White Dot Syndromes

A group of idiopathic multifocal posterior uveitis syndromes involving the CHOROID; RETINAL PIGMENT EPITHELIUM; and RETINA. They are characterized by multiple lesions of hypoautofluorescent dots in the FUNDUS OCULI and reduced VISUAL ACUITY. Several entities including BIRDSHOT CHORIORETINOPATHY are HLA-A ANTIGENS serotype A29 positive.

Also Known As:

Acute Macular Neuroretinopathies; Choroiditis, Serpiginous; Choroidopathy, Punctate Inner; Choroidopathy, Serpiginous; Inner Choroidopathy, Punctate; Macular Neuroretinopathy, Acute; Neuroretinopathy, Acute Macular; Punctate Inner Choroidopathies; Serpiginous Choroiditides; Serpiginous Choroidopathies; White Dot Syndrome; APMPPE; Acute Idiopathic Blind Spot Enlargement Syndrome; Acute Macular Neuroretinopathy; Acute Posterior Multifocal Placoid Pigment Epitheliopathy; Diffuse Subretinal Fibrosis Uveitis; MEWDS; Multiple Evanescent White Dot Syndrome; Punctate Inner Choroidopathy; Serpiginous Choroiditis; Serpiginous Choroidopathy

Networked: 468 relevant articles (29 outcomes, 27 trials/studies)

Relationship Network

Disease Context: Research Results

Eye Diseases: 3522
- Uveal Diseases: 16
  - Uveitis: 8470
    - Panuveitis: 512
      - Posterior Uveitis: 563
        White Dot Syndromes: 468
        Birdshot Chorioretinopathy: 178
        Multifocal Choroiditis: 109
        Acute zonal occult outer retinopathy: 59

Related Diseases

1.	Choroidal Neovascularization
2.	White Dot Syndromes
3.	Macular Edema
4.	Retinal Detachment (Retinal Detachments)
5.	Edema (Dropsy)

Experts

1.	Freund, K Bailey: 15 articles (05/2022 - 04/2006)
2.	Sarraf, David: 11 articles (05/2022 - 10/2013)
3.	Ishida, Susumu: 7 articles (06/2019 - 01/2012)
4.	Saito, Wataru: 7 articles (05/2016 - 07/2007)
5.	Jampol, Lee M: 6 articles (10/2021 - 07/2002)
6.	Mauget-Faÿsse, Martine: 6 articles (08/2019 - 09/2005)
7.	Bandello, Francesco: 6 articles (11/2018 - 01/2015)
8.	Mrejen, Sarah: 5 articles (01/2021 - 10/2013)
9.	Fawzi, Amani A: 5 articles (02/2019 - 10/2013)
10.	Hashimoto, Yuki: 5 articles (05/2016 - 01/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to White Dot Syndromes:

1.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2021 - "In patients with punctate inner choroidopathy, two distinct inflammatory signs observed with multimodal imaging display a differential response to systemic corticosteroids. " 09/01/2016 - "Active serpiginous choroiditis (SC) is a vision-threatening condition which requires intensive treatment using corticosteroids and/or immunosuppressives, especially if the lesions are involving or encroaching on the macula. " 01/01/2016 - "This is the first reported case of spontaneously resolving, unilateral VKH disease in the absence of high dose corticosteroid treatment with overlapping features of APMPPE. " 11/01/2013 - "We report a 15-year-old patient who had bilateral APMPPE, which was successfully treated with corticosteroids. " 03/01/2006 - "We suggest that systemic corticosteroids could be useful in selected cases of APMPPE with atypical clinical features."
2.	Ranibizumab (Lucentis)FDA Link 01/01/2018 - "This article highlights the efficacy of intravitreal Ranibizumab in the treatment of CNV associated with MEWDS. " 01/01/2021 - "The purpose was to demonstrate the diagnostic and therapeutic feasibility of swept source-optical coherence tomography angiography (SS-OCTA) by picturing neovascular changes secondary to a rare white dot syndrome following long-term intravitreal ranibizumab (IVR). " 01/01/2018 - "Intravitreal ranibizumab treatment represents a valuable therapeutic option for the management of CNV associated with MEWDS." 12/01/2017 - "The temporal relationship between ranibizumab injection and the onset of acute macular neuroretinopathy in this case report raises the possibility of a causal relationship." 12/01/2017 - "ACUTE MACULAR NEURORETINOPATHY AFTER RANIBIZUMAB INJECTION IN A DIABETIC PATIENT."
3.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 04/01/2015 - "To evaluate safety and efficacy of intravenous pulse cyclophosphamide (CyP) in acute macular serpiginous choroiditis (SC). " 10/05/2010 - "Long-Term Cyclophosphamide Treatment in a Case with Serpiginous Choroiditis." 10/05/2010 - "CONCLUSIONS: Long-term therapy with cyclophosphamide for serpiginous choroiditis is effective for improving vision and preventing recurrences." 04/01/2015 - "Pulse cyclophosphamide therapy in the management of patients with macular serpiginous choroidopathy." 10/05/2010 - "PROCEDURES: Oral cyclophosphamide therapy for 12 months in a case with unilateral active serpiginous choroiditis. "
4.	SteroidsIBA 04/01/2023 - "APMPPE is a self-resolving disease, unlike VKH, and early diagnosis can avoid unnecessary administration of steroids and related side effects." 11/01/2021 - "Although most patients with acute posterior multifocal placoid pigment epitheliopathy have a relatively short course and recover vision quickly, the use of steroids and immunosuppression may be of benefit for those patients with severe and prolonged visual loss." 05/17/2020 - "Thus, it seems that short-term oral steroids might be an alternative method of management for patients with MEWDS." 01/01/2017 - "Spectral-domain optical coherence tomography dynamic changes and steroid response in multiple evanescent white dot syndrome." 10/01/2016 - "We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. "
5.	Triamcinolone Acetonide (Azmacort)FDA LinkGeneric 09/01/2016 - "Efficacy of reduced dose of intravitreal triamcinolone acetonide in a case of active serpiginous choroiditis." 12/01/2006 - "To report the efficacy of intravitreal triamcinolone acetonide injection for acute treatment of a patient with serpiginous choroiditis. " 05/01/2019 - "Intravitreal and posterior subtenon triamcinolone acetonide for severe acute posterior multifocal placoid pigment epitheliopathy." 01/01/2010 - "To evaluate intravitreal triamcinolone acetonide (IVTA) in unilateral serpiginous choroiditis with macular involvement with special reference to effectiveness of treatment and its side effects. " 01/01/2010 - "Prospective evaluation of intravitreal triamcinolone acetonide in serpiginous choroiditis."
6.	Immunosuppressive Agents (Immunosuppressants)IBA 04/01/2006 - "Although therapy with immunosuppressive agents is currently accepted as the best option for treating active serpiginous choroiditis (SC), there is no consensus on the most effective immunosuppressive drug to use. " 05/01/2001 - "Our present observation corroborates the beneficial effect of a triple agent immunosuppressive therapy on the course of serpiginous choroiditis. " 05/01/2001 - "In the literature, until now only six patients with serpiginous choroiditis treated with a triple agent immunosuppressive therapy have been reported. " 02/08/2022 - "IGRA-positive serpiginous choroiditis (serpiginous-like choroiditis) could be halted by combined multiple tuberculostatic and multiple immunosuppressive agents, as seen in our study where 3/4 early treated patients had conserved central function and one late treated patient had recovered a substantial amount of visual field. " 02/08/2022 - "Tuberculosis-related serpiginous choroiditis: aggressive therapy with dual concomitant combination of multiple anti-tubercular and multiple immunosuppressive agents is needed to halt the progression of the disease."
7.	Prednisone (Sone)FDA LinkGeneric 03/01/2021 - "She was diagnosed with punctate inner choroidopathy and then treated with an increased dose of daily prednisone, which resulted in progressive improvement of her visual acuity and anatomical status." 02/01/1998 - "To demonstrate the effect of oral prednisone in the treatment of atypical isolated central lesions in two patients with serpiginous choroiditis. " 02/01/1998 - "Atypical central lesions in serpiginous choroiditis treated with oral prednisone." 07/01/2002 - "Resolution of punctate inner choroidopathy lesions with oral prednisone therapy." 02/01/1998 - "In two patients with visual loss due to an atypical central lesion in serpiginous choroiditis, regression of the lesion without the development of a typical chorioretinal scar and subsequent improvement of visual acuity was achieved with the administration of oral prednisone."
8.	Dexamethasone (Maxidex)FDA LinkGeneric 03/01/2021 - "Intravitreal dexamethasone implants can be used to stabilize acute posterior multifocal placoid pigment epitheliopathy lesions and help resolve the condition. " 01/01/2018 - "The use of dexamethasone 0.7 mg in punctate inner choroidopathy yielded encouraging results and long periods of stability. " 01/01/2017 - "To assess the efficacy and safety of dexamethasone (DEX) intravitreal implant in patients with active serpiginous choroiditis (SC) already receiving maximal tolerated systemic immunosuppressive therapy. " 01/01/2017 - "Dexamethasone intravitreal implant in serpiginous choroiditis." 01/01/2018 - "To compare the efficacy of pulse cyclophosphamide with pulse dexamethasone in acute macular serpiginous choroiditis (SC). "
9.	Cyclosporine (Ciclosporin)FDA LinkGeneric 09/01/2000 - "Early treatment with cyclosporin in serpiginous choroidopathy maintains remission and good visual outcome." 09/01/2000 - "Cyclosporin is a safe and effective option with which to manage serpiginous choroidopathy. " 01/01/2001 - "The etiology of MEWDS is still unknown but the absence of new episodes during cyclosporine treatment and the recurrence immediately after decreasing or discontinuing the drug suggests an autoimmune origin, with the involvement of cellular immunity in the pathogenic process." 01/01/2001 - "Treatment of multiple evanescent white dot syndrome with cyclosporine." 10/01/1990 - "Seven patients affected by bilateral inflammatory serpiginous choroiditis have been treated with Cyclosporine-A for 6-21 months. "
10.	Chlorambucil (Leukeran)FDA Link 01/01/2018 - "Chlorambucil in a relatively short duration of time, with an escalating dose guided by weekly WBC was well tolerated, as well as effective in preventing recurrence and maintaining vision in patients with serpiginous choroiditis." 01/01/2018 - "To evaluate the efficacy of chlorambucil in the treatment of serpiginous choroiditis. " 01/01/2018 - "The mean age of the 17 patients with the diagnosis of serpiginous choroiditis treated with chlorambucil was 46 years, and six patients (35%) were male. " 01/01/2018 - "In total, 17 patients with the diagnosis of serpiginous choroiditis treated with chlorambucil were identified. " 01/01/2018 - "Treatment of Serpiginous Choroiditis with Chlorambucil: A Report of 17 Patients."

Therapies and Procedures

1.	Therapeutics 12/01/2022 - "One month following her initial diagnosis, the patient's symptoms had resolved without any therapy, and a fundus examination revealed multiple relatively ill-defined brown-colored subretinal lesions in the nasal midperiphery, corresponding to the location of the previous MEWDS lesions. " 01/01/2022 - "To describe an alternative treatment for a patient with serpiginous choroiditis (SC) who was not tolerant to systemic therapies. " 01/01/2013 - "In this case, the rapid loss of vision was specific to the onset pattern of APMPPE, and the slow response to therapy was very different from the response typically observed in VKH disease. " 12/01/2001 - "We outline neurological complications in APMPPE and discuss the reported therapies. " 06/01/2021 - "Unlike uncomplicated APMPPE cases, in patients with neurological manifestations systemic therapy is advocated."
2.	Photochemotherapy (Photodynamic Therapy) 07/01/2003 - "Photodynamic therapy: a treatment option in choroidal neovascularisation secondary to punctate inner choroidopathy." 01/01/2007 - "Photodynamic therapy for subfoveal and juxtafoveal choroidal neovascularization associated with punctate inner choroidopathy." 09/01/2005 - "Photodynamic therapy for subfoveal classic choroidal neovascularization related to punctate inner choroidopathy (PIC) or presumed ocular histoplasmosis-like syndrome (POHS-like)." 02/01/2012 - "[Punctate inner choroidopathy - Improvement in vision after anti-VEGF and photodynamic therapy. " 04/01/2008 - "Photodynamic therapy combined with systemic corticosteroids for choroidal neovascularisation secondary to punctate inner choroidopathy."
3.	Lasers (Laser) 09/01/2015 - "The aim of this study is to quantitatively evaluate changes in choroidal circulation hemodynamics using laser speckle flowgraphy (LSFG) in patients with MEWDS. " 01/01/2018 - "Adaptive optics scanning laser ophthalmoscopy combined with multimodal imaging has the potential to enhance our understanding of photoreceptor involvement in APMPPE." 05/01/2016 - "In all APMPPE eyes, laser speckle flowgraphy was conducted to evaluate the mean blur rate (MBR), a quantitative index of relative blood flow velocity. " 12/01/2014 - "Enhanced-depth imaging optical coherence tomography and laser speckle flowgraphy in a patient with acute macular neuroretinopathy." 01/01/2014 - "The authors report adaptive optics scanning laser ophthalmoscopy (AO-SLO) findings in a case of acute posterior multifocal placoid pigment epitheliopathy. "
4.	Intravitreal Injections 06/12/2017 - "Intravitreal injection of conbercept significantly improved visual and anatomical outcomes in choroidal neovascularization secondary to punctate inner choroidopathy over a 6-month follow-up period. " 11/01/2007 - "To report the effect of a single intravitreal injection of ranibizumab in a patient with choroidal neovascularization (CNV) secondary to multiple evanescent white dot syndrome (MEWDS). " 01/01/2018 - "This is a retrospective case series of three female patients with punctate inner choroidopathy who were treated with intravitreal injections anti-vascular endothelial growth factor agent (bevacizumab, 1.25 mg/0.05 mL). " 01/01/2018 - "We report the outcomes after an essentially long follow-up period of up to 14 years and provide evidence of the effectiveness of intravitreal injections of bevacizumab and dexamethasone 0.7 mg in punctate inner choroidopathy patients with choroidal neovascular membrane formation. "
5.	Light Coagulation 04/01/2008 - "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. " 10/01/1996 - "In a 38-year-old woman with bilateral punctate inner choroidopathy, thalidomide therapy was initiated three days after laser photocoagulation of a choroidal neovascular membrane. " 01/01/2011 - "B-scan layer-by-layer analysis and unique SD-OCT reflectivity patterns of the following retinal white lesions are reviewed in the order of their retinal layer localization: myelinated nerve fiber layer, cotton wool spot, exudates, edema residues, drusen, fundus albipunctatus, Stargardt disease, Bietti crystalline dystrophy, punctate inner choroidopathy (PIC), presumed ocular histoplasmosis syndrome (POHS), post-photocoagulation chorioretinal scarring, and osseous choristoma. "