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Scleritis is typically a non-infectious granulomatous inflammatory process. It may be found in conjunction with systemic disease, more commonly in those patients with necrotising anterior scleritis. We describe a patient with posterior scleritis and polymyalgia rhematica. This association has not previously been reported.
The clinical differentiation of the various subtypes of scleral and episcleral inflammation is crucial for its early and adequate management. Particularly important is the recognition of necrotizing scleritis, since it is frequently associated with ocular complications, going along with a bad ocular prognosis. In patients who suffer from associated vasculitic systemic disease, the appearance of scleritis indicates a generalization of the vasculitis, which can result in potentially lethal systemic complications. Only the early diagnosis and an adequate aggressive therapy can preserve ocular functions and the patients life. Conventional steroid therapy generally fails to control the inflammatory activity in necrotizing scleritis. However, application of nonsteroidal immunosuppressive drugs has been shown to control the vasculitic conditions in the majority of cases, to improve ocular prognosis and to reduce mortality.
Posterior scleritis is an often misdiagnosed disease of the eye. Mainly it appears in elderly women and tends to be recurrent. Symptoms leading to diagnosis are swelling of the eye lids, a red eye, disturbances of the motility and protrusio bulbi. In rare cases you find exudative choroidal or retinal detachment, edema of the macula, or the optic nerve head, and secondary angle closure glaucoma. Often posterior scleritis is associated with general illness as herpes zoster, mixed connective tissue diseases, or Boeck's disease. Differential diagnosis are choroidal tumors as for example, melanoma, hemangioma, and metastases. The typical uveal effusion can also be caused by an arterio-venous fistula, panretinal photocoagulation, buckling procedure for retinal detachment, and by intraocular surgery in general. Especially cyclitis anularis pseudotumorosa has to be considered and shut out. Most important diagnostical means are ultrasound, and CT-scan. The underlying case describes an 81 years old woman that presented with acute angle closure glaucoma, and exudative choroidal detachment of the right eye. The ultrasound and CT-scan investigations confirmed the diagnosis of scleritis posterior. The acute angle closure glaucoma, and the choroidal detachment regressed immediately under the treatment with steroids given locally and systemically. There was no impact of miotics and peripheral iridectomy which both could not avoid recurrence of angle closure glaucoma.
The onset of post-operative corneal melting and necrotizing scleritis in patients with rheumatoid arthritis and collagen vascular disease who undergo cataract surgery can have devastating ocular and systemic consequences. Even though ocular surface factors such as sicca and surgical trauma are among the important variables that contribute to this entities, signs and symptoms of systemic disease need to be thoroughly investigated in order to prevent life-threatening complications associated with these ocular manifestations. The management of surgical induced corneal melting and necrotizing scleritis in these patients, include local therapy and in most instances, systemic immuno-modulation. Moreover, the development of corneal melting and necrotizing scleritis in an otherwise "healthy" patient after uncomplicated cataract surgery, can be the first manifestation of a serious occult systemic disease. Therefore, an aggressive approach regarding the diagnosis, workup and treatment should be initiated by the ophthalmologist in order to maximize a successful ophthalmic and medical outcome.
Nontuberculous mycobacterial (NTM) infections have become increasingly important in ophthalmology, particularly with keratorefractive surgery. We report a case of scleral buckle associated NTM scleritis occurring in a 69-year-old male after silicone sponge explant removal. Purulent scleral ulceration with nodule formation persisted despite topical antimicrobial therapy, buckle removal, and surgical debridement. Eventually, tissue biopsy revealed noncasseating granulomas with acid-fast bacilli that were identified in culture as Mycobacterium chelonae. The infection resolved only after administration of systemic antibiotics. NTM are important pathogens in scleral buckle associated scleritis and should be considered in persistent cases. Surgical therapy remains the cornerstone of therapy, but antimicrobials, particularly newer fourth generation fluoroqunilones, may have an important role in treating scleral buckle associated NTM scleritis.
Aspergillus scleritis is a potentially devastating ocular infection difficult to treat because of poor scleral vascularity. Most Aspergillus cases occur following ocular surgery, but others have been associated with trauma or intravenous drug use. No anti-fungal agents are consistently efficacious in the treatment of scleral fungal infections. We report a case of Aspergillus scleritis successfully treated with a combination of voriconazole and caspofungin, as well as a review of the literature concerning treatment of Aspergillus scleritis.
Nocardial necrotizing scleritis is uncommon, but potentially sight threatening. We report the occurrence of necrotizing scleritis in a 58-year-old farm worker after he sustained trauma with vegetable matter. Microbiological studies isolated Nocardia asteroides, sensitive only to cefazolin. Clinically the patient responded well to this drug, attaining a final visual acuity of 20/40, with complete resolution of the scleritis. Early isolation and testing for antibiotic sensitivity were responsible for the successful management of this patient.
PURPOSE: To report a case of congenital erythropoietic porphyria that presented as acute scleritis over a bilateral scleromalacia perforans in the interpalpebral fissure. METHODS: An 18-year-old man presented with painful red eye, a history of photophobia, and passing highly colored urine since childhood. Dermatological and biochemical evaluations were done. RESULTS: The patient had normal vision in both eyes with bilateral scleromalacia perforans. The right eye showed painful, nodular scleritis. Dermatological examination revealed multiple, vesciculobullous cutaneous lesions with atrophy and pseudoscleroderma changes, hypertrichosis, and bluish discoloration of teeth. Immunofluorescent microscopy of fresh peripheral smear showed brilliant red fluorescence of erythrocytes. Spectroscopic analysis of urine revealed excretion of porphyrin, thus confirming a diagnosis of congenital erythropoietic porphyria. The patient's condition improved with local and systemic steroid therapy along with general photoprotective measures for the exposed parts of the body. CONCLUSION: Acute scleritis could be the presenting feature in a rare case of congenital erythropoietic porphyria, warranting systemic evaluation.
PURPOSE: To review the efficacy of Etanercept as an alternate therapy for treatment of necrotizing anterior scleritis and sterile corneal ulceration unresponsive to traditional therapies. METHODS: A retrospective review of 10 patients treated with Etanercept for vision-threatening scleritis and sterile corneal ulceration. RESULTS: Etanercept alone or in combination with other immunosuppressive therapies controlled inflammation, arrested tissue ulceration, and in many cases permitted tapering or cessation of toxic immunosuppressive therapies. No complications or systemic toxicity were observed with Etanercept use. CONCLUSION: Etanercept is an effective treatment for scleritis and sterile corneal ulceration and has a favorable benefit-to-risk ratio. It may be considered for therapy of progressive disease or cases that are unresponsive to traditional therapies.
OBJECTIVE: To report the subpalpebral lavage therapy for the treatment of infectious scleritis and keratitis. METHOD: Six patients were admitted for treatment of severe infectious scleritis and keratitis and were initiated on the subpalpebral lavage system after showing no improvement with topical fortified antibiotics. A continuous antibiotic lavage was applied until clinical sterility was achieved and topical steroids were gradually added to control concomitant inflammation. RESULTS: All 6 patients had resolution of their infections and achieved a stable ocular surface with no inflammation. One patient required a corneal transplant for active inflammation and corneal thinning, 1 had a transplant for a dense central corneal scar, and 1 patient underwent corneal transplant and cataract extraction. One case was a Pseudomonas keratitis in a blind eye, which rapidly resolved and has remained stable. Four patients required additional surgeries, which included 3 corneal transplants, 2 cataract extractions, and 1 glaucoma aqueous shunt. CONCLUSION: Continuous irrigation of the eye can improve scleral penetration of antibiotics. Subpalpebral lavage provides continuous irrigation and may be effective in the treatment of infectious scleritis of a variety of etiologies.
A 46-year-old man developed surgically induced necrotizing scleritis (SINS) 7 months following uneventful primary bare sclera pterygium excision. The patient underwent two scleral patch grafts for SINS but despite the use of cyclophosphamide and azathioprine, the scleral patch grafts failed within 1 month of surgery on both occasions. Tacrolimus was used for his third scleral patch graft. No recurrence of necrotizing scleritis was observed for 2 years. Tacrolimus may be considered as a valuable adjunctive immunosuppressant in the management of resistant necrotizing scleritis.
Most cases of non-necrotising scleritis can be successfully treated with non-steroidal anti-inflammatory drugs. If these are ineffective, then high-dose systemic corticosteroids, with all their attendant side-effects, are usually required. We have used orbital floor injections of depot steroid in the management of nine patients with non-necrotising scleritis in an attempt to avoid the use of systemic steroids, or to allow the dose of steroids to be reduced while maintaining disease control. A temporary reduction in inflammation was achieved in all cases, which allowed the use of systemic steroids to be avoided altogether in two patients and delayed in the others. Non-steroidal anti-inflammatory drugs and systemic corticosteroids remain the mainstay of treatment for non-necrotising scleritis, but orbital floor injections may be a useful adjunct in certain cases.
BACKGROUND: Surgically induced necrotizing scleritis is primarily managed with immunosuppressive regimens. Such treatments can alter inflammatory cytokines and products such as tumor necrosis factor-alpha (TNF-alpha) and matrix metalloproteinase-9 (MMP-9), which are an important proinflammatory cytokine and a tissue-degrading protease involved in necrosis of some ocular tissues. OBJECT: To evaluate TNF-alpha and MMP-9 in the tear or sclera of surgically induced necrotizing scleritis. METHODS: After tear collection from healthy and diseased eyes of 2 patients, immunoblot analysis using monoclonal antibody specific for human TNF-alpha and MMP-9 was performed. In another patient, scleral tissues were obtained during scleral allograft surgery, and the same immunoblot analysis was done. RESULTS: The level of TNF-alpha and MMP-9 was increased in tear fluid from patients compared to that of control volunteers and returned to the control level after treatment. The diseased sclera showed an increased expression of MMP-9 compared to that of the normal donor sclera. CONCLUSIONS: TNF-alpha and MMP-9 may suggest disease activity of surgically induced necrotizing scleritis and can be altered by proper immunosuppressive treatment.
We retrospectively examined 52 patients (60 eyes) with episcleritis and scleritis. Their mean ages were 57 and 47 years, respectively. Slight female predominance was noted. Most patients had unilateral involvement. The ocular symptoms at the initial visit were redness and ocular pain. Some eyes with scleritis were complicated with uveitis, corneal lesions, and elevated intraocular pressure. In most affected eyes, corticosteroid eyedrops alone resolved the disease. We believe that in addition to ophthalmological examinations, standard physical and specific laboratory investigations should be performed in patients with episcleritis and scleritis.
PURPOSE: To evaluate ocular features of nodular posterior scleritis simulating choroidal melanoma. METHODS: A 60-year old woman presented with blurred vision in her right eye of two weeks duration. On examination she had a mild right-globe proptosis with an episcleral nodular mass as well as a large elevated nonpigmented choroidal mass involving the nasal quadrant. RESULTS: A and B-scan ultrasonography showed a medium to high-reflective solid choroidal mass. MRI demonstrated a bi-convex mass in the medial aspect of the right globe with signal characteristics compatible with choroidal melanoma. Biopsy of the extraocular lesion demonstrated chronic inflammatory cell infiltrate suggestive of posterior scleritis. She responded to corticosteroid therapy. On evaluation 41 months later she was noted to have a similar choroidal mass in the left eye. CONCLUSIONS: The physician should be aware of the clinical manifestations and diagnostic hall marks of nodular posterior scleritis in order to differentiate this inflammatory process from choroidal melanoma.
Approximately 50% of patients with scleritis are known to be associated with systemic connective tissue diseases or vasculitic diseases such as rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and systemic lupus erythematosus. The patients with scleritis in association with Behcet's disease (BD) have been rarely described in the literature. We report a 46-year-old female patient with BD who developed the nodular scleritis.
Necrotizing scleritis with inflammation of the right eye developed after bilateral eye muscle surgery for thyroid ophthalmopathy. Debilitating pain, delay in onset, and involvement of the sclera distinguish this condition from anterior segment ischemia. The surgery may have acted as a nonspecific trigger in an eye at risk for scleritis. Necrotizing scleritis has occurred infrequently after other types of eye surgery but, to our knowledge, has not been previously reported as a complication of eye muscle surgery.
PURPOSE: To study the clinical features, management guidelines, and the course of the disease in six patients with posterior scleritis. SUBJECTS AND METHODS: Six patients with posterior scleritis were enrolled in this retrospective study. All patients underwent a complete ophthalmic examination, fluorescein angiography, and B-scan ultrasonography. Systemic evaluation included physical examination and laboratory screening investigations. Medical management included systemic indomethacin (two cases), oral steroids (two cases), intravenous pulses of methylprednisolone followed by tapered oral steroids (two cases). One patient underwent immunosuppressive therapy (azathioprine) because of steroid dependence. RESULTS: The main presenting symptoms were pain (six cases) and decreased vision (four cases). Chorioretinal changes included chorioretinal folds (six cases), retinal folds (five cases), optic disc edema (five cases), serous retinal detachment (two cases), and a subretinal mass (one case). Ultrasonography disclosed sclerochoroidal thickening in all cases and retrobulbar edema in three cases. Systemic evaluation was unremarkable in five cases and revealed Behçet's disease in one case. All patients showed a good response to medical treatment. CONCLUSION: Posterior scleritis has protean manifestations and can be easily overlooked. This diagnosis must be considered in all inflammatory and painful ocular disorders with no obvious etiology. B-scan ultrasonography is the most useful diagnostic tool in such patients. The disease usually shows a good response to systemic anti-inflammatory therapy.