Immunopathology of scleritis.

Conjunctival and scleral biopsies from 25 patients with necrotizing scleritis and 5 patients with recurrent nonnecrotizing scleritis were studied by histopathologic, immunofluorescence, and immunoperoxidase techniques. Vasculitis with fibrinoid necrosis and neutrophil invasion of the vessel wall was present in 75% of the scleral and 52% of the conjunctival specimens. Vascular immunodeposits were found in 93% of the scleral and 79% of the conjunctival tissue tested by immunofluorescence techniques. A dramatic increase in the number of inflammatory cells over normal controls was detected in both tissues by immunoperoxidase techniques. In the conjunctival epithelium, there were significantly more T-helpers, macrophages, and B cells. In the conjunctival substantia propria, there were significantly more T cells of all types, macrophages, and B cells. Likewise, scleral specimens showed an increase over controls of T cells of all types and macrophages. HLA-DR expression was dramatically increased in both tissues. Immune-complex-mediated vasculitis plays a pivotal role in the pathogenesis of necrotizing scleritis and recurrent nonnecrotizing scleritis. Induced HLA-DR expression on ocular nonimmune cells and T cell controlled responses also may participate.     

Posterior scleritis in children.

Posterior scleritis was diagnosed in four adolescent boys. These patients represented a distinct subgroup of patients with posterior scleritis that differed from the adult variant by gender, lack of systemic disease, and absence of the associated ocular findings often seen in the adult variant of the disorder. The four patients had diminished visual acuity, ocular pain, and exudative retinal detachments at initial examination. Fluorescein angiography demonstrated multiple pinpoint leaks at the level of the retinal pigment epithelium in three patients with late-phase patchy staining of a mass-like lesion in one patient. B-scan ultrasonography demonstrated choroidal and scleral thickening with increased acoustic density of the choroid in all patients. Systemic evaluation of these patients disclosed no underlying disease. Clinical signs and symptoms resolved in three of the patients after treatment with low-dose, orally administered corticosteroid or noncorticosteroid anti-inflammatory medication. High systemic doses of corticosteroid in combination with noncorticosteroid anti-inflammatory medication and local corticosteroid therapy was required to induce remission in one patient. All patients recovered good visual acuity.     

Cyclosporin therapy for severe scleritis.

To ascertain the efficacy of systemic cyclosporin therapy in the management of scleritis we performed an open, uncontrolled study of the use of this drug in severe refractory disease. Five of seven patients whose disease had previously been poorly controlled with a combination of corticosteroids and immunosuppressive drugs responded to cyclosporin therapy (10 mg/kg/day). Systemic side effects occurred in all but one patient, with tremor, hirsutism, hypertension, and raised serum creatinine being common. Recurrence of disease activity on decreasing the dosage of cyclosporin was frequent. The results indicate that cyclosporin is a useful additional drug in the treatment of severe scleritis.     

Toxoplasmic scleritis

Although toxoplasmosis is the most common infectious cause of posterior intraocular inflammation, it is rarely described in association with scleritis. The authors present five cases of toxoplasmosis with scleritis. Two of the five cases were diagnosed clinically and serologically as having toxoplasmosis. Their retinochoroiditis and scleritis responded well to medical therapy. Retinochroiditis and scleritis that was refractory to treatment developed in the other three patients, two of whom had been receiving immunosuppressive therapy for systemic diseases. Their therapeutic regimens did not include treatment for toxoplasmosis. All three eyes became blind and were enucleated. Results of pathologic examination of all three enucleated eyes showed Toxoplasma gondii in the retina. There was severe inflammation of the retina, choroid, and sclera. Toxoplasmosis should be considered in the clinical differential diagnosis of scleritis associated with retinochoroiditis, particularly in immunosuppressed patients.     

Masquerade scleritis

PURPOSE: To report two cases in which malignancy masqueraded as scleritis, delaying the diagnosis. METHODS: Two patients initially diagnosed and treated for unilateral scleritis were referred for management of persistent inflammation. Additional evaluation uncovered underlying malignant processes. RESULTS: The first patient presented with scleritis initially responsive to systemic corticosteroids, with relapse one month later. Upon referral, peripheral fundus examination revealed elevated lesions. Additional studies confirmed the diagnosis of choroidal melanoma. The patient was treated with proton-beam irradiation. The second patient developed necrotizing scleritis unresponsive to systemic steroids, methotrexate, and cyclophosphamide. A scleral biopsy disclosed an undifferentiated high-grade carcinoma, likely metastatic. Exenteration was performed. CONCLUSIONS: Scleritis can present a diagnostic challenge. It is often the sole initial manifestation of an occult systemic problem. Treatment-resistant scleritis should raise the suspicion of an infectious or malignant masquerade.     

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.     

Nocardial endophthalmitis: report of two cases studied histopathologically.

We report two cases of nocardial endophthalmitis. Case 1 is a 66-year-old man whose left eye was enucleated about one month after onset of decreased vision. Ophthalmoscopic examination disclosed multiple choroidal masses. Six weeks before the onset of ocular manifestations he had undergone a prolonged carotid endarterectomy with intraoperative complications. The source of the ocular infection was probably exogenous. Case 2 is a 49-year-old woman who had systemic sclerosis (scleroderma) with severe pulmonary insufficiency. She had received moderate doses of corticosteroids. Seventeen months after initiation of therapy she developed ocular manifestations leading to enucleation of the eye one month later. Histopathologically, the enucleated eyes in both cases showed numerous branching, Gram-positive, filamentous organisms involving mainly the plane of Bruch's membrane and the subretinal space. The nosological and microbiological aspects of nocardiosis are reviewed.     

Posterior scleritis

Posterior scleritis must be considered in the differential diagnosis of many ocular conditions, including angle closure glaucoma, choroidal folds, optic disk edema, circumscribed fundus mass, choroidal detachment, and exudative retinal detachment. Because it is rare, a high index of suspicion is necessary. Anterior scleritis, pain, or a history of collagen-vascular disease, when present, help to alert the clinician to the correct diagnosis. Posterior scleritis affects women more often than men, but annular ciliochoroidal effusion and choroidal folds are more common in men. Exudative macular detachment and a circumscribed fundus mass are more common in women. This paper reviews the world literature on posterior scleritis and describes findings in a series of 43 patients seen at Wills Eye Hospital. It stresses the clinical features and ancillary diagnostic tests that help to establish the diagnosis.     

Squamous cell carcinoma with necrotizing scleritis.

PURPOSE: To report on a case of limbal squamous cell carcinoma (SCC) with necrotizing scleritis in a young, previously healthy, white Australian male. METHODS: A 31-year-old man presented with a left limbal lesion intermittently causing a red eye and foreign body sensation. He had enjoyed surfing for many years. Repeat HIV tests were negative and the lesion was biopsied. RESULTS: Biopsy showed a well-differentiated SCC apparently arising in an intra-epithelial (in situ) carcinoma of the conjunctiva. The lesion was excised and a corneoscleral graft repair was performed. CONCLUSION: The present case highlights the potential for a significant increase in the prevalence of ocular surface neoplasia in healthy young people who have had excessive UV-B exposure.     

Angle-closure glaucoma secondary to posterior scleritis.

This paper reports 3 patients who presented with angle-closure glaucoma secondary to posterior scleritis. Differentiation from primary angle-closure glaucoma is important, as medical treatment is markedly different while surgical treatment is not required.