Ocular Immunology and Inflammation, 14:29–33, 2006Copyright ©c Taylor & Francis Group, LLCISSN: 0927-3948 print; 1744-5078 onlineDOI: 10.1080/09273940500227192

ORIGINAL REPORT

Intravenous Pulse MethylprednisoloneTherapy for Acute Treatment of Serpiginous

ChoroiditisNikos N. Markomichelakis,Ioannis Halkiadakis,Simina Papaeythymiou-Orchan,Nikos Giannakopoulos,Nikos Ekonomopoulos,and Tasos KourisOcular Immunology andInflammation Service,Department of Ophthalmology,General Hospital of Athens,Athens, Greece

ABSTRACT Purpose: To evaluate the safety and efficacy of high-dose intra-venous steroid therapy (HDIST) for the acute treatment of vision-threateningserpiginous choroiditis. Methods: Retrospective review of the records of five pa-tients with serpiginous choroiditis who were treated with HDIST (1 g methyl-prednisolone for three days) in addition to their standard immunosuppressivetreatment. The visual acuities and improvement of ocular signs after HDISTwere evaluated. Results: Twelve episodes of macula-threatening choroiditis infive patients with serpiginous choroiditis were treated during a seven-year pe-riod. All patients responded to HDIST with evidence of a decrease in intraoc-ular inflammation immediately after and complete restoration of visual acuitywithin 10 days of commencing treatment. In one patient, medical interven-tion was required because of gastric distress. During the follow-up, three outof five patients experienced new attacks and two patients developed subretinalneovascularization. Conclusion: HDIST is effective in controlling severe vision-threatening serpiginous choroiditis and in improving visual function in a shortperiod of time. However, the effect of this treatment in long-term disease controlis uncertain.

KEYWORDS Intravenous pulse methylprednisolone; ocular inflammation; serpiginouschoroiditis

INTRODUCTIONSerpiginous choroiditis is a recurrent inflammatory disorder of the choroid

and the pigment epithelium. Visual prognosis is guarded due to involvementof the macula or the development of chorioretinal neovascularization (CNV).1

Corticosteroids used periocularly or systematically comprise the mainstay oftreatment of active disease. These drugs may also prevent recurrent disease; how-ever, patients cannot tolerate high doses for prolonged periods and recurrencesoccur as the dose is tapered. It has been shown that combination immuno-suppressive treatment is effective in reducing the number of recurrences.1,2

Accepted 20 June 2005.

Presented in part at the Associationfor Research in Vision andOphthalmology Annual Meeting, FortLauderdale, Florida, May 2003.

Acknowledgment: IoannisHalkiadakis, M.D., was financiallysupported by the Lilian VoudouriFoundation, Athens, Greece.

Correspondence and reprint requeststo: Nikos Markomichelakis, M.D.,Ocular Immunology andInflammation, General Hospital ofAthens, 106 Kykladon Street, Ag.Paraskevi, 15341, Athens, Greece.Tel/Fax: +30-210-7489454; e-mail:marnik@otenet.gr

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In certain cases, however, previous forms of corti-costeroid therapy cannot achieve adequate control ofthe inflammation.3 Initially, Nussenblatt (in Hooper& Kaplan3) supported the view that higher doses ofsteroids may achieve accelerated control of inflamma-tion and advocated the use of high-dose intravenoussteroid therapy (HDIST) (1 g intravenous methylpred-nisolone daily for three days) for macula-threateningcases of serpiginous choroiditis. In the present study,we report the results of using HDIST in five patientswith serpiginous choroiditis.

METHODSThe records of all patients who presented in our in-

stitution between January 1995 and January 2002 withthe diagnosis of serpiginous choroiditis were reviewedand five patients in whom HDIST was used were iden-tified. Twelve episodes of choroiditis were treated. Inall five patients, intravenous methylprednisolone (1 g)was administered over one hour for three days followedby initiation of oral methylprednisolone (0.5 mg/kg/d),which was gradually tapered over two weeks. All pa-tients received low doses of azathioprine (100 mg) andcyclosporine (150 mg) during the episodes. The doseof azathioprine was increased to 2 mg/kg/d and cy-closporine to 5 mg/kg/d after the infusion and thengradually tapered to lower levels. Case 1 received onlycyclosporine because she was intolerant to azathio-prine. Table 1 summarizes the demographic data andclinical findings in our five patients.

HDIST was used in all five patients to treat activechoroiditis threatening the macula: four out of five

TABLE 1 Clinical data of patients with serpiginous choroiditis who received HDIST.

Case Age Gender IVA WVA FVA Time to remission (d) F/U (mos) No. Complications

1 OD 40 F 20/200 20/200 12 None1 OS 20/25 20/50 20/25 11 12 OD 48 F 20/20 20/20 12 None2 OS 20/25 20/40 20/30 7 1 CNV3 OD 47 F 20/25 20/60 20/20 7–15 38 4 None3 OS 20/200 20/2004 OD 27 F 20/20 20/100 20/40 8–11 25 3 CNV, gastric distress4 OS 20/200 20/200 None5 OD 63 M 20/20 20/60 20/20 7 48 2 None5 OS 20/200 20/400 20/200 7 1

HDIST, high-dose intravenous steroid therapy; OD, right eye; OS, left eye; F, female; M, male; IVA, initial visual acuity; WVA, worst visual acuity duringrecurrences; FVA, final visual acuity; F/U, follow-up after HDIST; d, days; mos, months; No, number of episodes treated with HDIST; CNV, subretinalneovascularization.

patients (Cases #1, 3, 4, and 5) had lost central visionin one eye due to macular involvement of the disease.Three patients (# Cases 2, 3, and 4) received HDISTon more than one occasion. All patients responded totreatment. In all cases, partial regression of choroidalinfiltrates occurred within 24 hours, accompanied byimprovement of the patient’s visual symptoms. Best-corrected visual acuities were achieved within a meantime of 10 days (range: 7–15 d). There were no ocularor severe systemic steroid side effects. One patient (#Case 4) required treatment with a histamine receptorantagonist to control gastric distress. Unfortunately inour case series, recurrences occurred in three patients.Mean time between recurrences was 10.1–1.1 months(range: 4–14 months). Two patients (# Cases 2 and 5)developed CNV during the follow-up period. The me-dian interval between the last recurrence and the mostrecent visit was eight months (range: 6–24 months).

SELECTED CASE REPORTIn March 2000, a 47-year-old woman (Case 3) ini-

tially presented in our service with visual loss in herleft eye. Examination disclosed vision 20/25 OD and20/400 OS. Results of fundus examination were consis-tent with serpiginous choroiditis, which was active OD(Fig. 1A). The patient received HDIST. When she wasseen one week later, the margins were inactive. The pa-tient had two more relapses which were treated success-fully with HDIST (Fig. 1B). Eight months after the lastrelapse and while she was on cyclosporine (2 mg/kg) andazathioprine (100 mg), the patient’s vision decreasedto 20/50 OD. Fundus examination disclosed active

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FIGURE 1 Color fundus photographs of the right eye of Case #3. (A) View of the fundus at presentation showing a large serpiginouslesion surrounding the optic disk and extending to the arcades. Arrowhead indicates active lesion seen at the edge of an old atrophicarea. (B) Same view as in (A), two years later. Inflammation was inactive. There was no progress of the lesion towards the fovea. Threeprevious recurrences at the macular edge of the lesion were treated with HDIST. However, progress of the superior border of the lesion isevident and can be attributed to silent episodes of inflammation not affecting the macula and not causing significant visual symptoms.(C) View of the fundus showing the last recurrence. Note the acute fluffy white lesion inferiorly (arrowhead), which represents therecurrence. (D) The first day after the start of intravenous methylprednisolone. (E) Fundus photograph five days after starting infusionshowing partial resolution of inflammation. Visual acuity is 20/25 and patient reports marked improvement of visual symptoms. (F) Fundusphotograph 10 days after starting infusion showing complete resolution of inflammation. VA is 20/20.

31 Intravenous Steroids in Serpiginous Choroiditis

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inflammation threatening the macula (Fig. 1C). She wasagain administered HDIST. Signs of regression of theinflammation were apparent from the first day after in-fusion (Fig. 1D). Partial resolution of the inflammationwas evident five days after starting infusion (Fig. 1E)and complete restoration of visual acuity occurred tendays after the infusion (Fig. 1F). At the last recordedexamination six months after the last relapse, the pa-tient’s vision was 20/20 OD and 20/200 OS. There wasno active inflammation in the eye.

DISCUSSIONThe course of serpiginous choroiditis is insidious

and, in the absence of foveal involvement, can beasymptomatic. If the fovea is involved, visual loss isprofound and often irreversible. Therefore, in cases offovea-threatening lesions, aggressive accelerated con-trol of the inflammation should be the goal of treat-ment. In all of our cases, control of the inflamma-tion was achieved quickly and no eye lost visionbecause of an advancing border of lesions. Nussenblatt(in Hooper & Kaplan3) reported that signs of re-sponse to HDIST may be anticipated within the first24–36 hours of therapy. Our findings support thisobservation.

Pulse-steroid therapy has been shown to be effectiveas an initial therapy in severe acute attacks of otheruveitis entities.4 In our experience, the response of ser-piginous choroiditis to oral steroids occurs after twoweeks of treatment. The reason why HDIST is moreeffective than oral steroids in producing the rapid re-duction of the appearance of active serpiginous lesionsis not clear-cut. Although the doses of immunosuppres-sives were increased after the attack, the relatively longtime that it takes for immunosuppressives to have aneffect cannot alone explain the rapid response. How-ever, as an optic neuritis treatment trial has indicated,5

it is possible that different doses and different routesof steroid administration produce quite differenteffects.

Unfortunately, our treatment regimen did not seemto prevent the occurrence of CNV or relapses ofchoroiditis. Current data suggest that immunosup-pressive therapy along with steroids should be ini-tiated in patients with vision-threatening serpiginouschoroiditis.6 Akpek et al.7 also recently suggested us-ing alkylating agents for long-term disease control. Thisstudy provides evidence that pulse-steroid therapy is

an effective way to promptly control exacerbations ofdisease.

Pulse-steroid therapy is widely used in ophthalmol-ogy and is generally considered safe. More than 30 yearsof experience with this treatment for various disordershave shown some minor and transient side effects anda small risk of serious life-threatening complications.Common side effects include sleep disturbance, mildmood change, stomach upset, and facial flushing.8 Seri-ous complications such as sudden death,9 infections,10

bone loss,11 psychological disturbances, hypertension,or elevated glucose levels can also occur.4 Thus, closemonitoring of the patients, especially those with coex-isting medical problems such as diabetes12 or rheumaticdiseases,13 is required. In these patients, administrationof intravitreal triamcinolone may be considered.14

In conclusion, this report presents our experienceof using HDIST to treat 12 episodes of choroiditis infive patients. Immediate response to treatment was evi-dent in all cases, followed by complete resolution of in-flammation and restoration of visual acuity within twoweeks. Thus, we recommend that pulse-steroid therapyalong with immunosuppressives be considered as theinitial treatment in severe cases of serpiginous choroidi-tis for prompt control of inflammation and a faster re-covery of visual function.

REFERENCES[1] Christmas NJ, Oh KT, Oh DM, Folk J. Long term follow-up of

patients with serpiginous choroiditis. Retina. 2002;22:550–556.[2] Akpek EK, Baltatzis S, Yang J, Foster CS. Long-term immunosup-

pressive treatment of serpiginous choroiditis Ocul Immunol Inflamm.2001;9:153–167.

[3] Hooper P, Kaplan H. Triple agent immunosuppression in ser-piginous choroiditis. Nussenblatt RB, discussion. Ophthalmology.1991;98:944–952.

[4] Wakefield D, McCluskey P, Penny R. Intravenous pulse methylpred-nisolone therapy in severe inflammatory eye disease. Arch Ophthal-mol. 1986;104:847–851.

[5] Beck RW, Cleary PA, Anderson MM, et al. A randomized controlledtrial of corticosteroids in the treatment of acute optic neuritis. TheOptic Neuritis Study Group. N Engl J Med. 1992;326:581–588.

[6] Akpek EK, Ilhan-Sarac O. New treatments for serpiginous choroiditis.Curr Opin Ophthalmol. 2003;14:128–131.

[7] Akpek EK, Jabs DA, Tessler HH, Joondeph BC, Foster S. Successfultreatment of serpiginous choroiditis with alkylating agents. Oph-thalmology. 2002;109:1506–1513.

[8] Chrousos GA, Kattah JC, Beck RW, Cleary PA. Side effects of glu-cocorticoid treatment. Experience of the Optic Neuritis TreatmentTrial. J Am Med Assoc. 1993;269:2110–2112.

[9] Erstad BL. Severe cardiovascular adverse effects in association withacute high-dose corticosteroid administration. DICP, The Annals ofPharmacotherapy. 1989;23:1019–1023.

[10] Badsha H, Edwards CJ. Intravenous pulses of methylpred-nisolone for systemic lupus erythematosus. Semin Arthritis Rheum.2003;32(6):370–377.

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[11] Haugeberg G, Griffiths B, Sokoll KB, Emery P. Bone loss in patientstreated with pulses of methylprednisolone is not negligible: a shortterm prospective observational study. Ann Rheum Dis. 2004;63(8):940–944.

[12] Feldman-Billard S, Lissak B, Kassaei R, Benrabah R, Heron E.Short-term tolerance of pulse methylprednisolone therapy in pa-tients with diabetes mellitus. Ophthalmology. 2005;112:511–515.

[13] Baethge BA, Lidsky MD, Goldberg JW. A study of adverse effectsof high-dose intravenous (pulse) methylprednisolone therapy in pa-tients with rheumatic disease. Ann Pharmacother. 1992;26(3):316–320.

[14] Navajas EV, Costa RA, Farah ME, Cardillo JA, Bonomo PP. Indocya-nine green-mediated photothrombosis combined with intravitrealtriamcinolone for the treatment of choroidal neovascularization inserpiginous choroiditis. Eye. 2003;17(5):563–566.

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