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The current recommended treatment for mild PS is oral nonsteroidal anti-inflammatory drugs (NSAIDs); however, steroids and steroid-sparing immunosuppressive treatment are required for more severe cases.
Here, we present a 9-year follow-up study of a patient with pediatric PS with a history of multiple flare-ups while on oral steroids, methotrexate, and mycophenolate, who achieved resolution using infliximab and maintained remission after cessation of treatment. Our case represents only the second case report of infliximab use in paediatric PS.
We present a patient with childhood-onset PS. Written consent was obtained from the patient guardian. The data on patient's electronic chart, including history, systemic and ocular findings, laboratory results, investigations, and treatment, were reviewed.
A 9-year-old boy with a history of asthma, sickle cell trait, and no significant birth history presented with a 4-day history of fever, left eye pain and swelling, foreign body sensation, and tearing. Eye examination showed unilateral decreased visual acuity (right and left eyes 0.1 and 0.7 logMAR, respectively) with equal, round, and reactive pupils to light. There was pain with restriction of ocular motility in the left eye and 3 mm of proptosis, periorbital swelling and injected conjunctiva. Dilated fundus examination showed left optic nerve edema (Fig. 1A). The laboratory results showed leukocytosis with increased neutrophils. After a diagnosis of presumptive orbital cellulitis, he was started on intravenous (IV) cefotaxime, cloxacillin, and clindamycin. Computerized tomography (CT) scan showed diffuse scleral thickening of the left eye associated with swelling of the lateral pre- and postseptal soft tissues extending into retrobulbar fat and surrounding the distal optic nerve. Three days of treatment did not achieve clinical improvement, and a new finding of anterior uveitis was noted. Repeat CT scan showed worsening of the orbital signs more indicative of inflammatory disease rather than orbital cellulitis. An ultrasound B-scan confirmed the characteristic T sign of scleritis (Fig. 1B), and a diagnosis of unilateral severe PS was made. He received 2 weeks of IV methylprednisolone 900 mg/day, which controlled the inflammation and improved his vision to 0.0 logMAR.
All infective work-up including Tuberculosis, syphilis and the Herpes Simplex, Varicella-zoster, Cytomegalovirus, Epstein-Barr, Human Herpes, Human immunodeficiency, Hepatitis B and Hepatitis C viruses, as well as autoantibodies including antinuclear antibody (ANA), anti-dsDNA, antineutrophil cytoplasmic antibody, antimyeloperoxidase antibody, anti-proteinase 3 antibody, anticardiolipin antibody, and rheumatoid factor were negative. We excluded Immunoglobulin (Ig) G4 disease and the total serum levels of IgG, IgM, and IgA were also within the normal range. He was discharged on oral prednisolone 40 mg per day on a slow taper and methotrexate subcutaneous injection 25 mg per week. He re-presented with severe left scleritis 6 months later while on oral prednisolone 10 mg and methotrexate 25 mg, requiring further IV methylprednisolone treatment for 2 weeks. Mycophenolate 540 mg was also added to prednisolone 40 mg and methotrexate in order to control the scleritis. The patient exhibited 3 additional flare-ups of bilateral scleritis over the following 3 years (Fig. 2) as a consequence of tapering the oral prednisolone to 10 mg. These flare-ups were controlled by hospitalization and receiving pulse of IV methylprednisolone. The family's initial resistance to treatment with antitumour necrosis factor medication required further discussions at this stage. They agreed to infliximab 300 mg infusion every 4 weeks, and methotrexate and mycophenolate were stopped. The patient successfully weaned off oral prednisolone. He achieved sustained remission on infliximab alone for 4 years, except for one flare-up owing to treatment noncompliance, which resolved quickly on resumption of infliximab. He tapered off infliximab over a further period of 1 year. The patient had a mild flare-up of scleritis 6 months after stopping infliximab, which responded to ibuprofen 400 mg 3 times a day for a month with no further episodes. He stays in remission with 0.1 logMAR uncorrected vision and a normal eye examination 9 years after initial presentation.
PS is rare in children, with a nonspecific, heterogeneous clinical presentation. Ocular pain, redness, lid involvement, and restriction of extraocular muscle movement are the most common early signs of pediatric PS, which are also features of other common diseases affecting children. Our case was initially managed as orbital cellulitis owing to significant pain and restricted ocular motility, proptosis, fever, and raised white cell counts. Orbital cellulitis is much more common than PS and represents a life-threatening differential diagnosis, which must be excluded when acute orbital inflammation is noted. On the other end of severity scale, Mallick et al.
reported a case of a 14-year-old boy with eye redness, decreased vision, and conjunctival congestion who initially was managed as viral conjunctivitis. After careful clinical examinations and imaging, he was diagnosed with PS and was subsequently treated with oral NSAIDs and topical steroid for 2 weeks, resolving the eye inflammation and improving his visual acuity to 20/20.
Owing to exhibiting side effects or relapse on low-dose corticosteroid, at least one immunosuppressive drug was added to control the inflammation. In the current case report, the patient experienced 3 separate episodes of flare-up whilst on full dose of methotrexate and mycophenolate when oral prednisolone was reduced to 10 mg/day (0.2 mg/kg/day). Therefore, infliximab 300 mg every 4 weeks was successfully started to control his inflammation. Only one previous case report on paediatric PS used infliximab for disease resistant to methotrexate, methylprednisolone, and cyclosporine treatment.
Our case likely represents a more severe case requiring much longer immunomodulation with infliximab (5 years total) to achieve long-term remission. Although there are published data on infliximab therapy for childhood refractory uveitis,
it is encouraging to report safe and effective disease control in PS as well.
In conclusion, the majority of pediatric PS cases in literature report poor disease control with only NSAIDs or corticosteroids, with many requiring at least one immunosuppressive agent to avoid steroid side effects. We propose that infliximab can be considered an alternative treatment for severe paediatric PS resistant to treatment.
Footnotes and Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.