Open Journal of Rheumatology and Autoimmune Diseases, 2012, 2, 1-2 Published Online February 2012 ( 1
Unusual Presenting Syndrome of Rheumatoid Arthritis
Tatiana Reitblat1, Olga Reitblat2, Ori Elkayam2
1Rheumatology Unit, Barzilai Medical Centre, Ashkelon, Israel; 2Department of Rheumatology, Tel Aviv Medical Centre and The
“Sackler” Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Received November 4th, 2011; revised December 18th, 2011; accepted January 28th, 2011
We herewith report the rare case of a patient with juvenile rheumatoid arthritis who develo ped on 2 occasions the clini-
cal picture of an isolated unilateral palsy of the inferior oblique muscle—Brown’s syndrome, following pregnancies,
each time in a different eye. Although the eye is frequently involved in rheumatoid arthritis (RA), the Brown’s syn-
drome is seldom reported in literature with regards to RA.
Keywords: Brown’s Syndrome; Juvenile Rheumatoid Arthritis; Rheumatoid Arthritis
1. Introduction
The eye is frequently involved in rheumatoid arthritis
(RA). The most common manifestations are keratocon-
junctivitis sicca, often resulting in corneal involvement,
scleritis and episcleritis. However, other less frequent
ocular features have been described [1]. We herewith
report the case of a patient with juvenile rheumatoid ar-
thritis who developed on 2 occasions the clinical picture
of an isolated unilateral palsy of the inferior oblique
muscle. Following pregnancies, each time in a different
2. Case Report
A 40 years old woman, with a 25 year history of juvenile
rheumatoid arthritis (JRA), developed sudden right eye
gaze palsy together with arthritis exacerbation the first
week after giving birth to her second child. She reported
a normal 1st pregnancy without after delivery complica-
tions. At this time, a few days after completing her sec-
ond pregnancy, she presented with complains of diplopia
at an upward-in gaze. During the pregnancy, the patient
entered into remission of JRA, without need for medica-
tions. On physical examination, at presentation, she
demonstrated severe synovitis of wrists, 2, 3, MCPs, 2 -
4 PIPs and knees. The ophthalmologic examination was
consistent with right inferior oblique palsy. Laboratory
tests revealed a normocytic-normochromic anemia, and
raised ESR and CRP. Single bundle EMG of the ocular
nerves excluded neurological pathology and muscle my-
The patient was diagnosed as suffering from Brown
syndrome. She was treated with Prednisolone 30 mg a
day and Methotrexate (MTX) 15 mg a week. Her diplo-
pia slowly improved during the course of several weeks,
resulting in a slight permanent disturbance of eye move-
A few years later, she had an uneventful 3rd pregnancy
but after her fourth delivery, she pr esented with a clinical
picture similar to the one observed after her second
pregnancy: arthritis exacerbatio n and diplopia. This time,
the event involved th e left eye, at an upward-in gaze. The
patient was examined by an ophthalmologist, and diag-
nosed again as Brown syndrome. Treatment with MTX
15 mg a week was renewed and Prednisolone dose raised
to 30 mg a day (the patient was treated by prednisolone 5
mg once a day during her fourth pregnancy). Her eye
symptoms improved, although a mild residual diplopia
The patient was treated by Prednisone 30 mg/d till the
begining of improvement in her diplopia with slow Pred-
nisone tapering after that in both cases.
3. Discussion
In 1973, Brown reported an unusual motility disorder,
characterized by a limitation of upgaze in an adducted
eye [2]. The cause of this malfunction is an abnormality
of the superior oblique tendon, its sheath or the trochlea,
preventing free passage of the tendon through the tro-
chlea during inferior oblique action. These clinical fea-
tures have been reported in association with SLE [3],
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Unusual Presenting Syndrome of Rheumatoid Arthritis Exacerbation
Sjogren’s syndro me [4], JRA [5] and RA [6]. In the latter,
a stenosing tenosynovitis of the superior oblique tendon
sheath causes mechanical obstruction when attempting to
raise the eye in adduction. The problem resembles trigger
finger in the hands of RA patients [7]. Despite the com-
mon association of tenosynovitis with rheumatoid arthri-
tis, Brown syndrome is a rare complication and has been
seldom reported. While reviewing the literature, we
found only sporadic cases describing Brown’s syndrome
in association with RA or JRA. Hickling and Beck [8]
reported 6 cases during a period of 10 years. Other case
reports describe only one or two patient in each case.
Interestingly, Brown’ s syndrome may app ear when RA is
quiet under DMARDs treatment [9], as well as during
disease exacerbation. On the other hand, spontaneous
resolution of this condition may occur [10]. The tradi-
tional treatment of Brown’s syndrome is corticosteroids
with complete resolution of the disease in most cases
[11]. Our case is also unique since it appeared after
pregnancy as the first presenting sign of RA exacerbation
and recurred in the same circumstances in the another
eye. The mild residual symptoms observed in our patient
may suggest a possible congenital malformation of the
4. Conclusion
We have reported a rare case of recurrent Brown syn-
drome after pregnancy in a patient with JRA. Because of
rarity of this syndrome, it is probably often under diag-
nosed. Increased awareness to the possibility of teno-
synovitis of the superior oblique tendon as a manifesta-
tion of active RA may prompt its diagnosis and treat-
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