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NR 4-6/2008
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Non-malignant
Uveitis Masquerade Syndromes
Zespół maskujący niezłośliwe
zapalenia błony naczyniowej
Agnieszka Kubicka-Trząska, Bożena
Romanowska-Dixon
Department of Ophthalmology, Clinic of Ophthalmology and Ocular
Oncology,
Jagiellonian University, Medical College, Kraków, Poland
Head: Ass. Prof. Bożena Romanowska-Dixon, MD, PhD |
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| Summary: |
The Uveitis Masquerade
Syndromes (UMS) are a group of ocular diseases that
mimic chronic intraocular inflammation. Many
non-malignant conditions may result in an appearance
mimicking an uveitis. The authors review most common
non-malignant conditions which may be considered
masquerades such as: retained intraocular foreign body,
rhegmatogenous retinal detachment, myopic degeneration,
pigment dispersion syndrome, ocular ischemic syndrome,
infectious intraocular inflammation, retinitis
pigmentosa, multiple sclerosis and drug and
post-vaccination reactions. |
| Słowa kluczowe: |
zespół maskujący,
przewlekłe zapalenie wewnątrzgałkowe. |
| Key words: |
masquerade syndrome,
chronic intraocular inflammation. |
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Masquerade syndromes are disorders that occur
with intraocular inflammation and are often misdiagnosed as a
chronic idiopathic uveitis. Reports of masquerade syndromes are
rare (1). The most common conditions that can masquerade as
idiopathic uveitis are malignancies which were discussed in our
paper “Malignant Uveitis Masquerade Syndromes” (submitted in
“Klinika Oczna”). Among non-malignant diseases infectious
endophthalmitis are the most common conditions mimicking as
idiopathic intraocular inflammation (2). Other noninfectious
masquerade disorders which can be misdiagnosed as uveitis
include: intraocular foreign body, rhegmatogenous retinal
detachment, pigment dispersion syndrome, ocular ischemic
syndrome, retinitis pigmentosa, multiple sclerosis, drug and
post-vaccination reactions. We discuss the common and different
clinical manifestations of each masquerade disorder and its
diagnosis.
Infectious intraocular inflammation
Differential diagnosis between infectious and non-infectious
intraocular inflammation is the great challenge for an
ophthalmologist. Unlike the other masquerade syndromes,
initiating treatment with steroids in this group of intraocular
inflammation can result in devastating consequences. It is one
of the masquerade syndromes that should not be overlooked in any
patient presenting with intraocular inflammation of unknown
etiology. There are two forms of endophthalmitis masquerading as
non-infectious inflammation: chronic postoperative
endophthalmitis and endogenous endophthalmitis. Chronic
postoperative endophthalmitis develops 4 weeks to years
postoperatively and typically follows uneventful cataract
extraction with a posterior chamber intraocular implant. It may
rarely be precipitated by YAG-laser capsulotomy which releases
the sequestrated organisms from the posterior capsule into the
vitreous (3). The incidence of postoperative endophthalmitis
following cataract surgery has been reported as being between
0.07% and 0.33% (4). The infection is caused most frequently by
Propionibacterium acnes and occasionally Staphylococcus
epidermidis. One of the signs considered to be a hallmark of the
inflammation is the presence of a white plaque, usually located
between the intraocular lens and the lens capsule (5). Diagnosis
relies on isolation of the causative organism (4,5).
Endogenous endophthalmitis is an uncommon entity (2-8% of all
forms of endophthalmitis) (6). The infection is caused by
haematogenous spread of bacteria or fungi from a site of
infection in the body or from contaminated intravenous catheters
or needles. Potential causes include septic arthritis, urinary
tract infection, endocarditis, liver abscess, infected skin
wounds. Often the primary site of infection is occult. The most
frequent pathogen is Bacillus cereus although a wide variety of
organisms have been implicated including Proprionibacterium
acnes and streptococci (4). The infecting organisms enter the
uveal or retinal circulations and lodge in capillaries where
they establish a septic focus. Subsequently they break through
into the aqueous and vitreous. Immune compromised patients are
particularly susceptible to rapid involvement of the vitreous.
Both eyes are involved in about 25% of cases. The presenting
symptoms and signs are similar to those of uveitis of autoimmune
causes. Classically, endogenous endophthalmitis from bacteria
presents more explosively than does fungal infection. Diagnosis
relies on isolation of the causative microbes. The vitreous
biopsy or the anterior chamber aspirate are required and in
selected cases the retinal biopsy may be necessary (4).
Intraocular foreign body
Retained intraocular foreign body (IOFB) can cause various
degree of intraocular inflammation. Persistent anterior or
posterior uveitis is one of the most common complications
associated with IOFBs. Therefore IOFB should always be
considered in the differential diagnosis of chronic uveitis,
specially when the uveitis is refractory to treatment. Depending
on the type of the IOFB there are three major pathophysiologic
mechanisms of the eye injury: mechanical, chemical or toxic and
inflammatory (2). Nonorganic and nonmagnetic substances (stone,
glass, porcelain, gold, silver), cause nonspecific inflammation
by mechanical irritation to the ocular tissues. The mechanical
effect is essentially exudative and fibroplastic in order to
isolate and encapsulate the foreign body. Metal materials (iron,
copper, lead, zinc), cause the chemical damage due to
electrolytic dissociation of the metal or reaction with tissue
fluids, usually by oxidation. Highly toxic metal ions such as
iron and cooper lead to severe damage of ocular tissues called
siderosis and chalcosis, respectively. Organic materials can
produce a considerable tissue-reaction of the foreign body
granulomatous type. The pathologic reaction presents with a
low-grade chronic inflammatory response of a fibroblastic and
proliferative nature (7). An accurate and detailed history is
essential for making the correct diagnosis. Complete and
thorough ophthalmic examination is always required. Special
tests including radiography, computed tomography (CT),
ultrasonography are helpful in the detection and localization of
suspected IOFB. Magnetic resonance imaging (MRI) can be useful
for detection organic materials, glass or plastic IOFBs, but MRI
is contraindicated for metallic IOFBs because of the risk of
their intraocular movement. ERG and EOG testing have been used
to assess the degree of ocular injury from metallosis (8).
Rhegmatogenous retinal detachment
Some degree of intraocular inflammation is associated with
rhegmatogenous retinal detachment (RRD) which may present as
anterior and/or posterior uveitis and sometimes as panuveitis
(9). Usually slight flare and cells in the anterior chamber and
vitreous are common in eyes with RRD. In longstanding RRD
intraocular inflammation may be severe with the presence of
posterior synechiae, debris in the vitreous, detachment of the
ciliary body and choroid with hypotony. All cases of persistent
ocular inflammation with relative hypotony and a substantial
number of vitreous cells should be viewed with a high index of
suspicion for a possible underlying RRD. Sometimes longstanding
RRD can also present as an anterior cellular reaction with
secondary elevation of intraocular pressure – Schwartz’ syndrome
(10). In these cases if the detachment is not detected, the
patient may also be incorrectly treated for uveitis or glaucoma.
Misdiagnosis can be avoided by a detailed ophthalmic history and
thorough ocular examination completed with ultrasonography.
Pigment dispersion syndrome
Pigment dispersion syndrome (PDS), typically affecting young
white men with myopia, is characterized by release of pigment
from the pigment epithelium of the iris or ciliary body in both
eyes with attendant deposition of pigment on intraocular
structures such as cornea, the trabecular meshwork, the iris,
the lens. The dispersion of particles into the anterior chamber
can mimic the presence of inflammatory cells. Pigment particles
are often seen floating in the anterior chamber especially
following pupillary dilation and they may be mistaken for
inflammatory cells in a course of anterior uveitis (11). Iris
atrophy can mimic herpes simplex and herpes zoster uveitis (2).
However in viral infection the atrophy is sectoral while in PDS
the iris atrophy is localized at the midperiphery of the iris.
PDS should always be suspected when the pigment deposition is in
multiple location in both eyes, with midperipheral iris atrophy
and heterochromia, normal or elevated intraocular pressure,
keratic precipitates in a central, vertical, spindle-like
pattern and heavily pigmented trabecular meshwork.
Ocular ischemic syndrome
Ocular ischemic syndrome (OIS) is a rare condition of chronic
vascular insufficiency in which abnormalities may occur in both
anterior and posterior segment of the eye, which may mimic
intraocular inflammation. The major cause of OIS is a carotid
artery stenosis or obstruction (12). Pain, red eye, anterior
chamber cells and flare with extensive peripheral anterior
synechiae associated with rubeosis are common manifestation of
the advanced form of this disorder. Posterior segment changes
include: peripheral retinal haemorrhages, peripheral
microaneurysms, narrowed retinal arteries, optic disc and
retinal neovascularization (13). Diagnosis can usually be made
clinically, based on a detailed medical and ocular history,
complete ophthalmic examination and carotid artery evaluation.
Unilateral visual loss and the presence of characteristic
ischemic symptoms in the eye of an elderly person are all
important clues to suggest the diagnosis of OIS. Laboratory and
ancillary testing including fluorescein angiography, ERG, duplex
ultrasonography, carotid Doppler ultrasnography, carotid
arteriography, intravenous digital substraction angiography and
ophthalmodynamometry studies are helpful in establishing the
definitive diagnosis of OIS (2,13).
Retinitis pigmentosa
Retinitis pigmentosa (RP) is a group of hereditary retinal
degenerative diseases characterized by progressive degeneration
of retinal photoreceptors with associated pigmented epithelial
changes which manifest as bilateral night blindness, progressive
visual field loss and abnormal electroretinogram (ERG). In some
patients with RP the vitreous changes and cystoid macular edema
may be the earliest findings (14). In these cases the
inflammation associated with RP can masquerade as idiopathic
uveitis, especially when RP occurs as an isolated case with a
negative family history (50% of all cases), or in atypical cases
with minimal retinopathy (2). Because the classification of RP
is complicated and the clinical features associated with RP are
various at different stages, any inflammation involving both
eyes with subtle retinal pigmented changes or macular edema
should be highly suspected as a masquerade syndrome.
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Multiple sclerosis
Ocular signs and symptoms can be the first clinical
manifestation of multiple sclerosis (MS). Intermediate uveitis
is the form of ocular inflammation most commonly encountered in
patients with MS (15). Patients with MS develop a granulomatous
anterior uveitis with the presence of mutton-fat keratic
precipitates, in contrast to patients with idiopathic
intermediate uveitis who have minimal anterior segment
inflammation (16). The periphlebitis is most frequently located
at the posterior pole and there is no macular edema in contrast
to idiopathic intermediate uveitis. Optic neuritis may either
precede or follow the onset of intermediate uveitis (17). In
rare cases granulomatous iridocyclitis with iris nodule
formation and posterior uveitis may be present. Diagnosis of MS
is based on clinical criteria including a reliable history of at
least two episodes of neurologic deficit and objective clinical
signs of lesions at more than one site within the CNS. An MRI,
examination of the cerebrospinal fluid and evoked visual
potentials are helpful in establishing the diagnosis of MS (2).
Drug and post-vaccination reactions
There are many medications; drugs and vaccines that may cause
uveitis, however drug-induced uveitis is a rare event (18).
Drugs inducing intraocular inflamation include systemically,
topically administrated drugs, substances injected intraocularly
and some vaccines (Tab. I).
The drug can cause direct toxicity by itself or through its
metabolities (19). A drug can also cause uveitis by several
indirect mechanisms, mainly by stimulation of the immune system
and production of antidrug antibodies. It is also possible that
the drug nonspecifically stimulates the immune system acting as
an adjuvant. Some drugs have a high affinity for melanin and
they can also induce the release of toxic free radicals – these
can induce the intraocular inflammation due to enhanced
intrinsic uveitogenicity of melanin and free radicals toxicity
by themselves (19). Uveitis induced by drugs is characterized
most frequently by cellular infiltration in the anterior chamber
and rare in the vitreous. Anterior uveitis is usually
nongranulomatous.
In the vitreous the cell reaction is usually mild (2). There is
usually an absence of retinal or choroidal lesions. Drugs
inducing uveitis by systemic routes can produce both unilateral
and bilateral inflammation. Drug-induced uveitis is almost
always reversible within weeks of cessation of the medication
and the institution of topical treatment of the inflammation
(2).
Knowledge of the clinical features of each disease, the possible
masquerade syndromes, and the diagnosis as well as the
differential diagnosis between an ocular inflammatory disorder
and other ocular or systemic diseases is important for making a
correct diagnosis.
”We see only what we look for.
We look for only what we know”.
Johann Wolfgang Göthe
Praca została przedstawiona w formie wykładu
na I Międzynarodowym Sympozjum i Kursie: „Postępy w diagnostyce
i leczeniu zapaleń błony naczyniowej”, które odbyły się w
Krakowie w dniach 24-26.05.2007 r.
References:
1. Rothova A, Ooijman F, Kerkhoff F, Van Der Lelij A, Lokhorst
HM: Uveitis masquerade syndrome. Ophthalmology 2001, 108(2),
386-399.
2. Yao L, Foster S: Nonmalignant, Noninfectious Masquerade
Syndromes. [in]: Foster & Vitale: Diagnosis and treatment of
uveitis, chapter 50, 537-571, W.B. Saunders Company,
Philadelphia, 2002.
3. Lundstrom M, Wejde G, Stenevi U, Thorbum W, Montan P:
Endophthalmitis after cataract surgery: a nationwide prospective
study evaluating incidence in relation to incision type and
location. Ophthalmology 2007, 114(5), 866-870.
4. Samson CM, Foster CS: Masquerade Syndromes: endophthalmitis.
[in]: Foster & Vitale: Diagnosis and treatment of uveitis,
chapter 49, 528-536, W.B. Saunders Company, Philadelphia, 2002.
5. Posenauer B, Funk J: Chronic postoperative endophthalmitis
caused by Propionibacterium acnes. Eur J Ophthalmol 1992, 2,
94-97.
6. Okada AA, Johnson RP, Liles WC, D’Amico DJ, Baker AS:
Endogenous bacterial endophthalmitis. Report of a ten-year
retrospective study. Ophthalmology 1994, 101, 832-838.
7. Waheed NK, Young LH: Intraocular foreign body related
endophthalmitis. Int Ophthalmol Clin 2007, 47(2), 165-171.
8. De Souza S, Howcroft MJ: Management of posterior segment
intraocular foreign bodies: 14 years’ experience. Can J
Ophthalmol 1999, 34(1), 23-29.
9. Ivanisevic M: The natural history of untreated rhegmatogenous
retinal detachment. Ophthalmologica 1997, 211(2), 90-92.
10. Matsuo N, Takabatake M, Ueno H, Nakayama T, Matsuo T:
Photoreceptor outer segments in the aqueous humor in
rhegmatogenous retinal detachment. Am J Ophthalmol 1986, 101(6),
673-679.
11. Tugal-Tutkun I, Urgancioglu M: Bilateral acute
depigmentation of the iris. Graefes Arch Clin Exp Ophthalmol
2006, 244(6), 7427-746.
12. Bennett LW: Ocular ischemic syndrome as initial
manifestation of bilateral carotid occlusive disease. J Am Optom
Assoc 1997, 68(4), 250-260.
13. Brown GC, Magargal LE: The ocular ischemic syndrome.
Clinical, fluorescein angiographic and carotid angiographic
features. Int Ophthalmol 1988, 11(4), 239-251.
14. Nagpal A, Biswas J: Pseudouveitis – analysis of cases
misdiagnosed as posterior uveitis. Ocul Immunol Inflamm 2006,
14(1), 13-20.
15. Zein G, Berta A, Foster CS: Multiple sclerosis-associated
uveitis. Ocul Immunol Inflamm 2004, 12(2), 137-142.
16. Nussenblatt RB, Whitcup SM, Palestine AG: Intermediate
uveitis.[in]: Uveitis. Fundamentals and Clinical Practice.
Second ed., chapter 20, pp 279-289, St.Louis, Mosby, 1996.
17. Malinowski SM, Pulido JS, Folk JC: Long term visual outcome
and complications associated with pars planitis. Ophthalmology
1993, 100, 818-825.
18. Moorthy RS, Valluri S, Jampol LM: Drug-induced uveitis. Surv
Ophthalmol 1998, 42, 557-570.
19. Koneru PB, Lien EJ, Koda RT: Oculotoxicities of systemically
administered drugs. J Ocul Pharmacol 1986, 2, 385-404.
Praca włynęła do redakcji 20.12.2007 r. (1006)
Zakwalifikowano do druku 26.03.2008 r.
| Medication |
Type of intraocular inflammation and other
clinical signs |
Systemic drugs:
Antiproteases
Biphosphonates
Chlorpromazine
Cidofovir
Contraceptives
Diethylcarbamazine
Ibuprofen
Rifabutin
Streptokinase
Sulfonamides |
Anterior
Anterior, scleritis, episcleritis
Anterior
Anterior
Anterior, retinal vasculitis
Anterior, chorioretinitis
Anterior
Anterior, vitritis, retinal
vasculitis
Anterior
Anterior |
Topical drugs:
Beta-blokers
Coriticosteroids
Latanoprost
Mitomycin C
Anaestetics |
Anterior
Anterior
Anterior, CMO
Anterior
Anterior |
Intraocular injected substances:
Antibiotics (Amphotericin, bacitracin, tetracycline)
Cidofovir
Air
Perfluorocarbons
Silicone oil |
Anterior
Anterior
Anterior
Anterior
Anterior |
Vaccines:
Influenza
Hepatitis B
PPD skin test |
Anterior
Anterior, vitritis, optic neuritis
Acute multifocal placoid epitheliopathy |
Tab. I. Drugs and post-vaccinations
reactions.
Tab. I. Reakcja zapalna oka na leki i szczepionki.
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