Triple Agent Immunosuppression in Serpiginous Choroiditis

doi:10.1016/S0161-6420(91)32198-5

Ophthalmology

Volume 98, Issue 6, June 1991, Pages 944-952

https://doi.org/10.1016/S0161-6420(91)32198-5 Get rights and content

Abstract

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.

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Cited by (111)

Acute posterior multifocal placoid pigment epitheliopathy, serpiginous choroiditis and related diseases
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L’épithéliopathie en plaque et la choroïdite serpigineuse sont deux maladies classées dans les « syndromes des taches blanches ». Toutes deux sont des maladies inflammatoires/auto-immunes avec une atteinte primaire suspectée au niveau de la choriocapillaire. La première a habituellement un excellent pronostic, alors que la deuxième peut rapidement induire une cécité légale. Le choix de les décrire dans une seule revue est dû au fait qu’entre ces deux entités cliniquement bien définies existent des maladies frontières, plus récemment décrites, telles que la maculopathie placoïde persistante ou la choroïdite ampigineuse, qui partagent des caractéristiques de ces deux pathologies. Cette revue a pour but de décrire les éléments démographiques qui distinguent ces quatre pathologies et d’étayer les éléments d’imagerie multimodale permettant de les différentier.
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) and serpiginous choroiditis are two diseases classified as “white spot syndromes.” Both are inflammatory/autoimmune diseases with suspected primary involvement of the choriocapillaris. The former usually has an excellent prognosis, while the latter can rapidly induce legal blindness. Whereas these diseases are well defined and well known, other entities (such as persistent placoid maculopathy or ampiginous choroiditis) with features of both APMPPE and serpiginous choroiditis have been described more recently. This review aims to describe demographic characteristics and multimodal imaging features to help differentiate between these four diseases.
Classification Criteria for Serpiginous Choroiditis
2021, American Journal of Ophthalmology
To determine classification criteria for serpiginous choroiditis.
Machine learning of cases with serpiginous choroiditis and 8 other posterior uveitides.
Cases of posterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the infectious posterior uveitides / panuveitides. The resulting criteria were evaluated on the validation set.
One thousand sixty-eight cases of posterior uveitides, including 122 cases of serpiginous choroiditis, were evaluated by machine learning. Key criteria for serpiginous choroiditis included (1) choroiditis with an ameboid or serpentine shape; (2) characteristic imaging on fluorescein angiography or fundus autofluorescence; (3) absent to mild anterior chamber and vitreous inflammation; and (4) the exclusion of tuberculosis. Overall accuracy for posterior uveitides was 93.9% in the training set and 98.0% (95% confidence interval 94.3, 99.3) in the validation set. The misclassification rates for serpiginous choroiditis were 0% in both the training set and the validation set.
The criteria for serpiginous choroiditis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.
Chlorambucil combination therapy in refractory serpiginous choroiditis: A cure?
2021, American Journal of Ophthalmology Case Reports
To find a remedy for serpiginous choroiditis refractory to oral prednisone and chlorambucil treatment.
Eight eyes of four patients (all female) with advanced macular involvement secondary to serpiginous choroiditis were included in the study. The average age of the patients was 45.2 years. One eye of each patient was legally blind and the lesion was close to the fovea in the other eye. All four patients failed oral prednisone and chlorambucil therapy. However, case 1 responded to chlorambucil treatment after intravitreal dexamethasone implant implantation and discontinuation of oral prednisone. Case 2 responded to chlorambucil therapy when oral prednisone was stopped in combination with infliximab therapy. Due to long follow-up period of more than four years, these two cases are considered to be cured. Case 3 and case 4 were not able to achieve remission with chlorambucil and immunomodulatory therapy. They refused intravitreal steroid implant due to side effects profile.
The stability of WBC counts within toxic levels close to normal or lower limits of normal (3000–4500 cells/μl) during treatment with chlorambucil is an essential factor for the success of this therapy. A combination of dexamethasone intravitreal implant with chlorambucil therapy can be an effective and promising regimen in inducing and maintaining remission in refractory serpiginous choroiditis patients who fail a combination of systemic corticosteroid and chlorambucil therapy.
Whitcup and Nussenblatt’s Uveitis: Fundamentals and Clinical Practice
2021, Whitcup and Nussenblatt's Uveitis: Fundamentals and Clinical Practice
Swept-Source OCT Angiography of Serpiginous Choroiditis
2018, Ophthalmology Retina
To examine and quantify choriocapillaris lesions in active and quiescent serpiginous choroiditis (SC) using swept-source (SS) OCT angiography (OCTA) and en face image analysis.
Prospective, observational case series.
Patients with a clinical diagnosis of SC.
A SS-OCTA prototype was used to image active and quiescent serpiginous lesions longitudinally before and after anti-inflammatory treatment. En face slabs of choriocapillaris flow or outer nuclear layer structure were generated from OCTA and OCT data, respectively.
Qualitative and quantitative analyses on lesion boundary and area using a semiautomated MATLAB algorithm. Lesions also were compared with traditional multimodal imaging.
Six eyes of 3 patients were imaged. Choroidal lesions were identified and analyzed in 4 of 6 eyes. Lesions with well-defined boundaries were identified in the choriocapillaris slab in areas of both active and inactive choroiditis. The choriocapillaris slab lesion size and shape showed good correlation with lesions identified on indocyanine green angiography. The choriocapillaris slab lesion area increased with disease activity and decreased with corticosteroid treatment. During active disease, the choriocapillaris slab lesion area was larger than both the outer nuclear layer (ONL) slab and fundus autofluorescence lesion areas. Active choriocapillaris slab lesions not associated with corresponding abnormal autofluorescence resolved without clinical scarring after treatment. In inactive scars, the areas of retinal and choriocapillaris lesions were similar and did not change over time.
En face analysis of SS-OCTA choriocapillaris flow voids provide a noninvasive method for the detection of lesions in patients with SC. The presence of lesions in the choriocapillaris in the absence of retinal pigment epithelium and outer retinal abnormalities supports the hypothesis that choriocapillaris is the primary site of pathologic features in SC and may be a sensitive early sign of disease activity. We propose a simple grading system of SC lesions based on SS-OCTA and fundus autofluorescence findings. Swept-source OCTA is a promising noninvasive method for monitoring patients with SC.
Detection of mycobacterium tuberculosis genome in vitreous fluid of eyes with multifocal serpiginoid choroiditis
2015, Ophthalmology
To compare 3 different molecular techniques to detect the Mycobacterium tuberculosis genome in vitreous fluid of eyes with multifocal serpiginoid choroiditis (MSC).
Prospective, interventional case series.
Eleven patients (11 eyes) with active MSC in at least 1 eye underwent diagnostic pars plana vitrectomy (PPV) between October 2012 and December 2013.
Vitreous fluid samples were subjected to multitargeted polymerase chain reaction (PCR) for a M. tuberculosis assay, the Gene Xpert MTB/RIF assay (Cepheid, Sunnyvale, CA), and a line probe assay (GenoType MTBDRplus; Hain Lifescience, GmbH, Nehren, Germany). The samples with positive results were subjected to rpoB gene sequencing to demonstrate rifampicin resistance. The clinical details, digital fundus imaging, and treatment details and outcomes also were noted.
Detection of the M. tuberculosis genome and rifampicin resistance in the vitreous samples.
Of the 11 eyes subjected to PPV, the multitargeted PCR results for tuberculosis were positive for 10 eyes, the MTBDRplus assay results were positive in 6 eyes, and the Gene Xpert MTB/RIF assay results were positive in 4 eyes. Rifampicin resistance was detected in 3 eyes by rpoB gene sequencing, in 3 eyes by the MTBDRplus assay, and in 1 eye by the Gene Xpert MTB/RIF assay.
We detected the M. tuberculosis genome in the vitreous fluid of eyes with MSC using 3 different molecular techniques. Rifampicin resistance was detected for the first time in eyes with MSC.

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: Presented at the American Academy of Ophthalmology Annual Meeting, Atlanta, Oct/Nov 1990. Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc, New York, New York (Department of Ophthalmology and Visual Sciences, Washington University), and by a fellowship training grant from the Ontario Ministry of Health (Dr. Hooper).

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Triple Agent Immunosuppression in Serpiginous Choroiditis

Abstract

Am J Ophthalmol

Am J Ophthalmol

Am J Kidney Dis

Am J Ophthalmol

Am J Ophthalmol

Uveitis: Fundamentals and Clinical Practice

Immunosuppressive therapy for severe chronic uveitis

Arch Ophthalmol

Geographical choroidopathy

Br J Ophthalmol

Geographic helicoid peripapillary choroidopathy: clinical presentation and fluorescein angiographic findings

Trans Am Acad Ophthalmol Otolaryngol

Serpiginous choroiditis

Br J Ophthalmol

Failure of cyclosporine A in serpiginous choroiditis

J Ocul Ther Surg

Combined immunosuppressive therapy with cyclosporin A and azathioprine

Transplantation

Reduction of nephrotoxicity and improvement of immunosuppression by combination of cyclosporin A and azathioprine

Transplant Proc

The use of low doses of cyclosporin, azathioprine, and prednisone in renal transplantation

Transplant Proc