SYMPATHETIC OPHTHALMIA
VS MEWDS: Our patients illness is distinctive
because the disease process exhibits features that are consistent
with multiple evanescent white dot
syndrome (MEWDS) as well
as sympathetic ophthalmia. The absence of a history of viral
illness before the change in vision is consistent with either
condition. Mild anterior segment inflammation, which our
patient had, can occur in sympathetic ophthalmia as well
as in MEWDS. Optic nerve head swelling, periphlebitis, and
vitreous cell also have been described in both conditions.
While the yellowish-white lesions at the level of the RPE
can be seen with either disease, the fact that the lesions
were not elevated and spared the fovea is suggestive of
MEWDS. The sex and age of the patient are typical for MEWDS.
The history of previous trauma as well as the timing of
the inflammation in the second eye, however, are suggestive
of sympathetic ophthalmia.
DIAGNOSTIC FINDINGS
AND EXCLUSION OF MEWDS:
Features of the case that do not support the diagnosis of
MEWDS or are uncommon features of MEWDS include:
1) The absence of photopsia;
2) The presence of early blocked fluorescence (vs hyperfluorescence)
on fluorescein angiography;
3) Disease recurrence within several weeks of stopping steroids
(although early recurrences with MEWDS have been reported,
they mostly occur months to years after the initial presentation);
4) The development of focal areas of peripheral RPE atrophy
(although this has been reported with MEWDS).
Persistence of the wreath-like pattern of RPE changes may
be another important distinguishing feature from MEWDS.
It is not clear that the blind spot enlargement was disproportionately
large compared to the degree of optic nerve head edema.
In MEWDS, typical subfoveal RPE granularity
tends to occur in patients with a greater degree of deceased
visual acuity than this patient exhibited.
DIFFERENTIAL DIAGNOSIS:
Conditions that should be considered
in this patients differential diagnosis include:
- Sarcoidosis;
- Mycobacterial and syphilitic uveitis;
- Vogt-Koyanagi-Harada syndrome (VKH); and
- Acute posterior multifocal placoid pigment epitheliopathy
(APMPPE) as well as conditions that may be related to
MEWDS (eg, punctate inner choroidopathy and others listed
below). Sympathetic ophthalmia and sarcoid uveitis, for
example, can exhibit Dalen-Fuchs nodules, papillitis,
and choroiditis (1, 2). Sympathetic
ophthalmia, VKH, and sarcoid uveitis are associated with
choroidal infiltration by T lymphocytes (1-5).
Both VKH and sympathetic uveitis can exhibit antibodies
directed against retinal antigens (2).
IMMUNOPATHOLOGICAL
STUDIES: Immunopathological studies have
shown that the majority of patients with sympathetic ophthalmia
(and sarcoidosis) have choroidal infiltration by T lymphocytes
(1-3, 5, 6). (Early in the disease
these are CD4+ T helper cells, and later in the disease
they are mainly CD8+ T suppressor
cells.) Shah and coworkers, however, found that B lymphocytes
predominated in 4 of 29 cases of sympathetic ophthalmia
(7). (T lymphocytes predominated
in 20 of the cases.) The presence of B lymphocytes was correlated
with disease duration greater than 9 months and with phthisis.
Clinical and laboratory findings in our patient did not
strongly support the diagnoses of VKH, sarcoidosis, vitiliginous
choroiditis, presumed ocular histoplasmosis syndrome, punctate
inner choroidopathy, multifocal choroiditis and panuveitis,
APMPPE, acute idiopathic blind spot enlargement syndrome,
or syphilis (8-10).
THIS CASE:
Areas of granulomatous inflammation, although not as numerous
as typically seen in sympathetic ophthalmia, were present
in the injured eye. We believe that this finding, although
consistent with the diagnosis of sympathetic ophthalmia,
does not confirm it. Patients with sympathetic uveitis can
have mild, transient nongranulomatous inflammation (11-15).
These findings can occur if the exciting eye is enucleated
early or if immunosuppressive therapy is administered concomitantly
(14-17). Thus, it is possible that
little granulomatous inflammation was evident because the
patient was enucleated early in the course of the disease
or because the patient received a 1-week course of systemic
prednisone.
CONCLUSION:
The pathological and clinical findings in this case indicate
that sympathetic ophthalmia can mimic MEWDS.
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