Uwiteus – Rokowania, prognozy i postęp choroby

Uwiteus
Rokowania, prognozy i postęp choroby

Zapalenie błony naczyniowej (uveitis) jest istotną przyczyną utraty wzroku w krajach rozwiniętych, jednak przy wczesnej diagnozie i odpowiednim leczeniu rokowanie jest zazwyczaj dobre. Kluczowe czynniki wpływające na rokowanie to wiek wystąpienia choroby (starszy wiek koreluje z lepszym rokowaniem, zwłaszcza w pars planitis), czas do rozpoczęcia terapii (opóźnienie pogarsza rokowanie), lokalizacja zapalenia (segment tylny oka wiąże się z gorszym wynikiem), wyjściowa ostrość wzroku oraz obecność powikłań strukturalnych, takich jak torbielowaty obrzęk plamki czy zmiany na granicy szklistkowo-siatkówkowej. Przednie zapalenie błony naczyniowej, choć generalnie rokuje lepiej, w przewlekłych i nawracających postaciach wiąże się z ryzykiem jaskry, które wzrasta wraz z opóźnieniem w rozpoczęciu leczenia immunomodulującego (współczynnik ryzyka 1,006; 95% CI: 1,001-1,012; P=0,019). Wczesne wdrożenie leków immunomodulujących w ciągu pierwszych 2 lat choroby jest zalecane dla poprawy ostrości wzroku.

Rokowanie w zapaleniu błony naczyniowej (Uveitis/Uwiteus): przewidywanie wyników leczenia

Czynniki prognostyczne wpływające na wyniki leczenia
Rokowanie w różnych typach zapalenia błony naczyniowej
Rokowanie w zapaleniu błony naczyniowej u dzieci
Znaczenie specjalistycznej opieki w rokowaniu
Wpływ zabiegów chirurgicznych na rokowanie
Standaryzacja oceny wyników leczenia
Czynniki prognostyczne w obrzęku plamki związanym z zapaleniem błony naczyniowej
Najczęstsze powikłania długoterminowe wpływające na rokowanie
Kluczowe elementy wpływające na rokowanie
Kolejne rozdziały

Rokowanie w zapaleniu błony naczyniowej (Uveitis/Uwiteus): przewidywanie wyników leczenia

Uwiteus, będący jedną z głównych przyczyn utraty wzroku w krajach rozwiniętych, stanowi poważne wyzwanie terapeutyczne wymagające wczesnej diagnozy oraz właściwego leczenia. W większości przypadków, przy odpowiednim postępowaniu terapeutycznym, rokowanie w zapaleniu błony naczyniowej jest dobre. Mimo że znaczna część pacjentów rozwinie powikłania oczne, wczesne rozpoznanie i właściwe leczenie, w tym ewentualne zabiegi chirurgiczne, znacząco zmniejszają ryzyko trwałej utraty wzroku.¹² W Stanach Zjednoczonych około 10% przypadków prawnej ślepoty związanych jest bezpośrednio lub pośrednio z zapaleniem błony naczyniowej, przy czym negatywne wyniki leczenia są bardziej prawdopodobne w przypadku opóźnionej diagnozy i terapii.³

Uwiteus nie jest chorobą dziedziczną ani zwyrodnieniową, dlatego też nie ma charakteru postępującego. Choć niewątpliwie wpływa na jakość życia pacjentów, szczególnie w przypadku długotrwałych terapii, zasadniczo jest to schorzenie, które można kontrolować, a pacjenci z zapaleniem błony naczyniowej nie muszą stracić wzroku. Obecne podejście diagnostyczne i terapeutyczne, oparte na multidyscyplinarnej współpracy oraz nowoczesnych lekach stosowanych w zapaleniach błony naczyniowej o podłożu immunologicznym, zapewnia szybsze działanie i lepsze rokowanie dotyczące widzenia.⁴

Czynniki prognostyczne wpływające na wyniki leczenia

Badania wykazały szereg czynników, które mogą wpływać na długoterminowe rokowanie u pacjentów z zapaleniem błony naczyniowej:

Wiek wystąpienia choroby – starszy wiek w momencie początku choroby wiąże się z lepszym rokowaniem, szczególnie w zapaleniu typu pars planitis⁵⁶
Czas do rozpoczęcia leczenia – opóźniona diagnoza i leczenie znacząco pogarszają rokowanie⁷⁸
Lokalizacja zapalenia – zapalenie obejmujące segment tylny oka wiąże się z gorszym rokowaniem⁹
Wyjściowa ostrość wzroku – niska początkowa ostrość widzenia negatywnie wpływa na końcowy wynik funkcjonalny¹⁰
Obecność powikłań strukturalnych – występowanie torbielowatego obrzęku plamki żółtej, zmian na granicy szklistkowo-siatkówkowej oraz grubość centralnej siatkówki są czynnikami negatywnie wpływającymi na rokowanie¹¹¹²

Rokowanie w różnych typach zapalenia błony naczyniowej

Przednie zapalenie błony naczyniowej

Przednie zapalenie błony naczyniowej (anterior uveitis) tradycyjnie uważane jest za typ o lepszym rokowaniu w porównaniu do zapalenia pośredniego, tylnego czy całej błony naczyniowej (panuveitis). Jednak nawet w tym przypadku, przewlekłe i nawracające postacie wiążą się z większym niż oczekiwano ryzykiem powikłań ocznych, konieczności zabiegów chirurgicznych i upośledzenia widzenia.¹³

Badania wykazały, że ryzyko rozwoju jaskry koreluje z dłuższym okresem między diagnozą a rozpoczęciem leczenia immunomodulującego (współczynnik ryzyka = 1,006, 95% CI: 1,001-1,012, P = 0,019). Wczesne wdrożenie leków immunomodulujących (IMA) u pacjentów z przewlekłym lub nawracającym przednim zapaleniem błony naczyniowej w ciągu pierwszych 2 lat choroby może być związane z lepszą ostrością wzroku i powinno być uwzględnione w schemacie leczenia tych pacjentów.¹⁴

Pośrednie zapalenie błony naczyniowej

Naturalny przebieg pośredniego zapalenia błony naczyniowej (intermediate uveitis, IU) jest zmienny. Chociaż ogólnie uważane jest za chorobę łagodną, wiele przypadków wykazuje przedłużony przebieg z zaostrzeniami, a tylko około 10% pacjentów może mieć samoograniczający się przebieg choroby. Ponadto wielu pacjentów rozwija powikłania, które mogą prowadzić do utraty wzroku.¹⁵

W przypadku pars planitis u dzieci rokowanie wzrokowe jest generalnie korzystne, jeśli odpowiednie leczenie zostanie wdrożone we wczesnym stadium choroby. W badaniu przeprowadzonym przez Donaldsona i wsp., spośród 24 oczu pacjentów, 75% utrzymało ostrość wzroku 20/40 lub lepszą po 10 latach obserwacji. Ciężka utrata wzroku jest rzadka, ale istnieje wysoka częstość powikłań ocznych, takich jak obrzęk tarczy nerwu wzrokowego prowadzący do zaniku nerwu wzrokowego i obrzęku plamki, które są związane z upośledzeniem widzenia.¹⁶

Czynniki związane z lepszymi wynikami w pars planitis obejmują starszy wiek w momencie wystąpienia choroby i szybkie rozpoczęcie systemowego leczenia immunosupresyjnego oszczędzającego steroidy. Zaleca się ciągłe monitorowanie w celu oceny powikłań związanych z zapaleniem błony naczyniowej i stosowaniem steroidów, takich jak zaćma czy jaskra.¹⁷

Rokowanie w zapaleniu błony naczyniowej u dzieci

Najnowsze badania wskazują na poprawę rokowania wzrokowego zarówno w idiopatycznym, jak i związanym z młodzieńczym idiopatycznym zapaleniem stawów (JIA) zapaleniu błony naczyniowej u dzieci. W badaniu populacyjnym obejmującym 119 pacjentów w wieku do 16 lat z zapaleniem błony naczyniowej (23% z idiopatycznym zapaleniem i 77% z zapaleniem związanym z JIA), większość pacjentów miała prawidłową ostrość wzroku (Snellen 0,8 [6/7,5]) w dotkniętym chorobą oku, a obuocznie w 85% przypadków idiopatycznego zapalenia i 70% przypadków związanych z JIA. Tylko 5 pacjentów (4%) miało upośledzenie widzenia w jednym oku, a żaden nie miał upośledzenia widzenia w obu oczach.¹⁸

Wczesna diagnoza zapalenia błony naczyniowej i skuteczne leczenie nowoczesnymi lekami, w tym lekami modyfikującymi przebieg choroby (DMARD) oraz biologicznymi lekami modyfikującymi przebieg choroby (bDMARD) są kluczowe w zapobieganiu utracie wzroku. Odsetek upośledzenia widzenia zmniejszył się, a żaden pacjent w badanej populacji nie rozwinął upośledzenia widzenia.¹⁹

Jednakże, w przypadku dzieci z zapaleniem błony naczyniowej prezentujących ciężkie upośledzenie widzenia (ostrość wzroku ≤20/200) w momencie diagnozy, długoterminowe rokowanie dotyczące ostrości wzroku może być niekorzystne. Młodszy wiek w momencie wystąpienia zapalenia błony naczyniowej, opóźniona diagnoza oraz zapalenie obejmujące tylny segment oka były związane z gorszym wynikiem dotyczącym ostrości wzroku.²⁰

Znaczenie specjalistycznej opieki w rokowaniu

Badania wskazują, że średnia ostrość wzroku oczu z zapaleniem błony naczyniowej poprawiła się i zazwyczaj poprawa ta była utrzymana pod opieką subspecjalistów zajmujących się zapaleniem błony naczyniowej. Wyniki sugerują, że postępująca utrata wzroku występowała przed skierowaniem do specjalistów (36% do 45% pacjentów miało opiekę przez 2 lata przed skierowaniem), co wskazuje, że przypadki zapalenia błony naczyniowej mogą odnieść korzyść z wczesnego skierowania do subspecjalistów, gdy równoważna opieka nie jest dostępna w innym miejscu.²¹

Obserwacja, że przeciętny przypadek zapalenia błony naczyniowej pod specjalistyczną opieką ulega poprawie, sugeruje, że podejścia oparte na czasie do pogorszenia w badaniu ostrości wzroku jako wyników badań klinicznych mają istotne ograniczenia w badaniach nad leczeniem zapalenia błony naczyniowej.²²

Wpływ zabiegów chirurgicznych na rokowanie

Witrektomia

Witrektomia tylna przez część płaską ciała rzęskowego (PPV) ma korzystny wpływ na przebieg i powikłania przewlekłego zapalenia błony naczyniowej u dzieci i młodzieży w odniesieniu do wyników anatomicznych i funkcjonalnych. Analiza regresji wielorakiej wykazała, że przedoperacyjna ostrość wzroku (wyrażona jako logarytm minimalnej rozdzielczości kątowej – logMAR) oraz klinicznie istotny torbielowaty obrzęk plamki (CME) były najdokładniejszymi predyktorami wyniku funkcjonalnego. Niska przedoperacyjna ostrość wzroku i istniejący wcześniej CME miały negatywny wpływ na końcową ostrość wzroku.²³

Proponuje się rozważenie PPV jako opcji leczenia chirurgicznego we wszystkich przypadkach, gdy leki immunosupresyjne nie poprawiają funkcji wzrokowej i nie łagodzą aktywności zapalnej.²⁴

Standaryzacja oceny wyników leczenia

W celu lepszej oceny efektywności leczenia i rokowania, Wielonarodowa Interdyscyplinarna Grupa Robocza ds. Zapalenia Błony Naczyniowej u Dzieci zaproponowała swoiste i klinicznie istotne miary aktywności choroby, jej ciężkości i uszkodzeń w zapaleniu błony naczyniowej związanym z młodzieńczym idiopatycznym zapaleniem stawów (JIAU). Najbardziej znaczące zmiany od stanu wyjściowego do 6 miesięcy obserwowano w pomiarach aktywności komory przedniej (AC) dotyczących komórek i rozbłysku. Te pomiary korelują z obecnością wcześniej istniejących powikłań strukturalnych, co ma implikacje dla raportowania badań klinicznych wykorzystujących pojedynczy parametr jako główny punkt końcowy.²⁵

Zaproponowano również definicje skuteczności leczenia, remisji, nieaktywnej choroby i uszkodzeń dla JIAU. Badanie wykazało, że główne miary aktywności są moderowane przez współistniejące uszkodzenia, co potwierdza kliniczne przekonanie, że skuteczność leku prawdopodobnie nie będzie taka sama we wszystkich oczach, dlatego stratyfikacja według uszkodzeń chorobowych jest prawdopodobnie konieczna, aby uzyskać najlepszą miarę skuteczności leku.²⁶

Czynniki prognostyczne w obrzęku plamki związanym z zapaleniem błony naczyniowej

W badaniu oceniającym czynniki predykcyjne dla wyników wzrokowych i anatomicznych u pacjentów z obrzękiem plamki wtórnym do nieinfekcyjnego zapalenia błony naczyniowej, korzystne rokowanie obserwowano w 32,1% oczu. Rokowanie obrzęku plamki związanego z zapaleniem błony naczyniowej (UME) było negatywnie skorelowane z wyjściowym pogrubieniem dołka, zmianami w obrębie powierzchni szklistkowo-plamkowej i torbielowatym obrzękiem plamki.²⁷

Dostępne modalności leczenia UME mogą zapobiegać przewlekłemu UME i poprawiać wynik anatomiczny. Jednak odsetek poprawy funkcjonalnej obserwowanej podczas 12 miesięcy obserwacji jest niższy. Większa grubość centralnej siatkówki (CST), zmiany w obrębie powierzchni szklistkowo-plamkowej i torbielowaty obrzęk plamki mogą wskazywać na mniej korzystne rokowanie. Z kolei obustronność, choroba systemowa i komórki w komorze przedniej mogą być związane z korzystnym rokowaniem w UME.²⁸

Najczęstsze powikłania długoterminowe wpływające na rokowanie

Najczęstsze przyczyny słabego widzenia w długoterminowej obserwacji pacjentów z zapaleniem błony naczyniowej, szczególnie tych z ciężkim upośledzeniem wzroku przy prezentacji, obejmują:²⁹

Odwarstwienie siatkówki – prowadzące do trwałych uszkodzeń strukturalnych
Zanik gałki ocznej – w zaawansowanych i nieleczonych przypadkach
Zanik nerwu wzrokowego – jako konsekwencja długotrwałego zapalenia lub wysokiego ciśnienia wewnątrzgałkowego

Kluczowe elementy wpływające na rokowanie

Podsumowując, na podstawie dostępnych danych, następujące czynniki mają kluczowe znaczenie dla rokowania w zapaleniu błony naczyniowej:³⁰³¹³²³³

Wczesna diagnoza i leczenie – opóźnienie w rozpoznaniu i wdrożeniu terapii znacząco zwiększa ryzyko trwałej utraty wzroku
Współpraca multidyscyplinarna – opieka specjalistyczna z udziałem okulistów i reumatologów zapewnia lepsze wyniki leczenia
Adekwatna kontrola zapalenia – szybkie i skuteczne opanowanie stanu zapalnego ma kluczowe znaczenie dla zachowania funkcji wzrokowej
Wczesne rozpoznanie i leczenie powikłań – systematyczne monitorowanie powikłań pozwala na ich odpowiednio wczesne leczenie
Zastosowanie nowoczesnych terapii immunomodulujących – szczególnie w przypadkach zależnych od steroidów lub opornych na leczenie

Należy podkreślić, że zapalenie błony naczyniowej stanowi poważne zagrożenie dla wzroku, ale przy wczesnej diagnostyce i właściwym leczeniu, szanse na długoterminowe zachowanie funkcji wzrokowej są obecnie znacznie większe niż w przeszłości.³⁴³⁵

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Materiały źródłowe

#1 Uveitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK540993/
In most cases, the prognosis of uveitis is good assuming early detection and proper treatment. While a majority of patients will develop an ocular complication, appropriate treatment and surgery, if needed, make permanent vision loss much less likely. […] Identifying the underlying cause of uveitis is also important due to significant morbidity and mortality associated with some specific systemic diseases that can cause uveitis.
#2 Uveitis: Symptoms, Causes, Treatment & Types
https://my.clevelandclinic.org/health/diseases/14414-uveitis
Uveitis is a condition where you have inflammation of the uvea, which includes the iris, ciliary body and choroid. Its common, and vision loss is one of the possible complications (especially when uveitis is severe or goes untreated). […] In severe cases, uveitis (pronounced yoo-vee-EYE-tiss) can cause permanent vision loss and blindness. […] Uveitis is a common cause of vision loss and legal blindness. In the U.S., 10% of legal blindness cases happen because of, or in connection with, uveitis. Negative outcomes are more likely when there are delays in diagnosis and treatment. […] The odds of long-term vision issues are much lower with early diagnosis and treatment. When those happen, youre more likely to recover fully and avoid permanent vision issues.
#3 Uveitis: Symptoms, Causes, Treatment & Types
https://my.clevelandclinic.org/health/diseases/14414-uveitis
Uveitis is a condition where you have inflammation of the uvea, which includes the iris, ciliary body and choroid. Its common, and vision loss is one of the possible complications (especially when uveitis is severe or goes untreated). […] In severe cases, uveitis (pronounced yoo-vee-EYE-tiss) can cause permanent vision loss and blindness. […] Uveitis is a common cause of vision loss and legal blindness. In the U.S., 10% of legal blindness cases happen because of, or in connection with, uveitis. Negative outcomes are more likely when there are delays in diagnosis and treatment. […] The odds of long-term vision issues are much lower with early diagnosis and treatment. When those happen, youre more likely to recover fully and avoid permanent vision issues.
#4 Prognosis of Uveitis
https://www.clinicbarcelona.org/en/assistance/diseases/uveitis/disease-evolution
Uveitis is neither a hereditary or degenerative disease and so it is not progressive. It certainly has an impact on patient quality of life, especially in the case of long-term treatments, but in principle it is a disease that can be controlled and patients with uveitis will not go blind. […] Not only is diagnosis made increasingly sooner, the disease is also dealt with using a much more multidisciplinary approach. In addition, there are new medicines available for dealing with immune-mediated uveitis that now act more quickly and therefore offer a better visual prognosis.
#5 Intermediate Uveitis – EyeWiki
https://eyewiki.org/Intermediate_Uveitis
The natural history of IU is variable. Although generally considered a benign disease, many cases show a prolonged course with exacerbations, and only 10% of patients may have a self-limited course of disease. In addition, many patients develop complications that may lead to visual loss. Adequate control of inflammation and prompt detection and treatment of associated complications are thus essential in improving the overall prognosis of the patient. […] Overall, the visual prognosis of pars planitis in children is reported to be favorable if adequate treatment is implemented early in the disease course. In a study conducted by Donaldson, et. al., of 24 patient eyes, 75% maintained a visual acuity of 20/40 or better after 10 years of follow-up. […] Severe visual loss is uncommon, but there is a high incidence of ocular complications, such as optic disc edema leading to optic atrophy and macular edema, which are associated with visual impairment. Long-term follow-up suggests that although optic disc edema may indicate a risk for macular edema, it is noted that not all patients with optic disc edema develop substantial visual loss. […] Factors associated with better outcomes in pars planitis include older age of disease onset and prompt initiation of systemic steroid-sparing IMT. Ongoing monitoring is advised to assess for uveitic and steroid-induced complications such as cataracts or glaucoma.
#6 Long-term visual acuity outcome of pediatric uveitis patients presenting with severe visual impairment | Scientific Reports
https://www.nature.com/articles/s41598-023-29159-x
This study investigated the long-term visual acuity (VA) outcome in the eyes of children with uveitis and severe visual impairment (SVI; VA20/200) at presentation. […] The long-term VA outcome in uveitis-affected eyes with SVI at presentation was unfavorable. Younger age at uveitis onset, delayed presentation, and uveitis involving the posterior segment were associated with poorer VA outcome. […] A more favorable VA outcome was observed in eyes with uveitis onset at school age or older, anterior uveitis, and a shorter duration of ocular symptoms before uveitis diagnosis. […] The long-term course of VA outcome in this study demonstrated that, on average, eyes with both infectious and non-infectious uveitis experienced VA improvement shortly after receiving uveitis treatment. […] Although these improvements were statistically significant compared with the baseline VA, the final VA remained within the SVI level. […] The three most common causes of poor vision at final follow-up were RD, atrophic bulbi, and optic atrophy.
#7 Visual Acuity Outcome over Time in Non-Infectious Uveitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC7282970/
Mean VA of uveitic eyes improved and, typically, improvement was sustained under uveitis subspecialty care. […] The results imply that progressive visual loss occurred prior to referral (36% to 45% had care for 2 years prior to referral), suggesting that uveitis cases may benefit from early referral to uveitis subspecialists when equivalent care is not available in another setting. […] The observation that the average uveitis case under tertiary uveitis care improves suggests that time-to-worsening approaches to studying visual acuity as clinical research outcomes have important limitations in studying treatments of uveitis.
#8 Uveitis: Symptoms, Causes, Treatment & Types
https://my.clevelandclinic.org/health/diseases/14414-uveitis
Uveitis is a condition where you have inflammation of the uvea, which includes the iris, ciliary body and choroid. Its common, and vision loss is one of the possible complications (especially when uveitis is severe or goes untreated). […] In severe cases, uveitis (pronounced yoo-vee-EYE-tiss) can cause permanent vision loss and blindness. […] Uveitis is a common cause of vision loss and legal blindness. In the U.S., 10% of legal blindness cases happen because of, or in connection with, uveitis. Negative outcomes are more likely when there are delays in diagnosis and treatment. […] The odds of long-term vision issues are much lower with early diagnosis and treatment. When those happen, youre more likely to recover fully and avoid permanent vision issues.
#9 Long-term visual acuity outcome of pediatric uveitis patients presenting with severe visual impairment | Scientific Reports
https://www.nature.com/articles/s41598-023-29159-x
This study investigated the long-term visual acuity (VA) outcome in the eyes of children with uveitis and severe visual impairment (SVI; VA20/200) at presentation. […] The long-term VA outcome in uveitis-affected eyes with SVI at presentation was unfavorable. Younger age at uveitis onset, delayed presentation, and uveitis involving the posterior segment were associated with poorer VA outcome. […] A more favorable VA outcome was observed in eyes with uveitis onset at school age or older, anterior uveitis, and a shorter duration of ocular symptoms before uveitis diagnosis. […] The long-term course of VA outcome in this study demonstrated that, on average, eyes with both infectious and non-infectious uveitis experienced VA improvement shortly after receiving uveitis treatment. […] Although these improvements were statistically significant compared with the baseline VA, the final VA remained within the SVI level. […] The three most common causes of poor vision at final follow-up were RD, atrophic bulbi, and optic atrophy.
#10 Vitrectomy for juvenile uveitis: prognostic factors for the long-term functional outcome | Eye
https://www.nature.com/articles/6701845
PPV has a beneficial effect on the course and the complications of chronic uveitis in paediatric and juvenile patients with respect to the anatomical and visual outcome. […] Preoperative logMAR visual acuity and clinically significant CME were the most accurate predictors for the functional outcome in a way that a low preoperative logMAR visual acuity and a pre-existing CME had a negative impact on final logMAR visual acuity. […] Multiple regression analysis revealed that only two of the tested independent predictor variables, namely preoperative logMAR acuity and clinically present CME were significantly associated with the final postoperative logMAR acuity in our population. […] The results of the multiple regression analysis indicate that a low preoperative logMAR visual acuity and the existence of a CME had a negative influence on the final logMAR acuity. […] We propose PPV as a surgical treatment option in all cases where immunosuppressive medication fails to improve visual function and to resolve inflammatory activity.
#11 Predictors for functional and anatomic outcomes in macular edema secondary to non-infectious uveitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210799
We aimed to investigate predictive factors for visual and anatomic outcomes in patients with macular edema secondary to non-infectious uveitis. […] Favorable prognosis, as defined above, was observed in 18 (32.1%) eyes. UME prognosis was negatively correlated with baseline foveal thickening, alteration in the vitreo-macular interface and cystoid macular edema. […] Available treatment modalities in UME may avoid chronic UME and improve anatomic outcome. However, the proportion of functional amelioration observed during 12 months of follow-up is lower. Thicker CST, alteration in the vitreo-macular interface and cystoid macular edema may denote less favorable prognosis. Conversely, bilaterally, systemic disease and anterior chamber cells may be associated with favorable prognosis in UME.
#12 Vitrectomy for juvenile uveitis: prognostic factors for the long-term functional outcome | Eye
https://www.nature.com/articles/6701845
PPV has a beneficial effect on the course and the complications of chronic uveitis in paediatric and juvenile patients with respect to the anatomical and visual outcome. […] Preoperative logMAR visual acuity and clinically significant CME were the most accurate predictors for the functional outcome in a way that a low preoperative logMAR visual acuity and a pre-existing CME had a negative impact on final logMAR visual acuity. […] Multiple regression analysis revealed that only two of the tested independent predictor variables, namely preoperative logMAR acuity and clinically present CME were significantly associated with the final postoperative logMAR acuity in our population. […] The results of the multiple regression analysis indicate that a low preoperative logMAR visual acuity and the existence of a CME had a negative influence on the final logMAR acuity. […] We propose PPV as a surgical treatment option in all cases where immunosuppressive medication fails to improve visual function and to resolve inflammatory activity.
#13
https://journals.lww.com/ijo/fulltext/2024/02002/recurrent_and_chronic_anterior_uveitis__long_term.15.aspx
To study the long-term clinical outcome and treatment strategies of recurrent and chronic non-infectious anterior uveitis. […] Chronic and recurrent anterior uveitis were associated with greater risk than expected for ocular complications, surgeries, and vision impairment. Early initiation of immunomodulation should be strongly considered to improve clinical course and outcome. […] Anterior uveitis is considered to have a better prognosis in terms of visual acuity and rate of ocular complications compared to other types such as intermediate, posterior, and pan-uveitis. […] The risk of developing glaucoma correlated with a longer interval between diagnosis and starting IMAs (hazard ratio = 1.006, 95% CI: 1.0011.012, P = 0.019). […] Therefore, we infer that initiation of IMAs in patients with chronic or recurrent anterior uveitis in the first 2 years of the disease may be associated with better visual acuity and should be considered in the treatment regimen of these patients. […] The purpose of this study was to raise awareness for cases of recurrent and chronic anterior uveitis that are steroid-dependent, suffer from subsequent ocular morbidity, and may benefit from steroid-sparing IMAs.
#14
https://journals.lww.com/ijo/fulltext/2024/02002/recurrent_and_chronic_anterior_uveitis__long_term.15.aspx
To study the long-term clinical outcome and treatment strategies of recurrent and chronic non-infectious anterior uveitis. […] Chronic and recurrent anterior uveitis were associated with greater risk than expected for ocular complications, surgeries, and vision impairment. Early initiation of immunomodulation should be strongly considered to improve clinical course and outcome. […] Anterior uveitis is considered to have a better prognosis in terms of visual acuity and rate of ocular complications compared to other types such as intermediate, posterior, and pan-uveitis. […] The risk of developing glaucoma correlated with a longer interval between diagnosis and starting IMAs (hazard ratio = 1.006, 95% CI: 1.0011.012, P = 0.019). […] Therefore, we infer that initiation of IMAs in patients with chronic or recurrent anterior uveitis in the first 2 years of the disease may be associated with better visual acuity and should be considered in the treatment regimen of these patients. […] The purpose of this study was to raise awareness for cases of recurrent and chronic anterior uveitis that are steroid-dependent, suffer from subsequent ocular morbidity, and may benefit from steroid-sparing IMAs.
#15 Intermediate Uveitis – EyeWiki
https://eyewiki.org/Intermediate_Uveitis
The natural history of IU is variable. Although generally considered a benign disease, many cases show a prolonged course with exacerbations, and only 10% of patients may have a self-limited course of disease. In addition, many patients develop complications that may lead to visual loss. Adequate control of inflammation and prompt detection and treatment of associated complications are thus essential in improving the overall prognosis of the patient. […] Overall, the visual prognosis of pars planitis in children is reported to be favorable if adequate treatment is implemented early in the disease course. In a study conducted by Donaldson, et. al., of 24 patient eyes, 75% maintained a visual acuity of 20/40 or better after 10 years of follow-up. […] Severe visual loss is uncommon, but there is a high incidence of ocular complications, such as optic disc edema leading to optic atrophy and macular edema, which are associated with visual impairment. Long-term follow-up suggests that although optic disc edema may indicate a risk for macular edema, it is noted that not all patients with optic disc edema develop substantial visual loss. […] Factors associated with better outcomes in pars planitis include older age of disease onset and prompt initiation of systemic steroid-sparing IMT. Ongoing monitoring is advised to assess for uveitic and steroid-induced complications such as cataracts or glaucoma.
#16 Intermediate Uveitis – EyeWiki
https://eyewiki.org/Intermediate_Uveitis
The natural history of IU is variable. Although generally considered a benign disease, many cases show a prolonged course with exacerbations, and only 10% of patients may have a self-limited course of disease. In addition, many patients develop complications that may lead to visual loss. Adequate control of inflammation and prompt detection and treatment of associated complications are thus essential in improving the overall prognosis of the patient. […] Overall, the visual prognosis of pars planitis in children is reported to be favorable if adequate treatment is implemented early in the disease course. In a study conducted by Donaldson, et. al., of 24 patient eyes, 75% maintained a visual acuity of 20/40 or better after 10 years of follow-up. […] Severe visual loss is uncommon, but there is a high incidence of ocular complications, such as optic disc edema leading to optic atrophy and macular edema, which are associated with visual impairment. Long-term follow-up suggests that although optic disc edema may indicate a risk for macular edema, it is noted that not all patients with optic disc edema develop substantial visual loss. […] Factors associated with better outcomes in pars planitis include older age of disease onset and prompt initiation of systemic steroid-sparing IMT. Ongoing monitoring is advised to assess for uveitic and steroid-induced complications such as cataracts or glaucoma.
#17 Intermediate Uveitis – EyeWiki
https://eyewiki.org/Intermediate_Uveitis
The natural history of IU is variable. Although generally considered a benign disease, many cases show a prolonged course with exacerbations, and only 10% of patients may have a self-limited course of disease. In addition, many patients develop complications that may lead to visual loss. Adequate control of inflammation and prompt detection and treatment of associated complications are thus essential in improving the overall prognosis of the patient. […] Overall, the visual prognosis of pars planitis in children is reported to be favorable if adequate treatment is implemented early in the disease course. In a study conducted by Donaldson, et. al., of 24 patient eyes, 75% maintained a visual acuity of 20/40 or better after 10 years of follow-up. […] Severe visual loss is uncommon, but there is a high incidence of ocular complications, such as optic disc edema leading to optic atrophy and macular edema, which are associated with visual impairment. Long-term follow-up suggests that although optic disc edema may indicate a risk for macular edema, it is noted that not all patients with optic disc edema develop substantial visual loss. […] Factors associated with better outcomes in pars planitis include older age of disease onset and prompt initiation of systemic steroid-sparing IMT. Ongoing monitoring is advised to assess for uveitic and steroid-induced complications such as cataracts or glaucoma.
#18 Encouraging visual outcomes in children with idiopathic and JIA associated uveitis: a population-based study | Pediatric Rheumatology | Full Text
https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-023-00841-8
A total of 119 patients aged 16 years with uveitis were included. Uveitis was idio-U in 23% and associated with JIA in 77% of cases. Most patients had normal visual acuity (Snellen 0.8, [6/7.5]) in the affected eye and bilaterally in 85% idio-U and 70% JIA-U. Only 5 patients (4%) had visual impairment in one, but none in both eyes. […] Children with uveitis have good visual acuity and a low rate for visual impairment. In addition, modern treatment with DMARDs and bDMARDs seems to save vision. […] Our results show improvement in visual prognosis of idio-U and JIA-U in children. Early diagnosis of uveitis and efficient treatment with modern medications including DMARDs and bDMARDs are crucial in preventing visual loss. The rate for visual impairment has decreased and no patient in the present study population developed visual impairment.
#19 Encouraging visual outcomes in children with idiopathic and JIA associated uveitis: a population-based study | Pediatric Rheumatology | Full Text
https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-023-00841-8
A total of 119 patients aged 16 years with uveitis were included. Uveitis was idio-U in 23% and associated with JIA in 77% of cases. Most patients had normal visual acuity (Snellen 0.8, [6/7.5]) in the affected eye and bilaterally in 85% idio-U and 70% JIA-U. Only 5 patients (4%) had visual impairment in one, but none in both eyes. […] Children with uveitis have good visual acuity and a low rate for visual impairment. In addition, modern treatment with DMARDs and bDMARDs seems to save vision. […] Our results show improvement in visual prognosis of idio-U and JIA-U in children. Early diagnosis of uveitis and efficient treatment with modern medications including DMARDs and bDMARDs are crucial in preventing visual loss. The rate for visual impairment has decreased and no patient in the present study population developed visual impairment.
#20 Long-term visual acuity outcome of pediatric uveitis patients presenting with severe visual impairment | Scientific Reports
https://www.nature.com/articles/s41598-023-29159-x
This study investigated the long-term visual acuity (VA) outcome in the eyes of children with uveitis and severe visual impairment (SVI; VA20/200) at presentation. […] The long-term VA outcome in uveitis-affected eyes with SVI at presentation was unfavorable. Younger age at uveitis onset, delayed presentation, and uveitis involving the posterior segment were associated with poorer VA outcome. […] A more favorable VA outcome was observed in eyes with uveitis onset at school age or older, anterior uveitis, and a shorter duration of ocular symptoms before uveitis diagnosis. […] The long-term course of VA outcome in this study demonstrated that, on average, eyes with both infectious and non-infectious uveitis experienced VA improvement shortly after receiving uveitis treatment. […] Although these improvements were statistically significant compared with the baseline VA, the final VA remained within the SVI level. […] The three most common causes of poor vision at final follow-up were RD, atrophic bulbi, and optic atrophy.
#21 Visual Acuity Outcome over Time in Non-Infectious Uveitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC7282970/
Mean VA of uveitic eyes improved and, typically, improvement was sustained under uveitis subspecialty care. […] The results imply that progressive visual loss occurred prior to referral (36% to 45% had care for 2 years prior to referral), suggesting that uveitis cases may benefit from early referral to uveitis subspecialists when equivalent care is not available in another setting. […] The observation that the average uveitis case under tertiary uveitis care improves suggests that time-to-worsening approaches to studying visual acuity as clinical research outcomes have important limitations in studying treatments of uveitis.
#22 Visual Acuity Outcome over Time in Non-Infectious Uveitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC7282970/
Mean VA of uveitic eyes improved and, typically, improvement was sustained under uveitis subspecialty care. […] The results imply that progressive visual loss occurred prior to referral (36% to 45% had care for 2 years prior to referral), suggesting that uveitis cases may benefit from early referral to uveitis subspecialists when equivalent care is not available in another setting. […] The observation that the average uveitis case under tertiary uveitis care improves suggests that time-to-worsening approaches to studying visual acuity as clinical research outcomes have important limitations in studying treatments of uveitis.
#23 Vitrectomy for juvenile uveitis: prognostic factors for the long-term functional outcome | Eye
https://www.nature.com/articles/6701845
PPV has a beneficial effect on the course and the complications of chronic uveitis in paediatric and juvenile patients with respect to the anatomical and visual outcome. […] Preoperative logMAR visual acuity and clinically significant CME were the most accurate predictors for the functional outcome in a way that a low preoperative logMAR visual acuity and a pre-existing CME had a negative impact on final logMAR visual acuity. […] Multiple regression analysis revealed that only two of the tested independent predictor variables, namely preoperative logMAR acuity and clinically present CME were significantly associated with the final postoperative logMAR acuity in our population. […] The results of the multiple regression analysis indicate that a low preoperative logMAR visual acuity and the existence of a CME had a negative influence on the final logMAR acuity. […] We propose PPV as a surgical treatment option in all cases where immunosuppressive medication fails to improve visual function and to resolve inflammatory activity.
#24 Vitrectomy for juvenile uveitis: prognostic factors for the long-term functional outcome | Eye
https://www.nature.com/articles/6701845
PPV has a beneficial effect on the course and the complications of chronic uveitis in paediatric and juvenile patients with respect to the anatomical and visual outcome. […] Preoperative logMAR visual acuity and clinically significant CME were the most accurate predictors for the functional outcome in a way that a low preoperative logMAR visual acuity and a pre-existing CME had a negative impact on final logMAR visual acuity. […] Multiple regression analysis revealed that only two of the tested independent predictor variables, namely preoperative logMAR acuity and clinically present CME were significantly associated with the final postoperative logMAR acuity in our population. […] The results of the multiple regression analysis indicate that a low preoperative logMAR visual acuity and the existence of a CME had a negative influence on the final logMAR acuity. […] We propose PPV as a surgical treatment option in all cases where immunosuppressive medication fails to improve visual function and to resolve inflammatory activity.
#25 Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (
https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-019-0345-2
JIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. […] The main findings are that the most significant changes, from baseline to 6 months, are found in the AC activity measures of cells and flare. These measures correlate with the presence of pre-existing structural complications and this has implications for the reporting of trials using a single measure as a primary outcome. […] We propose distinctive and clinically significant measures of disease activity, severity and damage for JIAU. […] The definition of a response and damage index as well as the definition of uveitis remission and inactive disease was part of the second part of the meeting. […] We have also proposed definitions for treatment efficacy; remission, inactive disease and damage for JIAU.
#26 Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (
https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-019-0345-2
This study reports multiple outcomes of a representative population of patients with JIAU in a 6-month period following clinically-driven initiation of new DMARD treatment. […] Our finding that the primary measures of activity are mediated by coexisting damage confirm the clinical feeling that drug efficacy is unlikely to be the same in all eyes and therefore stratification by disease damage is likely to be necessary to obtain the best measure of drug efficacy. […] We have demonstrated multiple measures that demonstrate sensitivity to change over a clinically relevant period of time. […] This work is the initial step for developing a comprehensive assessment of outcomes of children with JIAU which incorporates the perspectives of rheumatologists, ophthalmologists, patients and families.
#27 Predictors for functional and anatomic outcomes in macular edema secondary to non-infectious uveitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210799
We aimed to investigate predictive factors for visual and anatomic outcomes in patients with macular edema secondary to non-infectious uveitis. […] Favorable prognosis, as defined above, was observed in 18 (32.1%) eyes. UME prognosis was negatively correlated with baseline foveal thickening, alteration in the vitreo-macular interface and cystoid macular edema. […] Available treatment modalities in UME may avoid chronic UME and improve anatomic outcome. However, the proportion of functional amelioration observed during 12 months of follow-up is lower. Thicker CST, alteration in the vitreo-macular interface and cystoid macular edema may denote less favorable prognosis. Conversely, bilaterally, systemic disease and anterior chamber cells may be associated with favorable prognosis in UME.
#28 Predictors for functional and anatomic outcomes in macular edema secondary to non-infectious uveitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210799
We aimed to investigate predictive factors for visual and anatomic outcomes in patients with macular edema secondary to non-infectious uveitis. […] Favorable prognosis, as defined above, was observed in 18 (32.1%) eyes. UME prognosis was negatively correlated with baseline foveal thickening, alteration in the vitreo-macular interface and cystoid macular edema. […] Available treatment modalities in UME may avoid chronic UME and improve anatomic outcome. However, the proportion of functional amelioration observed during 12 months of follow-up is lower. Thicker CST, alteration in the vitreo-macular interface and cystoid macular edema may denote less favorable prognosis. Conversely, bilaterally, systemic disease and anterior chamber cells may be associated with favorable prognosis in UME.
#29 Long-term visual acuity outcome of pediatric uveitis patients presenting with severe visual impairment | Scientific Reports
https://www.nature.com/articles/s41598-023-29159-x
This study investigated the long-term visual acuity (VA) outcome in the eyes of children with uveitis and severe visual impairment (SVI; VA20/200) at presentation. […] The long-term VA outcome in uveitis-affected eyes with SVI at presentation was unfavorable. Younger age at uveitis onset, delayed presentation, and uveitis involving the posterior segment were associated with poorer VA outcome. […] A more favorable VA outcome was observed in eyes with uveitis onset at school age or older, anterior uveitis, and a shorter duration of ocular symptoms before uveitis diagnosis. […] The long-term course of VA outcome in this study demonstrated that, on average, eyes with both infectious and non-infectious uveitis experienced VA improvement shortly after receiving uveitis treatment. […] Although these improvements were statistically significant compared with the baseline VA, the final VA remained within the SVI level. […] The three most common causes of poor vision at final follow-up were RD, atrophic bulbi, and optic atrophy.
#30 Uveitis – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK540993/
In most cases, the prognosis of uveitis is good assuming early detection and proper treatment. While a majority of patients will develop an ocular complication, appropriate treatment and surgery, if needed, make permanent vision loss much less likely. […] Identifying the underlying cause of uveitis is also important due to significant morbidity and mortality associated with some specific systemic diseases that can cause uveitis.
#31 Uveitis: Symptoms, Causes, Treatment & Types
https://my.clevelandclinic.org/health/diseases/14414-uveitis
Uveitis is a condition where you have inflammation of the uvea, which includes the iris, ciliary body and choroid. Its common, and vision loss is one of the possible complications (especially when uveitis is severe or goes untreated). […] In severe cases, uveitis (pronounced yoo-vee-EYE-tiss) can cause permanent vision loss and blindness. […] Uveitis is a common cause of vision loss and legal blindness. In the U.S., 10% of legal blindness cases happen because of, or in connection with, uveitis. Negative outcomes are more likely when there are delays in diagnosis and treatment. […] The odds of long-term vision issues are much lower with early diagnosis and treatment. When those happen, youre more likely to recover fully and avoid permanent vision issues.
#32 Visual Acuity Outcome over Time in Non-Infectious Uveitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC7282970/
Mean VA of uveitic eyes improved and, typically, improvement was sustained under uveitis subspecialty care. […] The results imply that progressive visual loss occurred prior to referral (36% to 45% had care for 2 years prior to referral), suggesting that uveitis cases may benefit from early referral to uveitis subspecialists when equivalent care is not available in another setting. […] The observation that the average uveitis case under tertiary uveitis care improves suggests that time-to-worsening approaches to studying visual acuity as clinical research outcomes have important limitations in studying treatments of uveitis.
#33 Intermediate Uveitis – EyeWiki
https://eyewiki.org/Intermediate_Uveitis
The natural history of IU is variable. Although generally considered a benign disease, many cases show a prolonged course with exacerbations, and only 10% of patients may have a self-limited course of disease. In addition, many patients develop complications that may lead to visual loss. Adequate control of inflammation and prompt detection and treatment of associated complications are thus essential in improving the overall prognosis of the patient. […] Overall, the visual prognosis of pars planitis in children is reported to be favorable if adequate treatment is implemented early in the disease course. In a study conducted by Donaldson, et. al., of 24 patient eyes, 75% maintained a visual acuity of 20/40 or better after 10 years of follow-up. […] Severe visual loss is uncommon, but there is a high incidence of ocular complications, such as optic disc edema leading to optic atrophy and macular edema, which are associated with visual impairment. Long-term follow-up suggests that although optic disc edema may indicate a risk for macular edema, it is noted that not all patients with optic disc edema develop substantial visual loss. […] Factors associated with better outcomes in pars planitis include older age of disease onset and prompt initiation of systemic steroid-sparing IMT. Ongoing monitoring is advised to assess for uveitic and steroid-induced complications such as cataracts or glaucoma.
#34 Prognosis of Uveitis
https://www.clinicbarcelona.org/en/assistance/diseases/uveitis/disease-evolution
Uveitis is neither a hereditary or degenerative disease and so it is not progressive. It certainly has an impact on patient quality of life, especially in the case of long-term treatments, but in principle it is a disease that can be controlled and patients with uveitis will not go blind. […] Not only is diagnosis made increasingly sooner, the disease is also dealt with using a much more multidisciplinary approach. In addition, there are new medicines available for dealing with immune-mediated uveitis that now act more quickly and therefore offer a better visual prognosis.
#35 Uveitis: Symptoms, Causes, Treatment & Types
https://my.clevelandclinic.org/health/diseases/14414-uveitis
Uveitis is a condition where you have inflammation of the uvea, which includes the iris, ciliary body and choroid. Its common, and vision loss is one of the possible complications (especially when uveitis is severe or goes untreated). […] In severe cases, uveitis (pronounced yoo-vee-EYE-tiss) can cause permanent vision loss and blindness. […] Uveitis is a common cause of vision loss and legal blindness. In the U.S., 10% of legal blindness cases happen because of, or in connection with, uveitis. Negative outcomes are more likely when there are delays in diagnosis and treatment. […] The odds of long-term vision issues are much lower with early diagnosis and treatment. When those happen, youre more likely to recover fully and avoid permanent vision issues.