{"orpha":279914,"name":"Intermediate uveitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["IU"],"definition":"A rare ophthalmic disorder characterized by intraocular inflammation primarily localized to the vitreous and peripheral retina. It incorporates pars planitis, posterior cyclitis, and hyalitis. Patients present with painless floaters, decreased or blurred vision, less frequently with pain, redness, and photophobia. On examination, snow banking, vitreous snowballs, peripheral retinal vascular sheathing, vitreous cells, and vitreous haze can be seen. Complications include epiretinal membrane formation, cataract formation, cystoid macular edema, or band keratopathy, among others. The condition may be idiopathic or occur in the context of infectious or systemic diseases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-279914/","orphanet_url":"https://www.orpha.net/en/disease/detail/279914","names":{"fr":{"name":"Uvéite intermédiaire","synonyms":["IU"],"definition":"Maladie ophtalmique rare caractérisée par une inflammation intraoculaire principalement localisée au niveau du vitré et de la rétine périphérique. Elle comprend la pars planite, la cyclite postérieure et la hyalite. Les patients présentent des corps flottants indolores, une diminution de la vision ou une vision floue, plus rarement une douleur, une rougeur et une photophobie. À l'examen, on peut observer la formation des bancs de neige, des boules de neige vitréennes, un engainement vasculaire rétinien périphérique, des cellules vitréennes et un voile vitréen. La formation, entre autres, d'une membrane épirétinienne, d'une cataracte, un oedème maculaire cystoïde ou une kératopathie en bande, fait partie des complications. La maladie peut être idiopathique ou survenir dans le contexte de maladies infectieuses ou systémiques."},"de":{"name":"Uveitis, intermediäre","synonyms":["IU"],"definition":"Eine seltene Augenerkrankung, die durch eine intraokulare Entzündung gekennzeichnet ist, die sich hauptsächlich auf den Glaskörper und die periphere Netzhaut beschränkt. Sie umfasst Pars planitis, posteriore Zyklitis und Hyalitis. Die Patienten präsentieren sich mit schmerzlosen Floater, verminderter oder verschwommener Sicht, seltener mit Schmerzen, Rötung und Photophobie. Bei der Untersuchung lassen sich Schneebänke, Glaskörperschneebälle, periphere Netzhautgefäßauskleidungen, Glaskörperzellen und Glaskörpertrübungen erkennen. Zu den Komplikationen gehören u. a. die Bildung einer epiretinalen Membran, Kataraktbildung, zystoides Makulaödem oder Bandkeratopathie. Die Erkrankung kann idiopathisch sein oder im Zusammenhang mit infektiösen oder systemischen Erkrankungen auftreten."},"es":{"name":"Uveítis intermedia","synonyms":["UI"],"definition":"Es un trastorno oftálmico poco frecuente caracterizado por inflamación intraocular localizada principalmente en el vítreo y en la retina periférica. Se manifiesta como pars planitis, ciclitis posterior e hialitis. Los pacientes manifiestan visión de cuerpos flotantes indoloros, visión disminuida o borrosa y, con menor frecuencia, dolor, enrojecimiento y fotofobia. En el reconocimiento, pueden observarse ''bancos de nieve'', ''bolas de nieve'' en el vítreo, revestimiento retiniano perivascular, células vítreas y neblina vítrea. Las complicaciones incluyen formación de membrana epirretiniana, formación de cataratas, edema macular cistoide o queratopatía en banda, entre otras. La condición puede ser idiopática u ocurrir en el contexto de enfermedades infecciosas o sistémicas."},"it":{"name":"Uveite intermedia","synonyms":["IU"],"definition":"Si tratta di una malattia oculare rara, caratterizzata da infiammazione intraoculare che interessa soprattutto il vitreo e la porzione periferica della retina. Comprende l'infiammazione della pars plana, la ciclite posteriore e la ialite. I pazienti presentano miodesopsie indolori, riduzione o offuscamento della vista e, più raramente, dolore, arrossamento e fotofobia. L'esame dell'occhio mostra formazioni con aspetto ''a banco di neve'' o ''a palla di neve'' a livello del vitreo, inguainamento vascolare della retina periferica, cellule vitreali e opacità del vitreo. Le complicanze comprendono la formazione di una membrana epiretinica, la cataratta, l'edema maculare cistoide e la cheratopatia a banda. La malattia può essere idiopatica o associata a malattie infettive o sistemiche."},"nl":{"name":"Intermediaire uveïtis","synonyms":["IU"],"definition":"Een zeldzame aandoening van oog, gekarakteriseerd door intraoculaire inflammatie die zich voornamelijk situeert in glasachtig lichaam en perifeer netvlies. Het omvat pars planitis, posterieure cyclitis, en hyalitis. Patiënten presenteren zich met pijnlijke glasvochttroebeling, verminderd of troebel zicht, en minder vaak met pijn, roodheid, en fotofobie. Bij onderzoek kunnen vertroebeling, witte afzettingen in glasvocht ('snowballs'), perifere retinale vasculaire schedevorming, cellen in glasachtig lichaam, en vertroebeling van glasvocht waargenomen worden. Complicaties zijn onder meer vorming van epiretinale membraan, vorming van cataract, cystoid macula-oedeem, of bandkeratopathie. De aandoening kan idiopathisch zijn of voorkomen in de context van infectieuze of systemische ziekten."},"pt":{"name":"Uveíte intermédia","synonyms":["IU"]},"pl":{"name":"Zapalenie pośredniej części błony naczyniowej oka","synonyms":["IU"]},"cs":{"name":"Intermediální uveitida","synonyms":["IU"]},"uk":{"name":"Помірно виражений увеїт","synonyms":["IU"],"definition":"A rare ophthalmic disorder characterized by intraocular inflammation primarily localized to the vitreous and peripheral retina. It incorporates pars planitis, posterior cyclitis, and hyalitis. Patients present with painless floaters, decreased or blurred vision, less frequently with pain, redness, and photophobia. On examination, snow banking, vitreous snowballs, peripheral retinal vascular sheathing, vitreous cells, and vitreous haze can be seen. Complications include epiretinal membrane formation, cataract formation, cystoid macular edema, or band keratopathy, among others. The condition may be idiopathic or occur in the context of infectious or systemic diseases."},"zh":{"name":"中间葡萄膜炎","synonyms":["IU"]},"tr":{"name":"Orta düzey üveit","synonyms":["IU"]}},"xrefs":[{"source":"ICD-10","code":"H30.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9B66","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"892860325"},{"source":"MONDO","code":"0006806","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D015867","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0042166","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10022557","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":98715,"name":"Uveitis","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-07-05","source":"29370567[PMID]_28449695[PMID]_31082037[PMID]","terms":[{"hpo":"HP:0040049","label":"Macular edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012122","label":"Anterior uveitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000585","label":"Band keratopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000518","label":"Cataract","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011505","label":"Cystoid macular edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100014","label":"Epiretinal membrane","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000501","label":"Glaucoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100653","label":"Optic neuritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011484","label":"Posterior synechiae of the anterior chamber","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007663","label":"Reduced visual acuity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002633","label":"Vasculitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100832","label":"Vitreous floaters","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030652","label":"Vitreous haze","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030661","label":"Vitreous snowballs","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031035","label":"Chronic infection","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0200056","label":"Macular scar","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003765","label":"Psoriasiform dermatitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001970","label":"Tubulointerstitial nephritis","frequency":"Very rare (<4-1%)","criteria":null}]}}