Scleritis associated with sarcoidosis

PURPOSE: To review scleritis cases associated with sarcoidosis and to evaluate clinical features, medical treatment, and clinical course. METHODS: Two cases of diffuse anterior scleritis and a nodular scleritis case associated with systemic sarcoidosis were evaluated. Scleritis was the first manifestation of sarcoidosis in two of the patients and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. The third patient had the diagnosis of systemic sarcoidosis when she developed scleritis. RESULTS: Based on the clinical and histological appearance, two patients presented with bilateral diffuse anterior scleritis and the third patient with an anterior nodular scleritis. One patient already had the diagnosis of systemic sarcoidosis at presentation, while ocular findings led to the diagnosis in the other two cases. Conjunctiva or skin biopsies revealed features compatible with the diagnosis of sarcoidosis. Scleritis attacks recurred in two patients. All patients responded well to systemic corticosteroid (prednisone) therapy. CONCLUSIONS: Scleritis associated with sarcoidosis has rarely been described and it may be the presenting sign of systemic sarcoidosis.     

Contact Lens-associated Nocardial Necrotizing Scleritis

A 52 year-old, contact lens-wearing man presented with progressive right eye pain and redness for one month. He had been evaluated and treated for necrotizing scleritis by multiple eye care specialists prior to presentation. He underwent a complete systemic work-up for both autoimmune and infectious causes of scleritis, including a culture. The culture revealed heavy growth of Nocardia asteroides complexes. The patient was treated with topical amikacin and oral Bactrim. Following several weeks of antibiotic treatment, the patient''s infection resolved completely, and his visual acuity returned to baseline status. Nocardia is a rare but potentially devastating cause of necrotizing scleritis that may affect contact lens wearers without an associated keratitis. Prompt recognition and early treatment with appropriate antimicrobial agents are critical to achieve a favorable outcome.     

Successful Tocilizumab Treatment for Scleritis

ABSTRACT

Purpose: To present a rare case of scleritis associated with a prior diagnosis of giant cell arteritis (GCA) that was unresponsive to glucocorticoids, nonsteroidal anti-inflammatory drugs (NSAIDs), methotrexate, and azathioprine, but reached and maintained a full remission with tocilizumab.

Observations: A 62-year-old Caucasian female presented with scleritis and headache. Four years earlier, the patient was diagnosed with GCA. Treatment with topical and systemic NSAIDs, prednisone and diverse disease-modifying antirheumatic drugsonly had a partial effect on the scleritis whilst the arthralgia and headaches increased. Despite the absence of laboratory evidence of active GCA, tocilizumab was started and the scleritis and headaches disappeared within several days. Prednisone could be fully tapered within 3 months and to date, 12 months after the start of tocilizumab, the patient has maintained a sustained remission.

Conclusions: Our patient demonstrates that tocilizumab might represent a therapeutic option for scleritis, and its further evaluation for this severe ocular disease is worthwhile.     

Uveitis and internuclear ophthalmoplegia as ocular manifestations of sarcoidosis

Sarcoidosis is a multisystemic granulomatous chronic disease of unknown etiology with a wide range of clinical presentations. Diagnosis of sarcoidosis in patients with ocular manifestations can be challenging.We first describe a case of sarcoidosis presented with pulmonary involvement and both uveitis and internuclear ophthalmoplegia as ocular manifestations.A 55-year-old caucasian woman with non-productive cough and weakness presented with bilateral granulomatous anterior uveitis. Few days later, the patient presented again complaining of horizontal diplopia due to internuclear ophthalmoplegia. The diagnosis of sarcoidosis was made as a result of clinical examination and systemic investigations. Particularly, high-resolution computed tomography scanning of the chest was able to identify bilateral hilar lymphadenopathy not previously detected by chest X-ray. Biopsy confirmed diagnosis showing classic non-caseating granulomas.     

Trabeculectomy with Mitomycin C in Refractory Glaucoma Associated with Nonnecrotizing Anterior Scleritis

Surgically induced necrotising scleritis (SINS) following sequential laser-assisted in situ keratomileusis (LASIK) and pterygium excision with conjunctival autograft.

Purpose:?To report a case of SINS occurring 1 month after uncomplicated pterygium excision with conjunctival autograft.

Setting:?Department of Ophthalmology, Singleton Hospital, Abertawe Bro Morgannwg University NHS trust, United Kingdom

Method:?A 70-year-old male presented with a 10 day history of redness and reduced vision in the right eye. Bilateral hyperopic LASIK and right eye pterygium excision with conjunctival autograft had been performed elsewhere, 6 months and 1 month prior to presentation. BCVA were 6/60 OD and 6/6 OS. A 2mm full thickness nasal corneal perforation with adjacent scleral melt (6.5x4mm) was evident. Tectonic full thickness corneal and scleral patch grafting with amniotic membrane graft overlay was performed with systemic immunosuppression (prednisolone 60mg od).

Results:?Recurrence of the corneascleral melt occurred one month postoperatively on reduction of prednisolone to 15mg od. This was initially managed successfully with cyclophosphamide 100mg and prednisolone 80mg. Two months postoperatively recurrent corneal perforation required the application of cyanoacrylate glue. At latest follow up four months postoperatively the corneal glue is insitu with no evidence of recurrent melt.

Conclusions:?SINS following pterygium excision with conjunctival autograft is rare with only 2 case reports in the literature. This is the first reported case of SINS occurring after pterygium excision with conjunctival autograft with preceeding LASIK.     

Aspergillus fumigatus Scleritis Associated with Monoclonal Gammopathy of Undetermined Significance

A 68-year-old woman presented with pain in her left eye. Necrosis with calcium plaques was observed on the medial part of the sclera. Aspergillus fumigatus was isolated from the culture of the necrotic area. On systemic work-up including serum and urine electrophoresis studies, the serum monoclonal protein of immunoglobulin G was detected. The patient was diagnosed with monoclonal gammopathy of undetermined significance and fungal scleritis. Despite intensive treatment with topical and oral antifungal agents, scleral inflammation and ulceration progressed, and scleral perforation and endophthalmitis developed. Debridement, antifungal irrigation, and tectonic scleral grafting were performed. The patient underwent a combined pars plana vitrectomy with an intravitreal injection of an antifungal agent. However, scleral and intraocular inflammation progressed, and the eye was enucleated. Aspergillus fumigatus was isolated from the cultures of the eviscerated materials. Giemsa staining of the excised sclera showed numerous fungal hyphae.     

Interferon-alpha-associated presumed ocular sarcoidosis

Background  Interferon alpha, used in the treatment of different viral, autoimmune and malignant diseases, is known to induce a variety of side effects. Recently, induction of sarcoidosis during interferon therapy has been reported. We analyzed patients for uveitis, possibly induced by interferon alpha. Methods  We report on three patients who had developed typical signs of ocular sarcoidosis under treatment with interferon alpha for chronic hepatitis C virus infection. In two patients, conventional interferon alpha was used and in another one, pegylated interferon alpha-2b. All patients additionally received ribavirin. Results  In all three cases, panuveitis was diagnosed. The mean duration of interferon treatment before development of uveitis was 10 months. Clinically, all patients demonstrated granulomatous panuveitis with choroidal granulomas of various sizes. In one case, the uveitis developed together with renal failure, fever and malaise. In this patient, an elevated ACE level was detected. In another patient, the diagnosis of sarcoid induced uveitis was confirmed by positive chest CT scan. The intraocular inflammation was managed with a reduction of the interferon dosage. The therapy with ribavirin was not changed. All patients received topical steroids. Systemic steroids were applied only in the case with systemic disease manifestations. Conclusions  Uveitis can be a sign of sarcoidosis induced by interferon alpha. Further studies are required to support the observation that with early diagnosis the prognosis of uveitis seems to be good.     

Granulomatous anterior uveitis associated with bimatoprost

Purpose: To describe a previously unreported case of anterior granulomatous uveitis in a patient using bimatoprost. Methods: A 72-year-old woman with a long-standing history of anisometropic amblyopia and pseudoexfoliative glaucoma in the right eye started therapy with bimatoprost 0.03% once a day in the right eye. She had no previous history of ocular inflammation or ocular surgery. Her medical history was negative for systemic diseases associated with ocular inflammation. Results: After one week, the patient developed severe conjunctival injection, cells and flare, and numerous ‘mutton fat’ keratic precipitates in the right eye. Examination of the left eye revealed no evidence of inflammation. Bimatoprost was discontinued; no topical steroid therapy was started. Systemic investigations were normal. The inflammation resolved over two weeks, solely with the discontinuation of bimatoprost. Conclusions: Bimatoprost is a synthetic prostamide, chemically related to prostamide F. Prostamides are naturally occurring substances, biosynthesized from anandamide in a pathway that includes COX2. Even though anandamide has proven suggestive potential pro-inflammatory effects, the mechanism of induction of inflammation by bimatoprost remains uncertain and speculative. In our report, the onset of acute uveitis in a patient using bimatoprost, after a long-term and well-tolerated treatment with a prostaglandin analog, suggests a distinct potential pro-inflammatory action of prostamides. This can indirectly support the concept that the target receptor of bimatoprost is different, and that the mechanism of action of prostamides is pharmacologically unique.     

Regression of peripapillary choroidal neovascular membrane in a patient with sarcoidosis after oral steroid therapy

Choroidal neovascular membrane (CNV) may occur in patients with posterior uveitis. Treatment of patients with corticosteroids induces regression of the inflammation in the posterior pole with downregulation of many cytokines including vascular endothelial growth factors. We report herewith, a case of biopsy proven sarcoidosis that developed posterior uveitis and peripapillary CNV membrane and subretinal hemorrhage with fluid. The patient was treated with systemic steroids. She demonstrated progressive regression of the CNV membrane and complete resolution of the subretinal hemorrhage and fluids. In conclusion, control of the posterior segment inflammation is crucial in the resolution of the CNV membrane in uveitis and the intravitreal anti-vascular endothelial growth factor may not be always indicated.     

Peripheral choroidal nodules in a case of proven systemic sarcoidosis

A case of sarcoidosis presenting as peripheral choroidal nodules has been described.     

Scleritis and other ocular side effects associated with pamidronate disodium

PURPOSE: To evaluate reported cases of scleritis and other ocular side effects associated with pamidronate disodium, with emphasis on previously unreported cases of scleritis.DESIGN: Observational case series. METHODS: Case reports from the National Registry of Drug-Induced Ocular Side Effects (Casey Eye Institute), the Food and Drug Administration, the World Health Organization, and the literature were reviewed to determine possible adverse ocular side effects associated with pamidronate disodium. The World Health Organization's Causality Assessment Guide was used to categorize an adverse drug reaction. RESULTS: Seventeen cases of unilateral scleritis and one case of bilateral scleritis occurred, usually within 6 hours to 2 days after intravenous pamidronate disodium. Six patients had positive rechallenge testing with the scleritis occurring after a repeat drug exposure. Other ocular side effects with positive rechallenge data, associated with pamidronate disodium, include blurred vision, nonspecific conjunctivitis, ocular pain, bilateral anterior uveitis, and episcleritis. CONCLUSIONS: This is the first report, with rechallenge data, of any drug causing scleritis. Pamidronate disodium can cause vision-threatening diseases, which may require discontinuing the drug in some uveitis cases and, in this series, all cases of scleritis.     

眼附属器结节病临床分析

目的 探讨17例眼附属器结节病临床特点和诊断治疗经验.方法 回顾性系列病例研究.回顾分析1993年1月至2008年10月在复旦大学附属眼耳鼻喉科医院治疗并经病理证实的17例眼附属器结节病患者的临床资料.结果 男性4例,女性13例;年龄15～70岁,平均46.9岁;病变位于眼眶8例,泪腺5例,眼睑4例.14例主诉局部肿块,2例主诉眼球突出,1例主诉眼睑肿胀.并发肺部结节病3例,葡萄膜炎3例,皮肤结节病1例.6例中有4例血管紧张索转化酶增高,其中2例高于正常七限2倍以上.11例眼眶CT表现为中等密度实质性占位影,1例表现为低密度囊性占位影.5例B超表现为内回声均匀的低回声占位,1例表现为内回声不均匀的低回声占位.所有患者均经手术治疗,7例患者辅以糖皮质激素口服治疗.14例患者随访1至15年,无复发或再生长,3例失访.结论 眼附属器结节病临床报道较少,多发生于中年女性,常表现为局部肿块,CT多表现为中等密度实质性占位,B超多表现为内回声均匀的低回声占位,应作为眼附属器占位性病变的鉴别诊断之一.病灶局限者可手术切除,病变广泛或并发全身病变者需辅以糖皮质激素治疗,并密切随访.     

Scleral nodule associated with sarcoidosis

PURPOSE: To describe an unusual case of an inflammatory scleral nodule associated with systemic sarcoidosis. DESIGN: Observational case report. METHODS: A patient presented with a red, painful, elevated scleral nodule on the left eye that was unresponsive to nonsteroidal anti-inflammatory drugs and topical corticosteroids. RESULTS: Laboratory evaluation was normal with the exception of elevated liver function tests. Further evaluation by a gastroenterologist revealed a granulomatous hepatitis secondary to sarcoidosis, which was confirmed by liver biopsy. Treatment with oral prednisone resulted in resolution of the hepatitis and scleral nodule. The patient has been recurrence-free over 7 months of follow-up. CONCLUSION: Although rare, scleral disease may be associated with sarcoidosis.     

Retinal macroaneurysm associated with ocular sarcoidosis

Purpose  

To characterize retinal macroaneurysm, which although rare, has been reported as a specific complication of ocular sarcoidosis.     

Ocular hypertension associated with ocular sarcoidosis

PURPOSE: To report bilateral ocular hypertension in association with ocular sarcoidosis. METHODS: Case note review of patients with a diagnosis of sarcoidosis-related uveitis. RESULTS: The authors identified 5 patients who fulfilled the diagnostic criteria for ocular sarcoidosis and who had intraocular pressures of > 40 mmHg in each eye. CONCLUSIONS: Physicians should be aware of the association of raised intraocular pressure with ocular sarcoidosis.