Editorial: Fuchs’ uveitis: from Imperial Vienna to global appraisal
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Of course heterochromia, a very obvious clinical sign, had been described much earlier. Aristotle named it heteroglaucos. A famous heterochromic in antiquity was Alexander the Great, who rode a horse, “Bukephalos”, which was itself heterochromic.
Heterochromia was to Fuchs’ uveitis what poliosis was to Vogt–Koyanagi–Harada disease. It was the flashy sign that first attracted the attention of clinicians but later functioned as the tree which prevents the forest being seen.
Indeed the strong position of heterochromia in early-phase disease definitions in caucasian populations with bi-colored irises later hampered the proper recognition of the reality of the disease. In populations with brown irises, heterochromia is always absent and discrete structural iris changes have to be searched for instead. This led to underdiagnosis of the disease as clinicians were searching for heterochromia, wrongly considered to be a disease-defining feature, but which was not present in brown iris populations. This is a strong reason to change the eponym of the disease to “Fuchs’ uveitis” instead of Fuchs’ heterochromic cyclitis.
After the clear description of vitreous involvement by Fuchs, another drift occurred. The articles and textbooks gradually forgot the posterior segment involvement and Fuchs’ uveitis came to be known as an anterior uveitis, again probably because the very particular aspect of the typical Fuchs’ KPs attracted and monopolized the attention of clinicians. Vitreous features were pushed into the background and intermediate uveitis as a differential diagnosis was no longer cited. The consequence was that clinicians were surprised when they saw Fuchs’ patients with vitritis and this led to misdiagnosis and diagnostic delay . In a recent article it was clearly shown that the most frequent clinical feature present was inflammatory vitreous involvement, found in almost 100% of cases . Clearly vitritis has to be put back into the limelight to drastically reduce misdiagnosis and diagnostic delay. The other feature which has not sufficiently been put forward and should replace the quest for heterochromia in patients with brown irises is the fine analysis of structural changes of the iris in the Fuchs’ eye as compared to the other eye.
In this issue, an article analyzing fluorescein angiographic findings in Fuchs’ uveitis broadens our knowledge of the disease . The authors showed that in almost 100% of Fuchs’ patients where a fluorescein angiography had been undertaken for diagnostic purposes in non-diagnosed cases, disc hyperfluorescence was detected which added to the confusion of the clinician who was not aware of this feature. This study also showed that in 100% of these cases no cystoid macular edema (CME) was detected despite years of inflammation, as long as the eyes had never undergone surgery. In contrast, Fuchs’ eyes after (cataract) surgery were no longer protected and CME was detected in some of them. Such a finding represents an important additional feature to define Fuchs’ uveitis, although there may be a bias towards overemphasis of disc hyperfluorescence as possibly only more severe cases have had an angiography performed. The vitreous and disc involvement tends to show that the disease is not a cyclitis but a uveitis involving other structures. This is the second reason to abandon the term cyclitis and to turn to the eponym “Fuchs’uveitis” as indicated above.
Another interesting article on Fuchs’ uveitis coming from a “brown-eyed area”, Saudi Arabia, is included in this issue . It shows that heterochromia is very rare but that iris strucure changes in the Fuchs’ eye were present in close to 100% of cases. Other articles coming from brown-eyed areas tend to show that when readjusted criteria are used and heterochromia is downplayed as a diagnostic criterion, the rate of Fuchs’ uveitis is comparable in most parts of the world, reaching the considerable percentage of 5–10% of total uveitis cases [11, 12, 13, 14, 15].
Because Fuchs’ uveitis is ubiquitous and represents such a high proportion of uveitis cases all over the world, it is important to define more global diagnostic criteria in order to decrease the diagnostic problem that this disease still causes. Major features such as vitritis, typical stellate retrodescemetic KPs, iris structure changes, absence of CME and absence of synechiae should be considered.
Confirmatory signs such as heterochromia, presence of lens opacification, presence of hypertrophic vessels in the irido-corneal angle and presence of disc hyperfluorescence should also be listed. New guidelines regarding diagnostic criteria for Fuchs’ uveitis are certainly needed and should be generated in an international workshop on Fuchs’ uveitis, similar to what has been achieved for ocular sarcoidosis (IWOS) . In this way misdiagnosis and diagnostic delays, sometimes the cause of deleterious effects and unnecessary immunosuppressive therapy, can in future be avoided in an increasing number of cases .
Recently evidence has been reported that rubella virus particles have been identified by PCR in intra-ocular samples from Fuchs’ eyes, giving more credit to an infectious rather than a inflammatory/degenerative cause for Fuchs’ uveitis [17, 18]. These new elements are not contradictory, as an infectious trigger has long been thought to be at the origin of a subsequently auto-maintained inflammation in Fuchs’ uveitis, and they do not fundamentally change the appraisal of Fuchs’ uveitis.
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