Treatment of idiopathic sclerosing inflammation of the orbit (myositis) with infliximab

Purpose: We report the successful use of infliximab in a young patient with idiopathic sclerosing orbital inflammation (myositis). Methods: We describe the patient’s medical records and review the literature. Results: Idiopathic myositis in this patient could not be satisfactorily treated with corticosteroids or chemotherapy. Biopsy showed sclerosing orbital inflammation. The patient had a prompt and sustained response with infliximab and methotrexate treatment. Conclusions: Infliximab and methotrexate might be considered in patients with idiopathic sclerosing orbital inflammation (myositis).     

Infliximab in the treatment of recalcitrant idiopathic orbital inflammation

A 15-year-old boy had bilateral idiopathic orbital inflammation refractory to treatment with corticosteroids. He was not only unresponsive to subsequent therapy with methotrexate but also had elevation of serum liver enzymes. In an attempt to minimize further side effects of corticosteroids and to avoid external beam radiation in an adolescent, we began treatment with infliximab, an anti-tumor necrosis-alpha antibody. His symptoms resolved and his disease has not recurred. Use of infliximab may be useful in the treatment of recalcitrant idiopathic orbital pseudotumor.     

Isolated lateral rectus myositis as a manifestation of idiopathic orbital inflammation

BACKGROUND: We report the case of of a patient with isolated, acute, monolateral and monomuscular lateral rectus myositis as a manifestation of a localized, nonspecific, idiopathic orbital inflammation. HISTORY AND SIGNS: A 25-year-old man presented to our clinic with a painful right eye and associated double vision on right gaze due to a limitation of abduction. Conjunctival hyperemia was observed in the area of the right lateral muscle insertion. A slight axial proptosis of the right eye was evident. On axial CT scans a fusiform enlargement of the right lateral rectus muscle including a thickened anterior tendon at its insertion was recorded. A thorough systemic and laboratory investigation showed neither any further abnormal findings nor any systemic disease. THERAPY AND OUTCOME: A monocular, monomuscular myositis as a clinical manifestation of idiopathic orbital inflammation was diagnosed by the clinical findings and imaging with orbital CT/MRI scans. By appropriate treatment with oral steroids slowly tapered over weeks, symptoms and signs showed full remission without any complications. Clinical improvement was observed within a few days after the beginning of steroid administration. CONCLUSIONS: Isolated lateral rectus myositis can be a manifestation of idiopathic orbital inflammation. Diagnosis is made by the clinical presentation and imaging with orbital MRI/CT-scans. Other local or systemic inflammatory, endocrine or neoplastic diseases must be ruled out.     

Assessment of inflammation in idiopathic orbital myositis with fat-suppressed T2-weighted magnetic resonance imaging

PURPOSE: To evaluate the inflammation in idiopathic orbital myositis by fat-suppressed T2-weighted magnetic resonance imaging (MRI). DESIGN: Observational case series. METHODS: The inflammation in nine patients with orbital myositis was evaluated with fat-suppressed T2-weighted MRI within three weeks of onset. Patients were mainly treated with steroid pulse therapy and followed to the acute improvement or chronic ocular motility restriction phases. RESULTS: Each patient was found to have localized inflammations in the extraocular muscles or associated fascial structures. Of the five patients with acute improvement, none had inflammation of the extraocular muscles, whereas the four patients with chronic ocular motility restriction had inflammation of the extraocular muscles. CONCLUSION: These results suggest that acute or chronic pattern in orbital myositis is correlated with the localized inflammation. The inflammation in the extraocular muscles may be one of the factors that lead to the chronic ocular motility restriction.     

Successful treatment of idiopathic orbital inflammation with infliximab: an alternative to conventional steroid-sparing agents

Therapy for orbital pseudotumor, also called idiopathic orbital inflammation, is nonspecific and mainly based on the use of corticosteroids. Two patients with steroid-dependent idiopathic orbital inflammation, one with myositis and the other with dacryoadenitis, and intolerant of standard steroid sparing agents (methotrexate or azathioprine), were treated with infliximab, a monoclonal antitumor necrosis factor alpha antibody. In both patients, orbital manifestations disappeared following treatment. After follow-up for at least 20 months, the 2 patients still receiving infliximab remained event free. Infliximab enabled steroid tapering to less than 5 mg per day, suggesting that infliximab could constitute an alternative to conventional steroid sparing agents.     

Acute and chronic orbital myositis

Ocular muscle diseases fall into two distinct groups: acute or subacute exophthalmic myositis and chronic oligosymptomatic myositis. The diagnosis can only be established with certainty by means of B-scan sonography and CT scanning; these examinations demonstrate the irregular thickening of one or several eye muscles. The disease is apparently primitive; in some cases concomitant autoimmune factors may be found. The course is spontaneous, with continual recurrence, and may have sequelae in the form of a definitive exophthalmos or an acquired retraction syndrome. It responds remarkably well to corticotherapy and, if necessary, immunosuppressors; in general, these lead to rapid and - if treatment is started in time - total regression of the disease.     

Bilateral infraorbital nerve involvement in idiopathic orbital myositis

We describe a case of bilateral infraorbital nerve involvement in idiopathic orbital myositis. Computed tomographic scanning of the orbits showed enlargement of extraocular muscles consistent with orbital myositis and enlargement of the ipsilateral infraorbital nerve. With disease progression, infiltration of the contralateral infraorbital nerve also developed. Biopsies of the inferior oblique muscle and infraorbital nerve showed the same inflammatory infiltrate: a mixed population of lymphocytes (B and T) with B cells expressing kappa and lambda light chains. Although orbital myositis may be associated with several conditions, to the best of our knowledge, this is the first time a case of idiopathic orbital myositis with bilateral infraorbital nerve involvement has been described.     

Successful treatment of idiopathic orbital inflammation with mycophenolate mofetil

PURPOSE: To report our experience with the use of mycophenolate mofetil for treatment of idiopathic orbital inflammation (IOI). DESIGN: Noncomparative interventional case series. METHODS: We reviewed the records of five patients with IOI who were treated with mycophenolate mofetil. The indications for use included recurrent inflammation after other therapies (four patients) and to avoid corticosteroid-induced blood sugar elevation in poorly controlled diabetes mellitus (one patient). Treated patients were followed for a median of 8.5 months (range, 4-30 months) after mycophenolate mofetil therapy was begun. Main outcome measures were response to treatment, treatment-related side effects, drug dosage, previous and final treatment regimens, and discontinuation of systemic corticosteroids. RESULTS: Mycophenolate mofetil therapy was discontinued in one patient because of nausea. Each of the remaining four patients experienced complete resolution of orbital inflammation. Of these, only one patient remains on prednisone, but at a significantly reduced dose (1 mg/d). CONCLUSION: Mycophenolate mofetil therapy resulted in the resolution of inflammation in patients with refractory or corticosteroid-dependent IOI and was used successfully for a first episode of IOI in a patient for whom corticosteroids were contraindicated.     

A review of sclerosing idiopathic orbital inflammation

PURPOSE OF REVIEW: Sclerosing idiopathic orbital inflammation is a rare, distinct subset of orbital inflammation. No consistent therapeutic regimen has been described and the majority of reports on this disease are case reports or small case series. This review looks at historical and current treatment modalities in an attempt to define the most current, effective management strategy for this disease. RECENT FINDINGS: Recently, the largest review to date of these patients was published, detailing clinical findings and responses to the various interventions. Two other case reports of patients who responded completely to therapy appeared in the literature. SUMMARY: Sclerosing idiopathic orbital inflammation is a rare disease with an unknown pathogenesis and poor prognosis. Current treatments are disappointing and often have little beneficial effect. There are anecdotal reports of success using combinations of immunosuppression, radiotherapy, and surgery, but a consistent, effective treatment course is lacking. Younger patients and those with a shorter duration of disease seem to have the best prognosis. Further research into the immunologic basis of the disease and a large, controlled study comparing the various sclerosing idiopathic orbital inflammation treatments are needed to determine the most effective treatment regimen.     

IgG4-related systemic disease as a cause of "idiopathic" orbital inflammation, including orbital myositis, and trigeminal nerve involvement

IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intractable disease, biopsy showed IgG4-RD involving the lacrimal gland, extraocular muscles, intraconal fat, and trigeminal nerve. Six months after initiating treatment with rituximab, his disease remained dormant, with improvement in his proptosis and normalization of serum IgG4 levels. We review the differential of idiopathic orbital inflammatory disease, including IgG4-RD, and emphasize the need for biopsy for accurate diagnosis and to guide appropriate treatment.