{"orpha":35686,"name":"Serpiginous choroiditis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Geographic helicoid peripapillary choroidopathy"],"definition":"A rare non-infectious posterior uveitis characterized by usually bilateral, chronic, progressive, recurrent inflammation of the choroid, retinal pigment epithelium, and choriocapillaris. In the classic or peripapillary geographic type of the disease, infiltrates originating in the peripapillary region progress in an irregular serpentine fashion centrifugally and resolve spontaneously after several weeks, leaving atrophic scars. Multiple recurrences, often with months to years of quiescence in between, result in progressive visual loss in one or both eyes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-35686/","orphanet_url":"https://www.orpha.net/en/disease/detail/35686","names":{"fr":{"name":"Choroïdite serpigineuse","synonyms":["Choroïdopathie géographique hélicoïdale péripapillaire"],"definition":"Uvéïte postérieure non infectieuse rare, caractérisée par une inflammation généralement bilatérale, chronique, progressive et récurrente de la choroïde, de l'épithélium pigmentaire de la rétine et des choriocapillaires. Dans le type géographique classique ou péripapillaire de la maladie, les infiltrats provenant de la région péripapillaire progressent en serpentin irrégulier de manière centrifuge et se résorbent spontanément après plusieurs semaines, laissant des cicatrices atrophiques. Les récidives multiples, souvent entrecoupées de mois ou d'années de quiescence, entraînent une perte visuelle progressive dans un oeil ou dans les deux."},"de":{"name":"Chorioditis, serpiginöse","synonyms":["Chorioidopathie, geografische helikoide peripapilläre"],"definition":"Eine seltene, nicht-infektiöse posteriore Uveitis, die durch eine meist bilaterale, chronische, progrediente, rezidivierende Entzündung der Aderhaut, des retinalen Pigmentepithels und der Choriocapillaris gekennzeichnet ist. Beim klassischen oder peripapillären geografischen Typ der Erkrankung schreiten Infiltrate, die ihren Ursprung in der peripapillären Region haben, unregelmäßig serpentinenförmig zentrifugal voran und bilden sich nach einigen Wochen spontan zurück und hinterlassen atrophische Narben. Mehrfache Rezidive, oft mit Monaten bis Jahren der Ruhe dazwischen, führen zu einem fortschreitenden Sehverlust in einem oder beiden Augen."},"es":{"name":"Coroiditis serpiginosa","synonyms":["Coroidopatía geográfica helicoidal peripapilar"],"definition":"Es una uveítis posterior no infecciosa poco frecuente caracterizada por una inflamación recurrente, progresiva, crónica y generalmente bilateral de la coroides, el epitelio pigmentario retiniano y la coriocapilar. En el tipo geográfico clásico o peripapilar de la enfermedad, los infiltrados que se originan en la región peripapilar progresan de manera centrífuga a modo de serpentina irregular y se resuelven espontáneamente después de varias semanas, dejando cicatrices atróficas. Las múltiples recurrencias, a menudo con meses o años de latencia, conllevan pérdida visual progresiva en uno o en ambos ojos."},"it":{"name":"Coroidite serpiginosa","synonyms":["Coroidopatia geografica elicoidale peripapillare"],"definition":"Si tratta di un'uveite posteriore rara non infettiva, caratterizzata da un'infiammazione in genere bilaterale, cronica, progressiva e ricorrente della coroide, dell'epitelio pigmentato della retina e dei capillari coroidei. Nella forma classica o localizzata peripapillare della malattia, gli infiltrati che originano nella zona peripapillare progrediscono in maniera centrifuga sotto forma di una serpentina irregolare, e si risolvono spontaneamente nell'arco di alcune settimane, lasciando cicatrici atrofiche. Le recidive multiple, che spesso di ripetono a distanza di alcuni mesi o anni, causano la perdita progressiva della vista in uno o in entrambi gli occhi."},"nl":{"name":"Serpigineuze choroïditis","synonyms":["Geografische helicoïde peripapillaire choroïdopathie"],"definition":"Een zeldzame, niet-infectieuze posterieure uveïtis, gekarakteriseerd door meestal bilaterale, chronische, progressieve, recidiverende inflammatie van vaatvlies, retinaal pigmentepitheel, en lamina choroidocapillaris. In geval van klassiek of peripapillair geografisch type van de ziekte evolueren infiltraten die ontstaan in de peripapillaire regio op onregelmatige en slingerende wijze centrifugaal en deze verdwijnen spontaan na enkele weken, waarbij atrofische littekens overblijven. Multipele recidieven, vaak met rustperiodes van maanden tot jaren tussenin, resulteren in progressief verlies van gezichtsvermogen in een of beide ogen."},"pt":{"name":"Coroidite em serpentina","synonyms":["Coroidopatia helicoidal peripapilar geográfica"]},"pl":{"name":"Choroidopatia pełzająca","synonyms":["Geographic helicoid peripapillary choroidopathy"]},"cs":{"name":"Serpiginózní choroiditida","synonyms":["Geografická helikoidální peripapilární choroidopatie"]},"uk":{"name":"Серпігінозний хоріоїдит","synonyms":["Географічна гелікоїдна перипапілярна хоріодопатія"],"definition":"A rare non-infectious posterior uveitis characterized by usually bilateral, chronic, progressive, recurrent inflammation of the choroid, retinal pigment epithelium, and choriocapillaris. In the classic or peripapillary geographic type of the disease, infiltrates originating in the peripapillary region progress in an irregular serpentine fashion centrifugally and resolve spontaneously after several weeks, leaving atrophic scars. Multiple recurrences, often with months to years of quiescence in between, result in progressive visual loss in one or both eyes."},"zh":{"name":"匐行性脉络膜炎","synonyms":["匐行性脉络膜萎缩"]},"tr":{"name":"Serpijinöz koroidit","synonyms":["Coğrafi helikoid peripapiller koroidopati"]}},"xrefs":[{"source":"ICD-10","code":"H30.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9B65.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1404422398"},{"source":"MONDO","code":"0018152","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0729842","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"31","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10081652","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":90061,"name":"Non-infectious posterior uveitis","classifications":[189]},{"orpha":716195,"name":"Rare inflammatory choroidal disorder","classifications":[189]},{"orpha":716348,"name":"Rare isolated progressive predominantly chorioretinal disorder","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}