Posterior scleritis mimicking macular serpiginous choroiditis

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

Clinical profile,multimodal imaging,and treatment response in macular serpiginous choroiditis

Purpose:To describe the clinical profile, multimodal imaging, and treatment response in macular serpiginous choroiditis (MSC).Methods:Clinical records of 16 eyes (14 patients) with MSC presenting to a tertiary eye care institute between 2015 and 2019 were analyzed retrospectively.Results:Mean age of 14 patients presenting with MSC was 33 ± 13 yrs with 64% males and 36% females. Mean visual acuity of the eyes with MSC at presentation was 0.43 ± 0.46 (logMAR) improving to 0.16 ± 0.28 (logMAR) at final visit. Thirteen eyes (81.3%) had active lesion at presentation. Mantoux test was positive in seven patients (50%) and QuantiFERON TB gold test positive in 10 patients (71%). HRCT chest showed latent tuberculosis in seven patients (50%). All patients underwent multimodal imaging. All patients received oral steroids as treatment therapy; 11 patients also received immunosuppressives, nine patients received additional anti-tubercular therapy (ATT). Mean duration of follow-up for the patients was 18 ± 10 months. A total of eight (50%) eyes had recurrence of lesions after an average duration of 14 ± 14 (3-36) months and were restarted on the treatment as per the requirement. At final follow-up, all eyes showed a good response to treatment and had healed lesions. Comparing the final BCVA to the initial BCVA, 38% (n = 6) showed improvement, 56% (n = 9) remained stable, and 6% (n = 1) eyes worsened at the final follow-up.Conclusion:Clinical profile and presentation of MSC is similar to that of CSC, and combination treatment with intravenous methyl prednisolone (IVMP), steroids, immunosuppressives, and ATT can salvage vision. A high suspicion of associated tuberculosis in endemic regions should be kept in mind.     

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.     

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.     

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.     

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.     

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.     

Multifocal choroiditis associated with serpiginous choroiditis and choroidal neovascularization: a case report

BACKGROUND: We treated a patient with multifocal choroiditis (MFC) associated with serpiginous choroiditis and choroidal neovascularization (CNV). CASE: In a 27-year-old woman whose left eye showed serpiginous choroiditis near the optic disc and multiple irregular chorioretinal scars and exudates in peripheral and near-peripheral zones, fluorescein angiography demonstrated diffusely scattered hyperfluorescent dots and late leakage from retinal vessels. The lesion near the optic disc showed hypofluorescence in the early venous phase, and hyperfluorescence in the late phase. Sub-Tenon injection of triamcinolone acetonide did not effectively treat the CNV secondary to serpiginous choroiditis, but direct laser photocoagulation was successful, achieving improved visual acuity. CONCLUSIONS: We suggest that this patient's MFC and serpiginous choroiditis represented a single process rather than independent events. With lesions at the posterior pole, CNV developed secondarily. In this case, direct laser photocoagulation proved useful.     

Long-term follow-up and angiographic findings in serpiginous choroiditis

BACKGROUND: Long-term follow-up and angiographic findings of serpiginous choroiditis, a chronic disease with recurrences of unknown origin, are reported. PATIENTS AND METHODS: A retrospective analysis of the data of 4 patients with serpiginous choroidopathy, who were treated in the eye clinic of Mainz in the past 10-18 years was made. Progression of disease was documented by photography, as well as fluorescein and indocyanine green angiography. Patients with acute lesions were treated with steroids per os for some weeks. RESULTS: All 8 eyes developed 2-7 recurrences with progression of the chorioretinal scars. In cases of macular involvement the functional results were worse. No choroidal neovascularizations were seen. During follow-up 3/8 eyes became blind, 4/8 eyes retained reading vision. Indocyanine green angiography usually demonstrated many more lesions than those seen with fluorescein. In 3 cases acute choroidal lesions were detected only with indocyanine green angiography whereas no sign of inflammation was demonstrated by fluorescein angiography. CONCLUSION: The functional prognosis of serpiginous choroiditis is bad because all eyes developed at least 2 recurrences during follow-up. Use of indocyanine green angiography may help to better describe the full extent of the disease, even before it is seen with fluorescein angiography. It can help the observer to determine the precise clinical progression of the disease.     

Subretinal and disc neovascularisation in serpiginous choroiditis.

Three out of 15 patients with serpiginous choroiditis who have been followed up for 1 to 10 years (mean 4.9 years) developed subretinal neovascularisation in the macula. In one eye new vessels were treated with argon laser without attaining permanent obliteration, in the second eye the neovascular membrane was regarded as untreatable because it was under the fovea, and in the third eye new vessels became obliterated spontaneously after atrophy of the surrounding choriocapillaris and the pigment epithelium of the retina. In a furth patient disc new vessels were seen at the active stage of serpiginous choroiditis; these new vessels disappeared after scarring of the initial chorioretinal lesions.