Macular serpiginous choroiditis

The authors describe in seven eyes of four patients a form of serpiginous choroiditis beginning in the macula without initial peripapillary activity. Eyes with macular serpiginous choroiditis often had a poor visual prognosis and sometimes developed subretinal neovascularization. Fluorescein angiography of the acute lesions showed hypofluorescence; some cases were thus initially diagnosed as having choroidal ischemia. Fluorescein angiography in one eye, however, suggested that at least some of the hypofluorescence seen acutely in eyes with serpiginous choroiditis is secondary to blocked fluorescence from "opaque" retinal pigment epithelium.     

Immunological studies on serpiginous choroiditis

Immunological studies on 15 patients with serpiginous choroiditis gave no definite indications of the aetiology of the choroidal vascular lesion which appears to be the initial failure in this disorder. On the basis of the patients' history, recurrencies, and clinical and fluorescein angiographic features, it was suspected that the disorder was of inflammatory origin, probably vasculitis due to an abnormal immune response. To some extent this was supported by the laboratory findings. Of the histocompatibility antigens, HLA-B7 was found more frequently than expected in a Finnish population (54.5% versus 24.3%;P < 0.05). Increased levels of antibacterial antibodies, ASO or ASTA, were found in eight patients and antiviral (herpes simplex) antibodies in two. One patient had earlier been treated due to positive syphilis serology, and one had an increased serum level of IgM as well as positive latex reaction in a dilution of 1 : 16. The serum concentration of complement component C3 was slightly decreased in three of the six patients studied and at the lower limit of the normal range in one. No manifestations suggesting systemic disease were found.     

New treatments for serpiginous choroiditis

Serpiginous choroiditis is a bilateral, inflammatory condition of the inner choroid and overlying retinal pigment epithelium with a distinctive retinal distribution. Although rare, serpiginous choroiditis deserves attention because it can lead to permanent loss of vision due to foveal involvement by inflammation or choroidal neovascularization from frequent recurrences. This article reviews the recent ophthalmic literature published on the management of serpiginous choroiditis.     

Therapeutic dilemma in serpiginous choroiditis

A 43-year-old woman had blurred vision in the left eye for 4 years. Her best-corrected visual acuity was 20/20 in the right eye and 20/200 in the left eye. The fundus showed grayish-yellow, jigsaw-puzzle-shaped lesions at the level of the retinal pigment epithelium and choriocapillaries emanating from the optic nerve head in both eyes. Fluorescein angiography showed late leakage in active lesions and hypofluorescence in hyperpigmented areas. Oral prednisolone and cyclosporine were given first. However, after posterior subtenon triamcinolone injections in both eyes and one intravitreal triamcinolone injection in the left eye, macular edema worsened. Steroid-induced central serous chorioretinopathy was suspected, so we tapered prednisolone rapidly and changed to azathioprine. Subsequent optical coherence tomography demonstrated retinal pigment epithelial detachment in the right eye subsided gradually. Fundus autofluorescence imaging showed progressively quiescent lesions. Unfortunately, acute myocardial infarction, atrial fibrillation, and ischemic stroke developed after 6 weeks azathioprine. In the acute phase of serpiginous choroiditis, corticosteroids are most commonly used. However, steroid therapy may be complicated with steroid-induced central serous chorioretinopathy. When we shift to other systemic immunosuppressive regimens, such as azathioprine, the possibility of acute myocardial infarction should be kept in mind.     

Triple agent immunosuppression in serpiginous choroiditis

Serpiginous choroidopathy is a progressive choroidal inflammatory disorder that typically has a variable saltatory course. Response to steroids is uncertain. By using azathioprine, cyclosporine, and prednisone in combination, the authors have observed rapid remission of active disease in five patients. Remissions have been maintained for periods up to 18 months. Because of the synergistic effects of this combination, doses could rapidly be reduced to maintenance levels without reactivation. Disease in two patients recurred immediately after discontinuation of low-dose therapy but was arrested when therapy resumed. Triple agent immunosuppressive therapy is well tolerated and appears to be effective.     

Fundus autofluorescence in serpiginous choroiditis

Background  Fundus autofluorescence is already used to evaluate inflammatory disorders affecting the chorioretinal interface. We investigated the autofluorescence characteristics of two cases of serpiginous choroiditis (SC) during recurrent acute episodes, and followed them until their resolution. We compared the autofluorescence findings with those obtained with other imaging techniques. Methods  Autofluorescence photographs of the eyes were taken in a 26-year-old female and a 68-year-old male with SC at the first appearance of active lesions and during a strict follow-up period. Patients had complete ophthalmological evaluations including optical coherence tomography (OCT) and fluorescein and indocyanine green (ICG) angiography. Autofluorescence findings were compared with features from other imaging techniques. Patients were treated with systemic or intravitreal steroids. Results  Hyperautofluorescence was detected 2 to 5 days after the appearance of the lesions, providing a clear delineation of the area of definitive retinal pigment epithelium (RPE) damage. This area was less extensive than the perfusion defect of the choriocapillaris indicated by ICG angiography. OCT showed very early increased reflectance of the photoreceptor layer in the area of hyperautofluorescence. A progressive decrease in autofluorescence was seen during the scarring phase of the disease. OCT changes in the photoreceptor layer were still present in the atrophic hypoautofluorescent lesions. Conclusion  Fundus autofluorescence seems to be a very sensitive imaging technique for detecting damage of the RPE in acute episodes of SC. A sequence of autofluorescence changes reflects the passage from activation to resolution of new lesions. Similarities, but also differences can be found by comparing our SC findings with those obtained with autofluorescence and OCT in posterior multifocal placoid pigment epitheliopathy. Supported by the Fondazione per la Macula Onlus, Genova, Italy. The authors have no proprietary interest in any aspect of this report. The authors have full control of all primary data, and they agree to allow Graefe’s Archive for Clinical anf experimental Ophthalmology to review their data upon request.     

Posterior scleritis mimicking macular serpiginous choroiditis

An unusual case of posterior scleritis mimicking macular serpiginous choroiditis is reported.     

Long-term immunosuppressive treatment of serpiginous choroiditis

Objective: To determine the efficacy of immunosuppressive treatment in serpiginous choroiditis. Design: The clinical courses were reviewed of six consecutive patients (12 eyes) with vision-threatening, steroid-dependent/resistant serpiginous choroiditis treated with a combination of immunosuppressive agents including azathioprine, cyclosporine, and cyclophosphamide. All patients underwent treatment for at least 12 months. Results: The follow-up period ranged from 17 to 105 months (mean 57, median 43). All patients were able to taper oral steroids. Five patients discontinued all immunosuppressive medications after a treatment period of 12 to 69 months (mean 39 months). Immunosuppressive treatment was continued in one patient at a ‘low’ maintenance dose. Ten eyes had improved visual acuities, while vision remained impaired in two due to macular scars. Recurrence was noted in two patients when an attempt was made to decrease the dose of immunosuppressive medication. Two patients experienced side effects which were reversed by decreasing the dose of the medications. Conclusion: Long-term immunosuppressive treatment appears to prolong remission and preserve vision in patients with serpiginous choroiditis.     

匍行性脉络膜炎的临床特征与治疗

目的 探讨匍行性脉络膜炎的临床表现、荧光素眼底血管造影(FFA)特征和治疗结果.方法 自1999年11月至2006年7月,连续收集行FFA检查的匍行性脉络膜炎患者14例26只眼,观察其临床表现、FFA特征和治疗效果.结果 14例患者中,男性6例,女性8例,平均年龄45.6岁.双眼12例,单眼2例.根据眼底病变部位及其临床特征分为典型性视乳头周围地图状脉络膜炎11例和黄斑部匍行性脉络膜炎3例.FFA早期表现为病灶区由于脉络膜毛细血管萎缩而呈低荧光,病灶边缘呈高荧光,非活动病变晚期出现纤维瘢痕和巩膜染色.经糖皮质激素治疗后,8例14只眼视力提高,3例6只眼视力稳定,3例6只眼视力恶化.结论 匍行性脉络膜炎是一种少见的双眼慢性进行性眼内炎症,主要累及视网膜色素上皮和脉络膜毛细血管层,早期发现和及时治疗可防止发生永久性视力损害.     

Cyclosporine-A in the treatment of serpiginous choroiditis

Summary Seven patients affected by bilateral inflammatory serpiginous choroiditis have been treated with Cyclosporine-A for 6-21 months. Nine outof the fourteen eyes showed a significant improvement in their visual acuity; five eyes did not change.Cyclosporine-A may, therefore, be considered effective in the treatment of this disease.Its usefulness seems to be greater when the serpiginous choroiditis is in its acute stage; chronic stages, however, also seem to improve under treatment.Its main indication is, in our opinion, the involvement of the macular region of the second eye, when the first eye is already damaged. We consider Cyclosporine-A, in these situations, to be a first choice treatment.     

Autoimmune hepatitis in a patient with serpiginous choroiditis

CASE REPORT: We report the case of a patient with serpiginous choroiditis who developed an autoimmune hepatitis. DISCUSSION: The etiology of serpiginous choroiditis remains uncertain. An immune-mediated mechanism has been reported. This case shows an association between serpiginous choroiditis and autoimmune processes.     

Intravitreal triamcinolone acetonide treatment for serpiginous choroiditis

PURPOSE: To report the efficacy of intravitreal triamcinolone acetonide injection for acute treatment of a patient with serpiginous choroiditis. METHODS: A 50-year-old male patient with serpiginous choroiditis presenting with the complaint of decreased visual acuity in his right eye for the last 10 days. The best corrected visual acuity (BCVA) of the patient was counting finger from 1 meter. Fundus examination and fundus fluorescein angiography of right eye revealed active macular choroiditis in right eye. Intravitreal triamcinolone acetonide (4 mg/0.1 ml) was injected into vitreous, and the patient was followed with visual acuity testing, intraocular pressure measurement, and fundus examination, including fundus fluorescein angiography. RESULTS: Visual acuity of the patient improved to 20/100 after 2 weeks in spite of the triamcinolone crystals, and to 20/50 after 4 weeks with a single dose intravitreal triamcinolone acetonide injection. Complete resolution of the active lesion has been maintained during the 6 months of follow-up. CONCLUSIONS: Single dose intravitreal triamcinolone acetonide injection is sufficient for controlling the active lesions in serpiginous choroiditis. It needs further evaluation as an alternative treatment for achieving rapid and significant visual acuity recovery.     

匍行性脉络膜炎诊治新进展

匍行性脉络膜炎是一种罕见的双眼慢性进行性脉络膜炎症,易反复发作,主要侵犯脉络膜毛细血管层和视网膜色素上皮.常见于中青年人,男性略多于女性.根据眼底表现可分为视盘周围型匍行性脉络膜炎、黄斑型匍行性脉络膜炎、广泛型匍行性脉络膜炎.视力的预后主要取决于病灶是否累及黄斑中心凹、旁中心凹以及是否继发脉络膜新生血管膜.病因及发病机制尚不明确,可能与自身免疫、感染因素、血管病变有关.眼底自发荧光、荧光素眼底血管造影及吲哚青绿血管造影对病灶的位置、大小及活动性的评估具有重要意义.由于本病少见、病程隐匿进展,目前仅从小样本中观察到使用免疫抑制剂和烷化剂能较好地控制病情,稳定视力,但治疗方法的有效评估需要大样本长期随访,因此尚需要多中心随机临床试验证实,才能确定最佳的治疗策略.