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Bilateral Sequential Trochleitis as the Presenting Feature of Systemic Lupus Erythematosus

Fonseca, Pedro MD; Manno, Rebecca L. MD; Miller, Neil R. MD

Journal of Neuro-Ophthalmology: March 2013 - Volume 33 - Issue 1 - p 74–76
doi: 10.1097/WNO.0b013e318275a597
Clinical Observation

Abstract: A 26-year old woman presented with headache and pain in the left superonasal orbit, which worsened with vertical eye movements. She had no relevant medical history, and ophthalmologic evaluation was unremarkable. An orbital ultrasound showed enlargement of soft tissue in the region of the left trochlea consistent with trochleitis. Treatment with prednisone and multiple local injections of corticosteroids and analgesic nerve blocks failed to relieve her symptoms. The patient subsequently experienced right trochleitis, and 2 years after the onset of her initial symptoms, she developed systemic symptoms and signs that led to a diagnosis of systemic lupus erythematosus (SLE). Systemic immunosuppressive therapy was instituted, and the patient experienced marked relief in her ophthalmic symptoms. This case is unique in that not only bilateral sequential trochleitis was the presenting feature of SLE but also the ocular manifestations preceded the systemic manifestations of SLE by over 2 years.

Neuro-Ophthalmology Division, The Wilmer Eye Institute (PF, NRM)

Department of Medicine, Division of Rheumatology (RLM), the Johns Hopkins Hospital, Baltimore, Maryland.

Address correspondence to Neil R. Miller, MD, Wilmer Eye Institute, The Johns Hopkins Hospital, Maumenee 127, 600 N Wolfe Street, Baltimore, MD; E-mail:

The authors report no conflicts of interest.

Trochleitis, or inflammation of the superior oblique tendon/trochlear pulley, was originally described in 1984 by Tychsen et al (1). Patients with this condition complain of orbital pain and tenderness when the affected region is palpated and pain on vertical eye movements, especially in adduction. Imaging studies generally show inflammation in the trochlear region, and symptoms usually improve with local or systemic corticosteroids.

Trochleitis causing an acquired Brown syndrome may be associated with systemic inflammatory diseases, including rheumatoid arthritis (2,3), juvenile idiopathic arthritis (4,5), and systemic lupus erythematosus (SLE) (6–9). We have examined an adult patient with bilateral sequential trochleitis with a poor response to local treatment, who later developed features of SLE.

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A 26-year-old woman developed a severe throbbing headache followed 2 days later by discomfort in the left superonasal orbit worsened by vertical eye movements. She had a history of gastroesophageal reflux but was otherwise in good health. Computed tomography of the brain and orbits was read as normal except for a polyp in the left sphenoid sinus. Otolaryngologic and ophthalmologic examinations revealed no abnormalities, and she was treated with analgesics and a trial of antibiotics without relief. Four weeks after the onset of symptoms, the patient was diagnosed with herpetic keratitis in the left eye and treated successfully with topical trifluridine. Orbital ultrasound showed thickening of the tissues in the region of the left trochlea. A diagnosis of trochleitis was made, and the patient was treated with oral prednisone, which increased the headache and caused drooping of the left upper eyelid. Prednisone was discontinued, and she was treated with a combination of opioid analgesics and valacyclovir with minimal relief.

Eight months after the patient developed left orbital pain, we evaluated her at our institution. Visual acuity was 20/20 in both the eyes, and all aspects of her neuro-ophthalmic examination were normal including extraocular movements. The patient was orthophoric at distance and near and in the cardinal positions of gaze; however, she complained of marked discomfort in the left superonasal orbit when attempting to look up and to the right. Palpation of the orbits revealed firmness and tenderness in the region of the left trochlea.

The patient was treated with an injection in the left trochlear region of a mixture of dexamethasone 0.4 mg and triamcinolone 4 mg with marked improvements in her symptoms; however, the symptoms recurred within several weeks, and over the ensuing months, she received several additional injections with no significant improvement.

Five months after the first injection, orbital ultrasound again showed “significant low-reflective widening of the soft tissues superonasally, in the region of the trochlea on the left” (Fig. 1A, B). Magnetic resonance imaging, that included magnified thin sections through the orbits with fat-saturated contrast-enhanced images, revealed no abnormalities in the region of either the trochlea or the superior oblique muscles. A mucocele of the sphenoid sinus was found for which the patient underwent uneventful transnasal endoscopic surgery. She continued to have swelling and pain in the left trochlear region.

Nineteen months after the onset of symptoms, the patient developed pain in the right superonasal orbit. Orbital ultrasonography showed changes consistent with bilateral trochleitis, and she was given an injection of a mixture of dexamethasone and xylocaine in the right trochlear region that failed to relieve the pain.

One month later, the patient underwent rheumatologic evaluation at which time she had no systemic symptoms or signs suggestive of a connective tissue disease. Autoantibody testing revealed a positive anti-nuclear antibody (ANA) at a low titer of 1:40 with a nucleolar pattern. Anti–double-stranded DNA antibodies were positive at a low titer of 1:10, but when repeated 3 weeks later, they were negative. In the absence of other stigmata, a diagnosis of a connective tissue disease could not be established.

Over the next year, the patient continued to experience severe pain superonasally in both orbits. She also experienced 2 weeks of oblique binocular diplopia but was not examined during that period. She was treated with several oral analgesic agents, including opioids, without relief.

Approximately 27 months after the onset of left trochleitis, the patient noticed blue-purple discoloration of her fingers on exposure to cold, consistent with Raynaud phenomenon. She also developed alopecia, paresthesias, and joint pain. Four months later, rheumatologic examination revealed livedo reticularis and markedly abnormal nail fold capillary microscopy with abnormal dilated capillary loops and areas of dropout. Electromyography and nerve conduction studies demonstrated nonirritable myopathy, and serologic evaluation showed persistently positive double-stranded DNA antibodies, hypocomplementemia (C3: 75 mg/dL [normal: 79–152 mg/dL]), and an ANA titer of 1:160 with a homogeneous pattern. A diagnosis of SLE was made, and the patient was started on hydroxychloroquine and given a short course of intravenous corticosteroids, followed by oral prednisone and azathioprine. Over the next 3 months, she reported marked improvement in her left orbital pain, and the swelling in the left trochlear region decreased significantly. Raynaud phenomenon persisted, but her livedo reticularis disappeared, double-stranded DNA antibodies became undetectable, and complement levels increased.

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In their original series of 13 patients with trochleitis, Tychsen et al (1) enumerated the diagnostic criteria for trochleitis: 1) inflammation of the superior oblique tendon/trochlear pulley in the superonasal aspect of the anterior orbit, 2) orbital pain and tenderness to palpation in that region, and 3) often pain with vertical eye rotations (especially in adduction). All had changes on orbital ultrasound, including increased thickness of the superior oblique muscle, superior oblique tendon, and peritrochlear tissues. Twelve patients underwent complete blood count with differential, erythrocyte sedimentation rate, ANA titers, and assay for rheumatoid factor. Two had a positive rheumatoid factor without other signs of connective tissue disease. Initially, all patients had complete resolution of their symptoms when treated with systemic steroids, local injection of steroids, or systemic nonsteroidal anti-inflammatory drugs. However, during an average follow-up period of 8 months, 3 experienced recurrent symptoms at 2, 6, and 11 months. Although none of the 13 patients had features of Brown syndrome, 3 had transient diplopia, suggesting some form of restriction of the oblique muscle tendon in the trochlea. Our patient also experienced a 2-week episode of oblique vertical diplopia.

A literature search using PubMed identified 4 cases of Brown syndrome associated with SLE involving 3 adults (6,7,9) and a 14-year-old girl (8). All 4 patients presented with diplopia and restricted elevation of the affected eye. Three of the patients were known to have SLE at the time of diagnosis; in the fourth patient (6), the diagnosis was made shortly after the onset of ophthalmic symptoms and signs. All 4 patients had unilateral trochleitis, with 3 responding to treatment with nonsteroidal anti-inflammatory drugs or oral prednisone. None of these patients were treated with local corticosteroid injection.

Our patient is unique in that she presented with painful left trochleitis that was unresponsive to oral treatment with prednisone. An initial injection of a mixture of dexamethasone and triamcinolone improved her symptoms, but the effect was short-lived, and subsequent treatment with local injections failed to relieve her pain. The patient subsequently developed right trochleitis, but it was more than 2 years after the onset of her initial symptoms that she was diagnosed with SLE. Only after systemic therapy, including hydroxychloroquine, intravenous corticosteroids, followed by oral prednisone and azathioprine, did the patient’s orbital pain improve significantly. Although this is only 1 case, our report suggests that in a patient with trochleitis, a rheumatologic assessment should be performed, and the patient should be carefully followed for the development of a connective tissue disease.

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© 2013 by North American Neuro-Ophthalmology Society