|Year : 2011 | Volume
| Issue : 3 | Page : 139-141
Adalimumab in the treatment of recurrent idiopathic bilateral nodular scleritis
Ahmed M Bawazeer, Lina H Raffa
Department of Ophthalmology, King Abdulaziz University Hospital, Jeddah, Saudi Arabia
|Date of Web Publication||29-Dec-2011|
Ahmed M Bawazeer
Department of Ophthalmology, King Abdulaziz University Hospital, P.O. Box 80215, Jeddah 21589
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Our objective is to report a case of bilateral nodular scleritis in a 34-year-old patient, resistant to steroids and traditional disease modifying anti-rheumatic drugs, who was successfully treated with subcutaneous injections of 40 mg adalimumab. Adalimumab resulted in rapid control of scleritis in both eyes within 3 months with no recurrence over 5 years of follow-up. No side effects were reported during treatment.
Although a large prospective study and a longer follow-up are required to reach a conclusive result, adalimumab has a potential role in the treatment of the above condition with the control of inflammation.
Keywords: Adalimumab, resistant scleritis, TNF-alpha antagonists
|How to cite this article:|
Bawazeer AM, Raffa LH. Adalimumab in the treatment of recurrent idiopathic bilateral nodular scleritis. Oman J Ophthalmol 2011;4:139-41
|How to cite this URL:|
Bawazeer AM, Raffa LH. Adalimumab in the treatment of recurrent idiopathic bilateral nodular scleritis. Oman J Ophthalmol [serial online] 2011 [cited 2020 May 30];4:139-41. Available from: http://www.ojoonline.org/text.asp?2011/4/3/139/91271
| Introduction|| |
Scleritis is a chronic, painful, and potentially blinding inflammatory disease that is characterized by edema and cellular infiltration of the scleral and episcleral tissues. Scleritis affects females slightly more frequently than males and is most commonly found in patients who are 40-60 years of age.  Patients who are symptomatic in only one eye typically have bilateral involvement within 5 years.  Scleritis may be the initial or only presenting clinical manifestation of potentially lethal disorders. Correct and rapid diagnosis coupled with the appropriate systemic therapy can not only halt the relentless progression of both ocular and systemic processes but also prevent the destruction of the globe and prolong survival.
Ocular inflammatory disorders such as uveitis and scleritis usually follow a benign clinical course; however, more serious forms may lead to vision loss and even enucleation of the eye.  Immunosuppressive drugs seem to be a rescue therapy for relapses of ocular inflammation or as maintenance therapy where corticosteroids have failed. 
In this case report, we present a case of bilateral nodular scleritis resistant to both steroids and traditional disease modifying anti-rheumatic drugs (DRAMDs) treated with adalimumab.
| Case Report|| |
A 34-year-old male patient presented with redness, pain in both eyes, and persistent headache that lasted for 5 weeks. He was diagnosed 8 years ago with idiopathic bilateral nodular scleritis. The patient tested negative for rheumatoid arthritis, Wegener's granulomatosis, Sjogren's disease, sarcoidosis, gout, syphilis, diabetes, or hypertension.
Ophthalmic examination revealed visual acuity of 20/20 both eyes. Slit lamp examination of both eyes showed sectoral nodular scleritis inferotemporally [Figure 1]. Severe tenderness was noted in these areas. Cornea was clear. There were trace cells in the anterior chambers of both eyes. The intraocular pressures were within normal range. On ophthalmoscopy, the posterior segment in both eyes was normal. The ultrasound images did not indicate posterior scleritis.
|Figure 1: This image illustrates sectoral nodular scleritis inferotemporally before treatment with adalimumab|
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Systemic work-up was negative for rheumatoid factor (RF), anti-nuclear antibody (ANA), anti-neutrophil cytoplasmic autoantibody (ANCA), venereal disease research laboratory (VDRL), rapid plasma regain (RPR), and Treponema pallidum emagglutination (TPHA). Further investigations including complete blood analysis, fasting blood sugar, uric acid, liver and renal function tests, urine analysis, angiotensin converting enzyme (ACE), erythrocyte sedimentation rate, and C-reactive protein levels were within normal ranges. Purified protein derivative (PPD) test and chest x-ray showed no remarkable findings. Evaluation by the internal medicine and rheumatology departments revealed no abnormalities. Bilateral temporal artery biopsies were taken and revealed no significant findings. The magnetic resonance imaging of the brain was normal.
The patient was started on pred forte eye drops hourly, voltaren eye drops qid, and oral ibuprofen 400 mg three times a day for 6 weeks but was unresponsive. Oral prednisolone was therefore added at a dose of 60 mg/day (1 mg/kg) and was tapered gradually to 15 mg per day over 3 months. Further attempts to reduce the dose resulted in recurrence of the inflammation. The patient was maintained on 15 mg/day of prednisolone for an additional 3 months. Both voltaren and pred forte eye drops were discontinued 6 months after starting the prednisolone. Azathioprine 100 mg daily was added as a steroid sparing agent for 2 months but was later discontinued due to the patient's intolerance and development of severe skin rash. Cyclosporine was deferred as the patient had uncontrolled hypertension. Methotrexate was given at a dose of 12.5 mg weekly for 4 months but was stopped as it resulted in elevated liver enzymes. The patient decided against cyclophosphamide treatment due to the side effects related to the medication. Infliximab was then started and continued for 9 months; however, it failed to control his attacks. In July 2006, he developed bilateral exacerbation of severe nodular scleritis. Consequently, oral prednisolone was increased to 60 mg/day and pred forte eye drops were resumed hourly, voltaren eye drops four times daily and oral ibuprofen three times daily. Voltaren and pred forte eye drops were discontinued after 3 months. Adalimumab 40 mg was started in the same period subcutaneously every 2 weeks with rapid control of scleritis in both eyes within 3 months [Figure 2]. It was continued for 2 years. No side effects were reported during treatment with adalimumab and the patient was regularly followed by a rheumatologist. Oral prednisolone was tapered slowly to 7.5 mg/day after 1 year and the patient has been on 5 mg daily for the past 4 years. Currently, the patient is stable and his visual acuity is 20/20 both eyes and is maintained on voltaren eye drops four times a day, oral ibuprofen 400 mg t.i.d PRN, and oral prednisone five mg daily after having stopped adalimumab for 3 years. The total follow-up period from the first visit is 7 years and the follow-up period after stopping adalimumab is 3 years.
|Figure 2: This figure illustrates rapid control of scleritis post treatment with 40 mg adalimumab subcutaneously within 3 months|
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| Discussion|| |
Tumor necrosis factors (TNFs) are pleiotropic cytokines that are considered to be the primary modifiers of inflammatory and immune reactions. As a proinflammatory cytokine, TNF-α has an important role in the etiology of ocular inflammation.  Blocking this cytokine can interrupt the cascade of events that cause damage to involved tissues. Based on the improved understanding of the immune system, new pharmaceutical agents including etanercept, infliximab, daclizumab, and adalimumab have been designed to control inflammation in various forms of ocular inflammatory disorders. 
Recent reports have proved that anti-TNF alpha therapy was efficacious in the treatment of different forms of uveitis involving Juvenile Idiopathic Arthritis and Behcet's disease. 
Adalimumab is a subcutaneously administered, recombinant, human IgG1 monoclonal antibody specific for human TNF. Adalimumab gained FDA approval for the treatment of rheumatoid arthritis in December 2002. In addition, it has been approved for the treatment of psoriatic arthritis, ankylosing spondylitis, and Crohn's disease.  The most common side effect of adalimumab is injection site reactions. Its most notable infectious complication is the reactivation of tuberculosis. Rare side effects include skin rashes, worsening or initiation of congestive heart failure and neurological diseases, lupus-like syndrome, promotion of lymphoma, and medically significant cytopenia. 
Restrepo et al. and Huynh et al. reported similar results in cases of severe nodular scleritis treated successfully with adalimumab. , Even though a larger spectrum study needs to be conducted to give a definitive answer, adalimumab might prove to be a milestone in the treatment of the aforementioned condition.
| References|| |
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[Figure 1], [Figure 2]
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