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Scleritis and IgA nephropathy.

Follow-up studies on 113 patients with various types of primary glomerular diseases were performed for one to 33 months to determine the clinical spectrum of primary glomerulonephritis. Of those studied, six patients exhibited scleritis. All of these six patients with scleritis were identified as having "IgA nephropathy." None of the patients other than those with IgA nephropathy showed scleritis during the study period. It is suggested that some autoimmune mechanisms similar to the manifestation of IgA nephropathy may be involved in the development of scleritis.

Adult↗

Immunopathological findings in posterior scleritis.

Posterior scleritis can present with a variety of symptoms, and its clinical diagnosis is therefore difficult. Little is known about the pathogenesis and the cellular effector mechanisms. This case report presents the immunopathological findings of posterior scleritis in the enucleated eye of a 28-year-old female with no known underlying disease. The cells infiltrating the scleral fibers consisted predominantly of T cells. Many of them were CD4 cells. Clusters of T and B cells were found in perivascular areas. No signs of primary vasculitis were seen. The cellular infiltrate in posterior scleritis shows features compatible with a T-cell-mediated (autoimmune) disorder.

Adult↗

[Posterior scleritis in Wegener disease].

BACKGROUND: Wegener's granulomatosis is a focal necrotizing granulomatous vasculitis of unknown etiology. It manifests initially as localized inflammation, usually of the lungs, and preceeds to the state of generalisation which can be life-threatening due to renal failure. Ocular and orbital involvement occurs in 28-58% of patients with Wegener's granulomatosis. More often, the anterior segments of the eye are involved whereas inflammation of the posterior segments is quite uncommon. PATIENTS AND METHODS: We report a case of posterior scleritis as the initial manifestation of Wegener's granulomatosis in a 65-year-old woman. RESULTS: Posterior scleritis responded favourably to therapy with systemic steroids and cyclophosphamide. CONCLUSIONS: Wegener's granulomatosis can have a poor prognosis if not diagnosed and treated in time. Knowledge of posterior scleritis as a possible presenting symptom may therefore facilitate early diagnosis and treatment and prevent progression of the disease.

Aged↗

[Cyclosporin A therapy in severe anterior scleritis. 5 severe courses without verification of associated systemic disease treated with cyclosporin A].

BACKGROUND: Patients with severe scleritis who do not respond to high-dose corticosteroid therapy, or who require a daily corticosteroid maintenance dose higher than 30 mg prednisone should be treated by other immunosuppressants. PATIENTS AND METHODS: In five patients with various types of severe anterior scleritis a long-term high-dose steroid treatment failed to control scleral inflammation. They therefore received cyclosporin (CsA). Follow-up was 16-26 months. RESULTS: Scleral inflammation and ocular complications were controlled in all patients by a regimen of systemic CsA combined with a low maintenance steroid dose below the Cushing threshold. We observed no side effects under CsA serum levels of 120-150 ng/ml. In only one patient was scleral inflammation totally and lastingly eliminated. CONCLUSIONS: Systemic CsA therapy is of definite therapeutic value in the symptomatic management of steroid refractory severe anterior scleritis without associated systemic disease. Complete healing, however, is achieved only in a minority of cases.

Adult↗

Evaluation of subconjunctival triamcinolone for nonnecrotizing anterior scleritis.

PURPOSE: Subconjunctival corticosteroid injection (SCI) for nonnecrotizing anterior scleritis remains controversial, partly because long-term follow-up is not available. This study documents the efficacy and adverse effects of SCI. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Thirty-eight eyes of 35 patients with nonnecrotizing, noninfectious anterior scleritis resistant to prior systemic or local therapy. INTERVENTION: Subconjunctival triamcinolone acetonide injection. MAIN OUTCOME MEASURE: Persistence or resolution of signs and symptoms of anterior scleritis and development of complications. RESULTS: Thirty-six of 38 eyes had complete resolution of signs and symptoms within 6 weeks of SCI. Fifteen eyes had follow-up of > or =30 months. There were no instances of scleral melting or perforation; adverse events included subconjunctival hemorrhage (5 patients), transient ocular hypertension without evidence of glaucoma (4 patients), cataract (2 patients), and glaucoma (2 patients). Subconjunctival corticosteroid injection resulted in reduced dependence on systemic medications. CONCLUSION: Subconjunctival corticosteroid injection in eyes that failed other therapies is effective, reduces dependence on systemic medications, and did not result in scleral necrosis over a median follow-up period of 29 months.

Adolescent↗

Acute posterior multifocal placoid pigment epitheliopathy and scleritis in a patient with pANCA-positive systemic vasculitis.

PURPOSE: To present acute posterior multifocal placoid pigment epitheliopathy and scleritis occurring in association with pANCA (perinuclear antineutrophil cytoplasmic antibody)-positive systemic vasculitis. DESIGN: Interventional case report. METHODS: A 69-year-old woman developed bilateral acute posterior multifocal placoid pigment epitheliopathy concurrent with rapidly progressive glomerulonephritis and bronchioalveolitis in both lungs. RESULTS: The patient showed a high titer of pANCA (myeloperoxidase-ANCA). She then developed scleritis in the left eye and multiple gastric ulcers. CONCLUSION: Acute posterior multifocal placoid pigment epitheliopathy and scleritis in this patient are manifestations that are part of pANCA-positive systemic vasculitis. This association further supports that acute posterior multifocal placoid pigment epitheliopathy is caused by vasculitic inflammation of the choriocapillaris.

Acute Disease↗

Nocardia scleritis.

PURPOSE: To describe a case of Nocardia scleritis, an unusual ocular infection. METHODS: Case report and review of pertinent literature. RESULTS: An 83-year-old man with leukocytoclastic vasculitis was initially examined for infectious necrotizing scleritis after explantation of an extruded scleral buckle. The patient was successfully treated with sulfonamides. CONCLUSIONS: Nocardia asteroides may cause infectious scleritis in the absence of cataract surgery or trauma. Treatment with sulfonamides can result in a satisfactory outcome.

Aged↗

Posterior scleritis with annular ciliochoroidal detachment.

PURPOSE: We studied an unusual case of posterior scleritis in a patient with sarcoidosis. METHOD: The medical record was reviewed for clinical manifestation, course, and tests performed, including laboratory evaluations, fluorescein angiography, and ultrasonography. RESULTS: The patient had posterior scleritis and unilateral angle-closure glaucoma caused by an annular ciliochoroidal detachment. Sarcoidosis was confirmed by biopsy of an enlarged parotid gland. CONCLUSION: The mechanism of angle-closure glaucoma may not be clinically or echographically apparent for a week or more in patients who develop annular ciliochoroidal detachment. We also found an unusual association of sarcoidosis and annular ciliochoroidal detachment secondary to posterior scleritis.

Adrenergic beta-Antagonists↗

Scleritis and Wegener's granulomatosis in children.

We treated two children with scleritis (one unilateral, one bilateral), in whom Wegener's granulomatosis was diagnosed on the basis of pathologic changes in respiratory tract mucosa. Both patients were girls, 13 and 14 years of age, respectively. One patient had otitis media and a nodular scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate and microscopic hematuria. A biopsy of the sinus confirmed the diagnosis of Wegener's granulomatosis. The second patient had fever, arthralgias, a nonproductive cough, and bilateral scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate, positive test results for rheumatoid factor, and bilateral pulmonary nodules on chest x-ray. Open-lung biopsy confirmed the diagnosis of Wegener's granulomatosis. Both patients responded well to treatment with a combination of prednisone and cyclophosphamide.

Adolescent↗

Necrotizing scleritis of scleral flaps after transscleral suture fixation of an intraocular lens.

A 56-year-old woman with rheumatoid arthritis underwent intracapsular cataract extraction and sulcus fixation of an intraocular lens using transscleral fixation sutures buried under partial-thickness scleral flaps. Necrotizing scleritis confined to the area of the scleral flaps developed one month postoperatively, resulting in exposure and loosening of one fixation suture and lens implant decentration. The scleritis responded to systemic prednisone and cyclophosphamide treatment, with healing in two weeks. The final visual acuity was 20/30. Surgical trauma may stimulate local intravascular immune complex deposition and initiate the inflammatory process, thereby leading to necrotizing scleritis. This process should be considered when contemplating the use of scleral flaps in patients with collagen vascular disease and systemic vasculitis.

Arthritis, Rheumatoid↗

Indocyanine green angiographic features in posterior scleritis.

PURPOSE: To determine choroidal involvement in posterior scleritis by examining indocyanine green angiographic features. METHODS: Indocyanine green angiography was performed according to a standard uveitis angiographic protocol in eight consecutive patients with posterior scleritis. Indocyanine green angiography data were compared to fundus color photographs, red-free photographs, and fluorescein angiography. RESULTS: The principal indocyanine green angiographic feature was diffuse zonal choroidal indocyanine green hyperfluorescence in the intermediate (+/-10 minutes) and late (+/-40 minutes) phases of angiography present in all eight patients who regressed in response to anti-inflammatory therapy. In four patients (two with massive subretinal exudation), additional fluorescing pinpoints were present in the zonal hyperfluorescent areas. Additional features included irregular delayed choroidal perfusion (five of eight patients)--irregularly distributed dark dots that were present up to the intermediate phase and becoming isofluorescent in the late phase that gave a mottled aspect to the choroid--and enlargement of draining choroidal veins. In bilateral patients, clinical features and indocyanine green angiography signs were roughly symmetric. CONCLUSIONS: In posterior scleritis, indocyanine green angiography allowed us to identify areas of choroidal hyperfluorescence, possibly indicating areas of maximal inflammatory activity, and demonstrated regression of hyperfluorescence in response to therapy. Indocyanine green angiography was useful in assessing the extent of choroidal involvement and will probably serve as one of the follow-up parameters for disease evolution and response to therapy.

Adult↗

Orbital lymphoma misdiagnosed as scleritis.

OBJECTIVE: To describe two patients in whom orbital lymphoma was misdiagnosed and treated as scleritis. DESIGN: Two case reports with clinicopathologic correlation. METHODS: Each patient underwent clinical, radiologic, and histologic assessment. MAIN OUTCOME MEASURE: Histologic confirmation of the diagnosis of orbital lymphoma. RESULTS: The two patients who had been diagnosed and treated as having scleritis were found to have orbital lymphoma. CONCLUSION: The differential diagnosis for scleritis should include lymphoma.

Aged↗

Multifocal nodular episcleritis and scleritis with undiagnosed Hodgkin's lymphoma.

PURPOSE: To report the case of a patient with undiagnosed Hodgkin's lymphoma who presented with coexistent unilateral nodular episcleritis and scleritis. DESIGN: Interventional case report and literature review METHODS: Review of clinical history, laboratory findings, histology of episcleral and cervical lymph node biopsies, and follow-up. RESULTS: A 20-year-old female presented with a 5-month history of redness and pain in her left eye, with associated symptoms of dyspnea, malaise, and fever. The patient was found to have multifocal nodular episcleritis and scleritis that was not responsive to topical steroids or systemic nonsteroidal anti-inflammatory treatment. Laboratory tests subsequently revealed evidence of systemic inflammation, and radiologic studies showed extensive mediastinal and cervical adenopathy. A cervical lymph node biopsy showed Reed-Sternberg cells and a chronic lymphocytic infiltrate consistent with nodular sclerosing Hodgkin's lymphoma. Histopathologic analysis of an episcleral nodule revealed a necrotizing granuloma with vasculitis. Systemic chemotherapy was instituted for the Hodgkin's disease; this therapy abolished the nodular scleritis. CONCLUSIONS: This case raises the possibility of concurrent undiagnosed systemic vasculitis with only an ocular manifestation with Hodgkin's lymphoma, either as a coincidence or as a paraneoplastic syndrome. Moreover, it emphasizes the important role of tissue biopsy in establishing diagnosis and directing treatment.

Adult↗

Scleral grafting for necrotizing scleritis.

Although systemic immunosuppressive chemotherapy is effective in halting progressive necrotizing scleritis, the onset of its action may be too slow to prevent profound scleral thinning and/or traumatic or spontaneous perforation. Scleral homografts may be used to maintain the integrity of the globe until immunosuppressive drugs can take effect. The authors reviewed their experience with scleral homografts in 12 patients with progressive necrotizing scleritis; eight (all with autoimmune disease) had concomitant chemotherapy and four (two with autoimmune disease) did not. Grafts remained stable in patients receiving both surgical and drug therapy over a mean follow-up of 12 months. One graft melted after discontinuation of chemotherapy, but regrafting and renewed immunosuppression salvaged the eye. Grafts in two of the patients not initially given chemotherapy melted rapidly (within 14 and 45 days, respectively). Both eyes were salvaged by regrafting and/or addition of chemotherapy. Though rarely successful by itself against necrotizing scleritis, scleral grafting is a useful adjunct to chemotherapy.

Aged↗

[MALT-type B-cell lymphoma masquerading as scleritis or posterior uveitis].

PURPOSE: Diagnostic and therapeutic management of masquerade syndromes associated with MALT-type lymphoma. PATIENTS AND METHOD: We report the cases of three patients referred for acute or chronic severe loss of visual acuity. All of them suffered from uveitis or scleritis resistant to corticoid treatment. Each one had posterior uveitis associated with retinochoroidal infiltration, anterior scleritis associated with uveal effusion syndrome, or bilateral pan-uveitis associated with serous retinal detachment. Two patients had medical records of systemic malignancies. They underwent an extensive work-up. Search for an infectious, immune or malignant disease was negative but orbital imaging showed a posterior scleral thickening in all cases. RESULTS: Diagnosis of mucosa-associated lymphoid tissue (MALT) ocular lymphoma was assessed in all cases after scleral biopsy. Immunohistochemical analysis showed CD20(+) and CD5(-) scleral lymphocyte infiltrates. The three patients underwent chemotherapy associated with radiotherapy. Lesions were stabilized in the first case, whereas six lines of visual acuity improvement was noted in the second one, and full recovery was achieved after treatment in the third case. DISCUSSION: Clinical manifestations of this ocular lesion seem to be multiple, masquerading as different types of posterior uveitis or scleritis. In the three cases, we did found other systemic locations of MALT-type lymphoma. Diagnosis was mainly based on pathological analysis. Fast visual recovery in two cases demonstrated the importance of an early diagnosis and a specific treatment. All cases of chronic and corticoid-resistant posterior uveitis require a specialized radiological evaluation followed, if necessary, by multiple orbital biopsies.

Adult↗

Necrotizing scleritis after intraocular surgery associated with the use of polyester nonabsorbable sutures.

We present 2 elderly patients who developed necrotizing scleritis after cataract extraction in which the wound was sutured with polyester. In the first case, a 73-year-old woman who had right phacoemulsification via a limbal incision developed necrotizing scleritis 8 months after surgery. The eye became progressively painful and phthisical, necessitating enucleation. Microscopic examination of the enucleated globe showed a predominantly lymphocytic infiltration of the ocular tissues with no evidence of an infectious agent. In the second case, a 78-year-old woman had bilateral extracapsular cataract extraction through a limbal incision closed with a polyester suture. The patient presented 3 years later with bilateral necrotizing sclerokeratitis. No underlying systemic vasculitis or autoimmune condition was identified in either patient. To our knowledge, the association of necrotizing scleritis after intraocular surgery and polyester fiber suture material (Mersilene) has not been described.

Aged↗

Subretinal mass as a presenting sign of posterior scleritis: a case report.

We report a case of an unusual clinical presentation of posterior scleritis. A 40-year-old woman suffered from sudden blurred vision in her left eye for 1 day. She had no systemic or autoimmune diseases. Fundus examination revealed a subretinal mass (10.5 x 6 mm in basal diameter) in the nasal-superior quadrant extending from the optic disc to the periphery. Overlying retinal striae were obvious. Optic disc hyperemia and mild edema were noted. Fluorescein angiography did not show dye leakage or tumor-feeding vessels. Ultrasonography showed high internal reflectivity of the subretinal mass and thickened sclera with T-sign. Under the impression of posterior scleritis, corticosteroids and nonsteroidal anti-inflammatory drugs were given orally and topically. After 4 weeks, the subretinal mass disappeared without any sequelae. The patient recovered full vision. This case suggests that posterior scleritis is among the differential diagnoses for a fundus mass. There has been no other similar report in Taiwan.

Adrenal Cortex Hormones↗

Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis.

PURPOSE: The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. CASE REPORT/METHODS: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented. RESULTS: Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. The deafness had been due to inner ear failure, and the patient died of a cardiac failure. CONCLUSIONS: Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications.

Aged↗