White Dot Syndrome

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Classification according to ICD-10
H35.0 Retinopathies of the fundus and changes in the retinal vessels
H30.9 Chorioretinitis, unspecified
ICD-10 online (WHO version 2019)

The white dot syndromes , German  idiopathic syndromes with white spots , are a group of inflammatory diseases of the retina . It is a heterogeneous group with involvement of the retina, the retinal pigment epithelium and the choroid (chorioretinopathy). Contrary to the term "white dot syndrome", white dots are not produced in all diseases; common lesions are in the fundus .

Not to be confused by the white spot syndrome virus -induced white spot syndrome , also English White spot syndrome called.

The exact definition and allocation has not yet been uniformly established.

Classification

The following are considered to belong to this group:

  • Acute posterior multifocal placoid pigment epitheliopathy (APMPPE, AMPPPE)
  • Acute retinal pigment epitheliitis (ARPE)
  • Acute Zonal Occult External Retinopathy ( AZOOR )
  • Birdshot chorioretinopathy
  • Multiple evanescent white dot syndrome ( MEWDS )
  • Multifocal choroiditis and panuveitis (MCP)
  • Punctiform internal chorioretinopathy (PIC)
  • Serpiginous chorioretinopathy

distribution

The frequency is given as 0.45 per 100,000 per year. It can be associated with other immune diseases .

About 1 - 5% of all uveitis patients in childhood have this syndrome.

Most of those affected are younger than 50 years of age at the onset of the disease.

root cause

The cause is not yet known. Infectious, viral, genetic and autoimmune triggers are discussed.

Clinical manifestations

Clinical criteria are often visual impairment with blurred vision and visual field defects .

Diagnosis

The diagnosis is made as part of an ophthalmological examination.

literature

  • JC Wang, KM McKay, AB Sood, I. Laíns, L. Sobrin, JB Miller: Comparison of choroidal neovascularization secondary to white dot syndromes and age-related macular degeneration by using optical coherence tomography angiography. In: Clinical ophthalmology. Volume 13, 2019, pp. 95-105, doi: 10.2147 / OPTH.S185468 , PMID 30643383 , PMC 6318713 (free full text).
  • ML Raven, ALringenisen, Y. Yonekawa, MS Stem, LJ Faia, JL Gottlieb: Multi-modal imaging and anatomic classification of the white dot syndromes. In: International journal of retina and vitreous. Volume 3, 2017, p. 12, doi: 10.1186 / s40942-017-0069-8 , PMID 28331634 , PMC 5357819 (free full text) (review).
  • RB Pearlman, PR Golchet, MG Feldmann, LA Yannuzzi, MJ Cooney, JE Thorne, JC Folk, EH Ryan, A. Agarwal, KC Barnes, KG Becker, LM Jampol: Increased prevalence of autoimmunity in patients with white spot syndromes and their family members. In: Archives of ophthalmology. Volume 127, number 7, July 2009, pp. 869-874, doi: 10.1001 / archophthalmol.2009.132 , PMID 19597107 , PMC 5674788 (free full text).

Individual evidence

  1. a b Emedicine
  2. a b G. Spital, A. Heiligenhaus, A. Scheider, D. Pauleikhoff, CP Herbort: "White dot syndrome" in childhood. In: Clinical monthly sheets for ophthalmology. Volume 224, Number 6, June 2007, pp. 500-506, doi: 10.1055 / s-2007-963179 , PMID 17594620 (review).
  3. Medscape
  4. JV Forrester: IOIS posterior segment inflammation intraocular. Guidelines, 1998, p. 184. IOIS
  5. chorioretinopathy type birdshot. In: Orphanet (Rare Disease Database).
  6. Serpiginous chorioditis. In: Orphanet (Rare Disease Database).
  7. NE Abu-Yaghi, SP Hartono, DO Hodge, JS Pulido, SJ Bakri: White dot syndromes: a 20-year study of incidence, clinical features, and outcomes. In: Ocular immunology and inflammation. Volume 19, number 6, December 2011, pp. 426-430, doi: 10.3109 / 09273948.2011.624287 , PMID 22106911 , PMC 3346947 (free full text).
  8. ^ A b D. A. Quillen, JB Davis, JL Gottlieb, BA Blodi, DG Callanan, TS Chang, RA Equi: The white dot syndromes. In: American journal of ophthalmology. Volume 137, Number 3, March 2004, pp. 538-550, doi: 10.1016 / j.ajo.2004.01.053 , PMID 15013878 (review).
  9. LM Jampol, KG Becker: White spot syndromes of the retina: a hypothesis based on the common genetic hypothesis of autoimmune / inflammatory disease. In: American journal of ophthalmology. Volume 135, Number 3, March 2003, pp. 376-379, doi: 10.1016 / s0002-9394 (02) 02088-3 , PMID 12614757 (review).
  10. ^ JE Knickelbein, HN Sen: Multimodal Imaging of the White Dot Syndromes and Related Diseases. In: Journal of clinical & experimental ophthalmology. Volume 7, number 3, June 2016, p., PMID 27482471 , PMC 4959777 (free full text).