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Fuchs’ Uveitis and
Posner-Schlossman Syndrome
A. Fuch’s Uveitis
Carl P Herbort, Nadia Bouchenaki
INTRODUCTION AND HISTORICAL ASPECTS
EPIDEMIOLOGICAL ASPECTS
In 1906 Ernst Fuchs described a condition including
heterochromia, unilateral inflammatory signs such as
fine “microgranulomatous” keratic precipitates (KPs)
as well as unilateral degenerative signs including iris
atrophy and vitreous strands. He called the disease
heterochromic cyclitis, more precisely his words were
“cyclitis associated with heterochromia”, which would
later be known by the eponym of Fuchs’ heterochromic
cyclitis or iridocyclitis1 (Figures 1A and B).
In the 15th edition of Fuchs’ ophthalmology
textbook the full clinical picture is described including
the very prominent vitreous involvement and
infiltration2 (Figure 2).
Because the disease was described in Vienna, in a
Caucasian population, the most striking clinical sign,
heterochromia, was included in the eponym of the
disease called until today Fuchs’ heterochromic
cyclitis. It is thought that in the past the disease was
probably underdiagnosed in areas where irises are
nearly exclusively brown and heterochromia is not
part of the disease picture. The disease should therefore simply be named “Fuchs’ uveitis”. The disease
pattern with onset mostly from the second to the fifth
decade, points towards an external, probably infectious
trigger that is at the origin of the subsequently autoentertained disease in persons that are probably
genetically predisposed.3
The disease seems to be occuring in most parts of the
world with similar frequencies. In European reports,
the percentage of cases in uveitis series is going from
2.2% in Portugal,4 5.0% in the Netherlands,5 5.4% in
Switzerland6 to even 8.32% in Italy. 7 Around the
world, the proportion of Fuchs’uveitis (FU) in uveitis
patient series is 6.6% in Iran,8 3.5% in Saudi Arabia,9
2% in Japan,10 5.7% in a recent study performed in
southern China 11 and 4.2% in African American
patients.12 As can be concluded from this sample of
epidemiological data, the disease seems to be
ubiquitous and seems nowadays to be diagnosed with
equal performance in brown iris population countries
as shown by the recent chinese series.11
CLINICAL SYMPTOMS AND SIGNS
In contradiction to what is written in many textbooks
and articles, Fuchs’ uveitis is a granulomatous disease.
It has several clinical features such as Koeppe nodules
and granulomatous keratic precipitates (KPs) that
make up the definition of granulomatous uveitis. It
should be remembered that the term granulomatous
is a misnomer as terminology coming from pathology
is used to describe a clinical entity or group of diseases.
This term however serves very well clinical purposes
as the definition of granulomatous uveitis is very
precise and useful, including KPs that are more than
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Imaging in Uveitis
Figur 1B: Title page of 15th edition of
Fuchs’ ophthalmology textbook
Figures 1A and B: View of of the 15th edition of Fuchs’ textbook
on ophthalmology (1926) where the exact description of
“hetrochromia and cyclitis” is given (A) and view of 1st page of
textbook (B)
Figure 1A: 15th edition of Ernst
Fuchs’ ophthalmology textbook
just dust on the endothelium, Koeppe nodules at the
pupillary margin and Busacca nodules on or within
the iris. It is worthwile to clearly specify here that as
soon as KPs can be individually identified, meaning
that they have already an “architecture”, they should
be termed as granulomatous, which is not always
clearly stated in textbooks. This is at the origin of the
erroneous misclassification of Fuchs’ uveitis as a non
granulomatous uveitis. In Fuchs’ uveitis the granulo-
Figure 2: Original text on Cyclitis associated with heterochromia
in the 15th edition of Ernst Fuchs’ ophthalmology textbook
matous KPs have usually a quite specific aspect with
fluffy edges taking sometimes a stellate appearance
with fine spicules extending from the body of the KP
Fuchs’ Uveitis and Posner-Schlossman Syndrome
Figure 3: Iris atrophy. This patient with Fuch’s uveitis had no
frank heterochromia. However the iris is clearly atrophic. Note
sparsly distributed KPs visible on the cornea in front of the whole
pupillary area
with a diffuse distribution on the endothelial surface
(Figure 3).
SYMPTOMS
Most often floaters as well as blurred vision and visual
deterioration is pushing patients to consult. Very often
however the unilateral inflammation is a chance
discovery during a routine ophthalmological examination. If anterior involvement is not prominent it can
be missed and the attention of the ophthalmologist is
attracted by the more obvious vitreal involvement
often causing the diagnosis to be missed in favour of
intermediate uveitis or other diagnoses such as the non
specific diagnosis of “granulomatous uveitis”, “posterior uveitis” or “panuveitis”.
SIGNS (TABLE 1)
The signs that have mostly been put forward in the
literature is heterochromia because it is the most
spectacular sign as well as stellate granulomatous KPs
that are also very characteristic.This is at the origin of
the idea in the practitioner’s mind that Fuch’s uveitis
is a purely anterior uveitis. If series coming from
European countries are analysed, heterochromia is
reported in 82% in the Netherlands,13 in 87% in a
European study,14 in 80% in a North-American study15
and 70% in a United Kingdom study.16 When analysing studies coming from predominantly brown eyed
population countries the proportion of heterochromia
is by far lower and goes down to 14% in China17 and
to 23% in Japan.10 Instead of focussing on heterochromia, the iris signs to search for are iris atrophic
changes with or without heterochromia. In some series
atrophic iris changes are present in close to 100% of
cases (Figure 3).
The proportion of patients for whom characteristic
KPs were described do not differ among Caucasian
and brown eyed populations representing respectively 88% (Netherlands),13 96% (Belgium),14 81%
(UK)16 and 96% in a North American study15 which is
not sensibly different for Asian series where the
percentage is respectively 91.5% for China17 and 97%
for Japan10 (Figures 4A-C). See proportion of signs in
table one.18-20
Another set of signs is seen less consistently as the
two previous signs but is very helpful to make the
diagnosis more certain. This includes posterior polar
subcapsular cataract (Figure 5), iris (Koeppe > Bussaca)
nodules (Figure 6) and abnormal vessels in the iridocorneal angle. The latter sign is not always reported
in Fuchs’ uveitis series. These abnormal angle vessels
are also at the origin of the classically reported
“Amsler’s sign” consisting of occurence of a hyphema
when an anterior chamber puncture or tap is
performed. Similarly these vessels are also at the origin
of hyphema during cataract surgery.
One of the most neglected signs is vitreous infiltration which is even not reported in some series,
although it is one of the signs described originally by
Fuchs (Figure 7).
The degree of vitreous involvement may vary from
the presence of a few cells to an infiltration with dense
vitreous fibrous strands and condensations which may
explain an erroneous diagnosis such as intermediate
or posterior uveitis. In rare cases the heavily infiltrated
vitreous may be at the origin of peripheral retinal tears
and retinal detachment.
Finally absence of some clinical signs are almost
diagnostic and may be excluding signs if present but
are rarely reported. This is the case of “absence of
cystoid macular oedema” (CMO) and “absence of
posterior synechiae” on the crystalline lens which are
present if reported in a proportion of over 95%. In the
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88
FHC = Fuchs’ Heterochromic Cyclitis
FHU = Fuchs’ Heterochromic Uveitis
N/D = not done
100%
Absence of synechiae
Bilaterality
100%
12%
Iris nodules
Absence of CMO
13%
13%
Abn. angle vessels
9%
Glaucoma / Hypert.
Catrarct
55%
43%
Heterochromia
N/D
85%
Fuchs KPs
Iris changes (atrophy)
98%
23
Fuchs’ uveitis
Bouchenaki
2007
Switzerland
Vitritis
Reference
Name given to uveitis
Nbr of Cases
Name 1st author
Year
Country
N/D
FHC
La Hey
100%
98%
4%
10%
22%
82%
100%
82%
88%
84%
13
51
1991
Netherlands
1984
N/D
N/D
N/D
N/D
N/D
FHC
8%
19%
84%
82%
96%
69%
14
550
Dernouchamps
Belgium
N/D
FHC
73%
94%
70%
81%
84%
16
77
1995
98.70%
15.80%
32%
21.30%
Fearnley
U.K. (1)
N/D
N/D
FHU
Jones
U.K. (2)
19
103
1991
100%
7.80%
20%
26%
80.10%
100%
90.30%
83.80%
66.60%
Table 1
N/D
N/D
N/D
FHC
Velilla
Spain
4%
7.40%
14.80%
77.80%
14.80%
70.40%
100%
14.80%
20
26
2001
54
1982
N/D
N/D
N/D
syndrome
0%
70%
59%
90%
98%
80%
96%
55%
15
Fuchs’ uveitis
Liesegang
USA
N/D
N/D
N/D
N/D
FHC
Silva
Brasil
94%
21%
19%
70%
18%
34%
99%
18
132
1989
FHC
Higuchi
Japan
81%
98%
0%
83%
33%
12.10%
93%
100%
23%
98%
88%
10
43
1982
104
2006
N/D
N/D
N /D
rome
13.50%
28%
23%
70.70%
100%
14%
92%
73.8
17
Fuchs’ synd-
Yang
China
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Imaging in Uveitis
Fuchs’ Uveitis and Posner-Schlossman Syndrome
Figure 4A: Microgranulomatous Fuchs
KPs that do not cluster inferiorly
Figure 5: Fuchs KPs and posterior polar cataract. Picture
showing two planes; towards the right typical Fuchs KPs;
towards the left of the picture white posterior subcapsular
cataract
Figure 4B: Macroscopic view of Fuchs KPs showing spicules
or extentions from the “body” of the KP
Figure 6: Koeppe nodules. Fuchs uveitis in a brown eye. Two
Koeppe nodules on the iris border (yellow arrows). Note also
atrophic changes in the iris with loss of intensity of brown colour
Figure 4C: Macroscopic view of Fuchs KP showing fluffy
borders and extensions (yellow arrows)
Figures 4A-C: Fuchs’ typical microgranulomatous KPs with
diffuse spread-out disposition on whole endothelium (A) and
macroscopic view (B and C)
absence of the characteristic signs of heterochromia
and Fuchs’ KPs and the presence of vitritis these signs
have almost determining diagnostic value. However
both these signs are reliable only in patients that have
never undergone intraocular surgery. Fuchs’ uveitis
patients having had intraocular surgery are no more
protected against CMO nor irido-lenticular synechiae.
The proportion of any given sign in Fuchs’ uveitis
series is shown on Table 1, indicating some diversity
in the proportion of reported signs from one study to
another.
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It is however in every day practice that LFP has
proved especially useful for Fuchs’ uveitis. In those
Fuchs cases that have been misdiagnosed and are
under topical and sometimes even systemic
corticosteroid therapy, LFP allowed to show that, after
discontinuation of therapy, there is no rebound flare
increase or minimal flare increase indicating to the
patient and to the referring doctor that it is safe to
withdraw potentially harmful therapy that has no
impact on inflammation in this disease.
ANGIOGRAPHIC FINDINGS
Figure 7: Vitritis, the misleading sign. This young lady
consulted her ophthalmologist because of a suboptimal vision.
The presence of a dense vitritis rendering the fundus image
blurred was interpreted by the ophthalmologist as an
intermediate uveitis. He introduced systemic steroids followed
6 months later by cyclosporin that still had no effect. After seeing
the patient for a second opinion, inflammation suppressive
therapy was progressively withdrawn under laser flare
photometry monitoring without rebound inflammation and stable
evolution
LASER FLARE PHOTOMETRY
Laser flare photometry (LFP) is measuring back
scattered photons from anterior chamber proteins
when a laser light beam is shone into the anterior
chamber. Its principle is identical to slit-lamp anterior
chamber examination except that in LFP the light
source is monochromatic and quantified rather than
polychromatic. Also the light detector is objective
made of a photomultiplyer and photodetector rather
than the subjective human eye. 21 This method
therefore allows to measure objectively the disruption
of the blood-aqueous barrier and hence the exact level
of intraocular inflammation.22 When we measured
flare in our Fuchs’ uveitis patients at presentation the
mean flare value was 8.62 ± 4.3 photons per millisecond (ph/ms) which is only slightly above normal
values (4-6 ph/ms). This showed that in most Fuchs’
cases there is minimal disruption of the blood-aqueous
barrier, hence minimal anterior chamber inflammation.23 Moreover, at the end of follow-up this value
remained stable at 8.45 ± 4.3 ph/ms in our patients
and did not increase, showing that inflammation does
not progress in the absence of treatment. This has
recently been confirmed by a chinese study reporting
similar values.24
In case of a clinically sure diagnosis of Fuchs’ uveitis
there is no need to perform fluorescein angiography.
Very often however diagnosis is missed initially and
fluorescein angiography happens to be performed. We
analysed a series of 23 patients that had a fluorescein
angiography performed for diagnostic purposes.23
In all but one of these patients was there a slight to
moderate and sometimes pronounced disc hyperfluorescence (Figure 8). The only patient who had no disc
hyperfluorescence was a case that was known to have
disc atrophy previous to the diagnosis of Fuchs’
uveitis. In 4 patients slight mid-peripheral retinal
vascular leakage was seen. No cystoid macular
oedema could be seen in any of the 21 patients that
had never had intraocular surgery even after very
longstanding inflammation. Cystoid macular oedema
was present in two patients both of whom had
undergone cataract surgery.
Being aware of these results it might be helpful and
justified to use fluorescein angiography as a diagnostic
procedure in some cases where Fuchs’ uveitis is the
presumed diagnosis but where some of the principal
signs are missing. In such cases with longstanding
uveitis with prominent vitreous infiltration, the
absence of an angiographic CMO together with disc
hyperfluorescence can be considered confirmatory of
Fuchs’ uveitis.
DIFFERENTIAL DIAGNOSIS, EVOLUTION,
THERAPY AND PROGNOSIS
In the paragraphs on differential diagnoses given for
Fuchs’ uveitis, textbooks usually only cite other
anterior uveitis entities such as herpetic uveitis, zoster
uveitis, Posner Schlossman syndrome, etc. Only one
Fuchs’ Uveitis and Posner-Schlossman Syndrome
Figure 8: Disc hyperfluorescence on the side of Fuchs’ uveitis (lef eye on the right side of the picture)
textbook cited intermediate uveitis as a possible
differential diagnosis.25 This shows how strong the
idea is, even among textbook authors, that Fuchs’
uveitis is a purely anterior uveitis, making abstraction
of the vitreous involvement. In real ophthalmologic
practice the situation is quite different. In our series
in which 84% of patients werw referred, the list of
erroneous diagnoses, hence diagnoses that should be
listed in the differential diagnosis for Fuchs’uveitis,
was headed by intermediate uveitis in 37/59 (63%) of
non diagnosed cases followed by panuveitis in 11/59
(19%), posterior uveitis in 6/59 (10%) patients and
granulomatous uveitis in 5/59 (8%). The nature of
these diagnoses indicates that the clinician tends to rule
out Fuchs’ uveitis in the presence of inflammatory
vitreous involvement.
Evolution of Fuchs’ uveitis is mostly benign as the
sensitive structures such as the macula is nearly never
involved despite substantial and prolonged vitritis.
Therefore no therapy is necessary. Nevertheless
regular follow-up examinations are necessary in order
to detect complications such as intraocular hypertension or glaucoma as well as cataract which may be
part of the natural course of the disease but which are
often favoured by the abusive and/or long term use
of steroids. Prognosis is rather good because even if
cataract is present, it is operated with success because
these eyes tolerate surgery very well as was noted in
Fuchs’ original descriptions. Among the 10 to 20 % of
patients that develop glaucoma, some are difficult to
stabilise despite surgery and prognosis is reserved only
in this portion of Fuchs patients.
Despite the fact that about 15-20% of Fuchs’ eyes
develop hypertension, the mean pressure of the Fuchs
eye is lower than the fellow eye (12.04 ± 4 versus 12.87
± 4.2 mmHg), indicating that, as a rule, the affected
eye has a lower pressure than the normal eye.
Although corticosteroid therapy is not supposed to
be used because of absence of impact, there are rare
situations where inflammatory bouts occur that might
benefit from short courses of topical steroids.
WHY IS FUCHS’ UVEITIS SO OFTEN
MISDIAGNOSED OR DIAGNOSED WITH
SUBSTANTIAL DELAY? / DIAGNOSTIC
CRITERIA OF FUCHS’ UVEITIS
The reasons why Fuchs’ uveitis is so often misdiagnosed or underdiagnosed are several, including
bilaterality usually not associated with the disease but
which occurs in about 10%, search for heterochromia
which is often absent especially in brown eyed
countries and absence of the characteristic KPs. The
main reason however, as stated earlier, is the presence
of inflammatory vitreous involvement that, in the
mind of the practitioner, is usually not associated with
Fuchs’ uveitis when in reality it is a major diagnostic
sign. This leads to diagnostic delay which in our series
occured in 59/79 (75%) cases with a mean duration of
delay of 3.67± 4.34 years. Unawareness that vitritis is
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Imaging in Uveitis
part of Fuchs’ clinical picture is also at the origin of
inadequate, useless and potentially harmful therapy
such as systemic coricosteroids and/or even systemic
immunosuppressants that were given in 34/79 (43%)
patients in our study.
Therefore diagnostic criteria of Fuchs’ uveitis have
to be reconsidered. The most striking clinical signs
such as heterochromia and characteristic KPs are of
course very useful for the diagnosis when they are
present. However the diagnosis has to be made more
secure by considering also less spectacular signs albeit
highly consistent that put together allow to make the
diagnosis of the condition even without the more
obvious signs. A very high, almost 100% degree of
certainty is obtained when triad consisting of
heterochromia, typical KPs and vitritis is present. The
neglected or more discrete signs that are recorded with
a proportion of close to 100% in Fuchs’ series are, when
going antero-posteriorly (1) atrophic iris changes,
(2) absence of posterior irido-crystalline synechiae,
(3) vitritis or vitreous strands and (4) absence of cystoid
macular edema. The occurence of the combination of
these signs probably allows to reach an almost
identical degree of certainty. The association of “ minor
signs“ such as (1) iris nodules, (2) cataract and
(3) stromal pseudo-neovessels apparent because of iris
atrophy or abnormal iridocorneal angle vessels with
or without Amsler’s sign (hyphema following anterior
paracentesis) add to the degree of certainty of the
diagnosis.
PATHOGENESIS OF FUCHS’ UVEITIS
AND CONCLUSION
Fuchs’ uveitis typically has the profile of a disease
caused by an infectious triggering factor (probably
more than one) at the origin of an immune autoentertained disease in genetically susceptible patients.
Most cases are diagnosed between the 2nd and the 5th
decade, the period during which individuals are
exposed to most of the infectious agents encounter
throughout life including the agent(s) that can trigger
Fuchs’uveitis.13,15,19 Above the age of sixty the disease
is less frequently diagnosed as the probability to
encounter the infectious trigger is much less. HLA-B27
related acute anterior uveitis has an identical
epidemiological profile. For this disease the triggering
agents are known to be debris from gram-negative
bacteria and the genetical predisposition is the
presence of the HLA-B27 antigen.
The evidence accumulated so far indicating
immune dysfunction in Fuchs’ patients and Fuchs’
eyes is sufficient to allow us to consider immune
mechanisms as the common effector pathway causing
lesions in Fuchs’ uveitis. Very early, when immunological investigations were still very rough, increased
IgG in aqueous humour was found.26 This was later
confirmed by PI Murray et al who also found increased
interleukin-6 in the aqueous humour.27,28 Other studies
showed the presence of autoimmunity to corneal
antigens, a possible explanation of the spread-out
disposition of Fuchs KPs.29 Immunohistochemical
analysis of iris biopsy specimens from patients with
Fuchs’ heterochromic cyclitis showed evidence of an
inflammatory cell infiltrate, which was a mixture of
interleukin-2 receptor-negative T helper and suppressor cells, B lymphocytes, and plasma cells.30 More
recently Labalette and colleagues showed the presence
of CD8-positive CD28-negative T cell clonotypes
indicating an antigen-driven process involving these
cells at the origin of Fuchs’ uveitis.31 In addition a
cellular autoimmune response to S antigen was found
in patients with Fuchs’ uveitis, a possible explanation
of posterior segment involvement,32 which seems to
have received clinical support by a study that showed
subclinical retinal damage using electroretinography.33
As far as the infectious trigger in Fuchs’ uveitis is
concerned, several agents have been put forward. For
some time toxoplasma Gondii had the favours.34,35
only to be seriously put in doubt some years later.36
Recently the detection of an intraocular synthesis of
anti-rubella antibodies in all tested patients as well as
the rubella genoma itself in 20% of eyes, represented
a very strong argument for Fuchs’ uveitis being a
rubella virus driven uveitis.37 Similar results were also
found in another group.38 Although there are presently
quite convincing arguments for rubella to be a trigger
for Fuchs’ uveitis, other possible triggers might also
play a role.
In conclusion, Fuchs’ uveitis is an ubiquitous
disease involving the vitreous and the anterior uvea.
The inflammatory nature does make no doubt and the
presently favoured pathogenetic mechanism is an
immune process triggered by an infectious agent
thought to be in many cases rubella virus, other agents
Fuchs’ Uveitis and Posner-Schlossman Syndrome
not being completely excluded. The disease is often
misdiagnosed because of the textbook generated
unawareness of the quasi 100% rate of association of
vitritis with the disease. Anti-inflammatory treatment
has mostly no or minimal impact but evolution is
favourable unless complications such as glaucoma or
cataract occur.
KEY POINTS
• Fuchs’ uveitis is a mostly unilateral uveitis involving the
anterior segment and the vitreous body.
• It is usually diagnosed between the second and fifth
decade, a disease pattern indicating the association of
an external trigger associated with susceptibility to the
disease.
• Moderate decrease of visual function and floaters are
the main symptoms but very often the disease is
diagnosed by chance during a routine visit.
• The main signs of Fuchs’uveitis with a proportion of
presence between 90-100% are (1)characteristic Fuchs’
KPs, (2) iris atrophic changes, (3) vitritis, (4)absence of
synechiae, (5) absence of CMO, (6) unilaterality
• Complications include cataract formation (> 60%,
depending on lenght of follow-up) and glaucoma
(12-20%)
• No treatment is necessary for the primary disease.
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Invest Ophthalmol Vis Sci 1990;31:917-20.
29. van der Gaag R, Broersma L, Rothova A, Baarsma S,
Kijlstra A. Immunity to a corneal antigen in Fuchs’ heterochromic cyclitis patients. Invest Ophthalmol Vis Sci 1989;
30:443-8.
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30. Murray PI, Mooy CM, Visser-deJong E, Baarsma GS, de
Vries J, de Jong PTVM, et al. Immunohistochemical
analysis of iris biopsy specimens from patients with Fuchs’
heterochromic cyclitis. Am J Ophthalmol 1990 ; 109 :3949.
31. Labalette P, Caillau D, Grutzmacher C, Dessaint JP,
Labalette M. Highly focussed clonal composition of
CD8(+)CD28(neg) T cells in aqueous humnour of Fuchs’
heterochromic cyclitis. Exp Eye Res 2002;75:317-25.
32. La Hey E, Broersma L, van der Gaag R, Baarsma GS,
Rothova A, Kijlstra A. Does S Antigen play a role in Fuchs’
heterochromic cyclitis ? Br J Ophthalmol 1993;77:436-9.
33. Murray DC, Stavrou P, Good PA, Murray PI.
Electroretinographic findings in Fuchs’ heterochromic
Cyclitis. Eye 1997;11:102-8.
34. De Abreu MI, Belfort R, Hirata PS. Fuchs’ heterochromic
cyclitisand ocular toxoplasmosis. Am J Ophthamol 1982;
93:739-44.
35. Saraux H, Laroche L, Le Hoang P. Secondary Fuchs’
heterochromic cyclitis: a new approach to an old disease.
Ophthalmologica 1985;190:193-8.
36. La Hey E, Rothova A, Baarsama GS, de Vries J, van
Knapen F, Kijlstra A. Fuchs’ hetrochromic cyclitis is not
associated with ocular toxoplasmosis. Arch Ophthalmol
1992;110:806-11.
37. Quentin CD, Reiber H. Fuchs’ heterochromic cyclitis :
rubella virus antibodies and genoma in aqueous humour.
Am J Ophthalmol 2004;138:46-54.
38. de Groot-Mijnes JD, de Visser L, Rothova A, Schuller M,
van Loon AM, Weersink AJ. Rubella virus associated with
Fuchs’ heterochromic iridocyclitis.
B. Posner-Schlossman Syndrome
Bechir Jelliti, Moncef Khairallah
INTRODUCTION
Posner Schlossman syndrome (PSS) as originally
described in 1948 by Posner and Schlossman is a selflimiting and benign condition characterized by
unilateral, recurrent attacks of mild, granulomatous
iritis with elevated intraocular pressure (IOP) during
the acute attack, open angle, normal visual field and
optic disc.1
It is classified as an inflammatory glaucoma
because it is by definition always accompanied by
uveitis.1 The inflammation may follow the acute rise
in intraocular pressure (IOP) by some days or be so
mild as to be overlooked, so the etiology of the rise in
pressure may not be clear at first presentation.
EPIDEMIOLOGY AND PATHOGENESIS
PSS tends to affect patients between 20 and 50 years
of age. Males are more often affected. There is no
reported ethnic or racial predilection. Recent reports
suggest an association with the human leukocyte
antigen (HLA) Bw54.2,3 Recurrence is common and can
be in either eye but intervals between attacks tend to
increase during the course of the disease.4
The etiology of PSS is uncertain and no recognized
associated systemic conditions have been reported. The
mechanism responsible for glaucomatocyclitic crisis is
unclear. Cytomegalovirus (CMV), varicella-zoster
virus and herpes simplex virus (HSV) may be linked
to the disease.5 The trabecular meshwork is innervated
by the trigeminal nerve and is a conduit for HSV. One
postulated mechanism for the syndrome is that HSV
causes an inflammation of the trabecular meshwork,
impeding aqueous outflow and increasing IOP.
The allergic response is also under investigation.
High levels of prostaglandin E have been found in the
aqueous humor during attacks. These levels have been
observed to return to normal during remissions.2,6
CLINICAL FEATURES
Typically, patients describe a history of intermittent
episodes of mild visual blurring, colored haloes around
lights, and minimal discomfort in one eye. Some
patients have no symptoms.
The hallmark feature of PSS is that the IOP (often
45 mmHg or higher) does not correspond to the
amount of ocular inflammation. The affected eye
typically appears white and quiet on gross inspection,
but a small amount of inflammation can usually be
detected. The anterior chamber is deep. A few, small,
round non-pigmented keratic precipitates (KPs) tend
to accumulate on the lower one third of the corneal
endothelium within three days of onset (Figure 1).
Fuchs’ Uveitis and Posner-Schlossman Syndrome
associated with elevated IOPs, diffuse epithelial edema
of the cornea, and a few fine keratic precipitates . The
IOP is normal between attacks and the angles are
open.10
DIFFERENTIAL DIAGNOSIS
Figure 1: Isolated KPs towards (arrows) and in the iridocorneal angle (not shown) in a case of PSS
A common finding is that the pupil is slightly dilated
in the affected eye compared to the unaffected eye.
Gonioscopy reveals normal, open angles without
abnormal pigmentation. 7 Posterior and anterior
synechiae do not develop in this condition. Signs may
be present for a month or longer without producing
substantial symptomatology.
PSS is a self-limiting condition that lasts a few
weeks to a month. During the acute and remission
phases of PSS, anterior chamber angles are typically
open with normal pigmentary density and no
associated synechiae. Although attacks are self limited,
glaucomatous cupping and field loss may occur.
Recurrent attacks characterize the illness; however
the frequency of such attacks is highly variable
between patients. Usually only one eye is affected;
rarely however, a patient may experience subsequent
episodes in either eye.8
Posner-Schlossman syndrome does not always
follow a completely uncomplicated course.
Repeated episodes of elevated intraocular pressure
can cause long-term sequelae such as glaucoma. In a
study of 53 cases of PSS, 14 (26.4%) were found to have
glaucomatous optic nerve damage.9
DIAGNOSIS
There are no specific laboratory tests for this entity and
a presumptive diagnosis of PSS was made based on
the following findings: recurrent episodes of mild iritis
When diagnosing PSS, we must rule out other conditions that could cause unilateral or bilateral uveitis.
These include Fuchs’ uveitis, acute angle-closure
glaucoma, pigmentary glaucoma, herpes simplex virus
infection, uveitic glaucoma, neovascular glaucoma,
and secondary angle-closure glaucoma due to
peripheral anterior synechiae.11
History, biomicroscopy, tonometry and dilated
fundoscopy are essential for the differential diagnosis.
Past ocular, systemic, social and family histories are
necessary to rule out suspected underlying etiologies.
TREATMENT
The treatment for PSS focuses on lowering IOP and
reducing inflammation. Therapy may include topical
beta blockers, topical alpha agonists topical/oral
carbonic anhydrase inhibitors and even hyperosmotic
agents.2 Myotic agents are contra-indicated for the
treatment of PSS and uveitic glaucoma because they
enhance the risk for the formation of posterior synechia, cause discomfort to the patient by aggravating
ciliary muscle spasm and increase inflammation in the
eye by amplifying the breakdown of the blood aqueous
barrier. Pain and discomfort may be addressed with
cycloplegic agents such as atropine 1%, homatropine
5%, or scopolamine 0.25%. Inflammation can be treated
with topical steroidal preparations, every two hours
depending on the severity of the episode. Prophylactic
anti-inflammatory therapy is somewhat controversial.
Most practitioners do not feel that it prevents
recurrences.2,5
KEY POINTS
• PSS is a condition characterized by unilateral attack of
mild, granulomatous iritis with elevated IOP during the
acute attack, white eye, open angle, normal visual field
and optic disc.
• The etiology of PSS is uncertain, but several viruses,
including CMV,VZV, and HSV have been suggested as
potential causes of the syndrome
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• Recurrent attacks characterize the illness. PSS is a selflimiting condition, but glaucomatous optic nerve damage
may occur as consequence of repeated episodes of
elevated IOP
• The treatment for acute attacks of PSS focuses on
lowering IOP and reducing inflammation. Prophylactic
anti-inflammatory therapy does not seem to prevent
recurrences.
REFERENCES
1. Moorthy RS, Mermoud A, Baerveldt G, Minckler DS, Lee
PP, Rao NA. Glaucoma associated with uveitis. Surv
Ophthalmol 1997;41:361-94.
2. Chandler PA, Grant WM. Glaucoma due to intraocular
inflammation. In: Glaucoma, Lea and Febinger,
Philadelphia, 1986;363-3775.
3. Hirose S, Ohno S, Matsuda H. HLA-Bw54 and glaucomatocyclitic crisis. Arch Ophthalmol 1985;103: 1837-9.
4. Kanski JJ. Clinical Ophthalmology. A Systematic
Approach, 5th ed. London: Butterworth Heinemann, 2003.
p 238.
5. Geurds EA, Gurwood AS. Anterior uveitis, IOP, signal
Posner Schlossman syndrome. Review of Optometry 1998;
135(3):114-9.
6. Kanski J, McAllister J, Salmon J. Inflammatory glaucomas.
In: Glaucoma, A Colour Manual of Diagnosis and
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7. Narang SK, Shah SJ. Glaucomatocyclitic crisis (PosnerSchlossman syndrome) case report. Indian J Ophthalmol
1972;20:25-7.
8. Sangha SS. Posner Schlossman syndrome. Ophthalmology
2002;109(3):409.
9. Jap A, Sivakumar M, Chee SP. Is Posner-Schlossman
syndrome benign? Ophthalmology 2001;108:913-8.
10. Chee SP, Bacsal K, Jap A, Se-Thoe SY, Cheng CL, Tan BH.
Clinical features of cytomegalovirus anterior uveitis in
immunocompetent patients. Am J Ophthalmol 2008;
145(5):769-71.
11. Goldberg I. Ocular Inflammatory and Steroid-Induced
Glaucoma. In: Yanoff M, Duker JS, (Eds): Ophthalmology.
2nd ed. St. Louis: Mosby, 2004;1211:1516-7.