimmune in origin and frequently managed with corticosteroids and other immunossupressants. Tuberculosis testing is important in this setting since SLC has a different therapeutic approach and clinical course.

Additional Resources

References

1)Bacin F, Larmande J, Boulmier A, Juliiar G. Serpiginous choroiditis and placoid epitheliopathy. Bull Soc Opthalmol Fr. 1983; 83:1153-62

2)Abu el-Asrar AM. Serpiginous (geographical) choroiditis. Int Ophthalmol Clin. 1995; 35:87-91

3)Ciulla TA, Gragoudas ES. Serpiginous choroiditis. Int Ophthalmol Clin. 1996; 36:135-43.

4)Chang JH, Wakefield D. Uveitis: a global perspective. Ocul Immunol Inflamm. 2002; 10(4):263-279.

5)Akpek EK, Jabs DA, Tessler HH, Joondeph BC, Foster CS. Sucessful treatment of serpiginous choroiditis with alkylating agents. Ophthalmology. 2002; 109(8): 1506-13.

6)Christmas NJ, Oh KT, Oh DM, Folk JC. Long-term follow-up of patients with serpiginous choroiditis. Retina. 2002; 22(5): 550-6.

7)Weiss H, Annesley WH, Shiels JA, et al. The clinical course of serpiginous choroidopathy. Am J Ophthalmol. 1979. 87:133-42.

8)Erkkila H, Laatikainen L, Jokinen E. Immunological studies on serpiginous choroiditis. Graefes Arch Clin Exp Ophthalmol. 1982; 219:131-4.

9)Broekhuyse RM, van Herck M, Pinckers AJ, et al. Immune responsiveness to retinal S-antigen and opsin in serpiginous choroiditis and other retinal diseases. Doc Ophthalmol. 1988; 69: 83-93.

10)Priya K, Madhavan HN, Reiser BJ, et al. Association of herpesviruses in the aqueous humor of patients with serpiginous choroiditis: a polymerase chain reaction-based study. Ocul Immunol Inflamm. 2002; 10(4):253-261.

11)Bansal R, Gupta A, Gupta V, Dogra M, Sharma A, Bambery P. Tubercular serpiginous-like choroiditis presenting as multifocal serpiginoid choroiditis. Ophthalmology. 2012; 119(11): 2334-42.

12)Carreno E, Portero A, Herreras JM, Lopez MI. Assesment of fundus autofluorescence in serpiginous and serpiginous-like choroidopathy. Eye. 2012; 26: 1232-36.

13)Gupta V, Agarwal A, Gupta A, et al. Clinical characteristics of serpiginous choroidopathy in North India. Am J Ophthalmol. 2002; 134: 47-56.

14)Steinmetz RL, Fitzke FW, Bird AC. Treatment of cystoids macular edema with acetazolamide in a patient with serpiginous choroidopathy. Retina. 1991; 11:412-5.

15)Blumenkranz MS, Gass JD, Clarkson JG. Atypical serpiginous choroiditis. Arch Ophthalmol. 1982; 100: 1773-5.

16)Hoyng C, Tilanus M, Deutman A. Atypical central lesions in serpiginous choroiditis treated with oral prednisone. Graefes Arch Clin Exp Ophthalmol. 1998; 236: 154-6.

17)Laatikainen L, Erkkila H. Subretinal and disc neovascularization in serpiginous choroiditis. Br J Ophthalmol. 1982; 66: 326-31.

18)Masi RJ, O´Connor GR, Kimura SJ. Anterior uveitis in geographic or serpiginous choroiditis. Am J Ophthalmol. 1978; 86: 228-32.