Nodular Scleritis Research Articles

Nodular scleritis and panuveitis with erythema elevatum diutinum

PurposeTo report a case of nodular scleritis and panuveitis associated with erythema elevatum diutinum, a rare immunocomplex-mediated skin disease. DesignObservational case report. MethodsA 22-year-old woman who was diagnosed with erythema elevatum diutinum developed nodular scleritis and panuveitis of the right eye. She had experienced peripheral ulcerative keratitis with corneal perforation. ResultsAll other known causes of nodular scleritis and panuveitis were investigated and ruled out. ConclusionsErythema elevatum diutinum should be considered as an underlying systemic disease associated with nodular scleritis and panuveitis as well as peripheral keratitis.

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.

Giant Nodular Posterior Scleritis Simulating Choroidal Melanoma and Birdshot Retinochoroidopathy

A case of nodular posterior scleritis simulating both a choroidal tumor and birdshot chorioretinopathy is described. Fluorescein angiography, indocyanine green angiography, and ultrasound were performed. Results of laboratory tests were normal, including HLA-A29, erythrocyte sedimentation rate, rheumatoid factor, and antinuclear antibodies. A chest x-ray was normal. A diagnosis of nodular posterior scleritis was made and the minimally symptomatic patient without ocular pain was observed unless symptoms progressed. In this case, certain clinical features led to the proper diagnosis.

Mycophenolate mofetil for the treatment of scleritis

Purpose To evaluate the usefulness of mycophenolate mofetil (MMF) (CellCept, Roche, Nutley, NJ), an antimetabolite immunosuppressant with a selective antiproliferative effect on T and B lymphocytes, for the treatment of scleritis. Design Retrospective, noncomparative case series. Participants Eight patients with scleritis treated with MMF in a tertiary referral center. Methods Review of the clinical records of patients evaluated at the National Eye Institute and prescribed MMF for the treatment of scleritis. Main outcome measures Control of scleral inflammation, the ability to taper prednisone or other immunosuppressive medications, and adverse events were recorded for each patient. Mycophenolate mofetil was determined to be an effective steroid-sparing agent if the daily prednisone dosage could be reduced by 50% or more and was determined to be an effective adjunctive immunosuppressive agent if the scleral inflammation was controlled in patients with active scleritis. Results Four patients with diffuse anterior scleritis, two with necrotizing scleritis with inflammation, one with nodular anterior scleritis, and one with nodular anterior and posterior scleritis, were identified. Mycophenolate mofetil administration was initiated as a steroid-sparing agent in 4 patients with controlled scleritis and as an additional immunosuppressive agent in 4 patients with active scleritis receiving concomitant treatment with prednisone and cyclosporine or methotrexate. In 3 of the 4 patients started on MMF as a steroid-sparing agent, the scleritis remained controlled while the prednisone dosage was tapered by more than 50%. One of the patients started on MMF as a steroid-sparing agent had recurrent scleritis, and each of the patients with active scleritis continued to have persistent scleral inflammation requiring additional immunosuppressive therapy. Adverse effects recorded in 4 of the 8 patients included a rash, gastrointestinal symptoms, paresthesias, and laboratory evidence of hepatotoxicity and renal toxicity. Conclusions Although MMF maybe be useful as a steroid-sparing agent, it was not effective as an adjunctive immunosuppressive agent in patients with active scleritis in our small, tertiary referral series. The adverse effects encountered with the use of MMF in this study cannot be attributed conclusively to MMF and are more likely complications of the multiagent systemic immunosuppressive therapy required for the treatment of recalcitrant scleritis.

Indication and effect of immunosuppression in patients with scleritis

The value of immunosuppressive drugs for the therapy of scleritis patients is unclear. The authors investigated the indications and effects of immunosuppression in a group of patients with scleral inflammation. Retrospective study of patients treated for scleritis (n = 87) or episcleritis (n = 18). The demographic factors, clinical symptoms, visual outcome, course of inflammation, ocular complications resulting from inflammation, topical and systemic antiinflammatory medication, and associated systemic diseases were analysed. Only one patient with episcleritis, but 37 with scleritis presented with ocular complications (P = 0.003). The vision was impaired in 15 patients with scleritis, but not in episcleritis patients (P = 0.022). In the group of patients with episcleritis, only those with frequent relapses required more than topical antiinflammatory drugs, especially systemic non-steroidals. In contrast, systemic therapy was indicated in all of the scleritis patients. Ocular complications were found more often in patients with necrotising (n = 7/10) or posterior scleritis (n = 10/11) than in those with diffuse (9/39) or nodular (11/27) scleritis. Compared with the other patients, associated systemic autoimmune diseases were more common in patients with necrotising scleritis (P = 0.03). The need for immunosuppression was associated with vision-threatening complications (glaucoma, uveitis, peripheral ulcerative keratitis) (P < 0.01), systemic autoimmune disease, and necrotising and posterior form of scleritis (P < 0.01). Quiescence of scleritis was obtained in 59 of the scleritis patients, and improvement of inflammation was achieved in further 21. In 26 patients, scleritis did not improve with systemic steroid or non-steroidal treatment, but with immunosuppression. Scleritis is often associated with life-threatening systemic diseases and vision-threatening ocular complications. In patients with severe scleritis, especially with the posterior and necrotising form, improvement can often be achieved with immunosuppression.

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. © 2003 by the American Academy of Ophthalmology.

Ocular and extracutaneous involvement in pyoderma gangrenosum

Purpose To report an unusual presentation of pyoderma gangrenosum with ocular and extracutaneous involvement. Design A single, interventional case report. Methods A 28-year-old female presented with nodular scleritis in the right eye and left orbital inflammation lasting 1 year. The patient initially responded to systemic steroids, but then ocular inflammation became more severe, associated with onset of fever, myalgias, and elevation of the erythrocyte sedimentation rate, accompanied by formation of multiple cutaneous abscesses. Main outcome measures Ocular and systemic disease control. Results The diagnosis of pyoderma gangrenosum was made 1 year after the occurrence of ocular symptomatology, when the patient presented with ocular and skin lesions and abscesses in the spleen and liver. Conclusions Pyoderma gangrenosum is a rare, necrotizing, noninfectious ulcerating skin disease. Ocular and extracutaneous involvement rarely occur, but the disease should be included in the differential diagnosis of scleritis and orbital inflammation.

O emprego da biomicroscopia ultra-sônica no diagnóstico e evolução clínica dos diferentes tipos de esclerite anterior

Objetivo: Correlacionar achados da biomicroscopia ultra-sonica (UBM) com tipos de esclerite anterior. Metodos: Foram avaliados seis pacientes encaminhados ao Setor de Ultra-som do Departamento de Oftalmologia da Universidade Federal de Sao Paulo - Escola Paulista de Medicina, com suspeita clinica de esclerite anterior, utilizando-se o ultra-som de alta frequencia (transdutor de 50 MHz) para elucidacao das alteracoes histopatologicas encontradas na esclerite anterior. Resultados: Pacientes com esclerite nodular apresentaram lesao escleral bem delimitada, homogenea, hiporrefletiva, com espessamento localizado e hiporrefletividade dos tecidos adjacentes. Pacientes com esclerite difusa apresentaram espessamento escleral heterogeneo, de aspecto moteado. Pacientes com esclerite necrotizante apresentaram perda de tecido com afinamento escleral e alteracoes vitreas adjacentes. Conclusao: A biomicroscopia ultra-sonica e excelente metodo nao-invasivo para se diferenciar os tecidos oculares acometidos durante o processo de esclerite anterior, auxiliando o profissional no diagnostico e, consequentemente, no tratamento das lesoes.

Episcleritis and scleritis: clinical features and treatment results

PURPOSE: To evaluate the clinical experience with episcleritis and scleritis at a tertiary care eye center. METHODS: Retrospective chart review. RESULTS: One hundred thirty-four patients with scleral inflammation were seen over a 12-year period. Thirty-seven patients had episcleritis, and 97 patients had scleritis. Ocular complications occurred in only 13.5% of patients with episcleritis but in 58.8% of patients with scleritis ( P < .0001). No patient with episcleritis had a decrease in visual acuity, whereas 15.9% of patients with scleritis did. Only 16.7% of patients with episcleritis required more than topical corticosteroids for treatment, and these patients required oral nonsteroidal anti-inflammatory drugs. Conversely, 30.4% of patients with scleritis required nonsteroidal anti-inflammatory drugs, 31.9% oral prednisone, and 26.1% systemic immunosuppressive drugs ( P < .0001). Necrotizing scleritis and posterior scleritis more often were associated with ocular complications, occurring in 91.7% and 85.7%, respectively, than were diffuse anterior scleritis and nodular anterior scleritis ( P = .020). Patients with necrotizing scleritis and posterior scleritis were more likely to be treated with oral corticosteroids or immunosuppressive drugs (90% and 100%, respectively) than were patients with diffuse anterior scleritis and nodular anterior scleritis (56.4% and 21.4%, respectively, P = .002). CONCLUSIONS: Scleritis is a severe ocular inflammation, often associated with ocular complications, and nearly always treated with systemic medications. Nearly 60% of these patients will need oral corticosteroids or immunosuppressive drugs to control the disease.

Anterior segment indocyanine green angiography in scleral inflammation

To assess the feasibility of using indocyanine green angiography in scleral inflammation; to define the characteristic patterns of the anterior segment vasculature for this anterior segment disease; and to correlate the findings with those of anterior segment fluorescein angiography. Anterior segment fluorescein and indocyanine green digital angiography were used to evaluate scleral inflammation in 3 patients with diffuse episcleritis, 2 patients with nodular episcleritis and 5 patients with nodular scleritis. Angiograms from both techniques were assessed based on the time for complete disappearance of the dye and the type of leakage. Both fluorescein and indocyanine green dye appeared in vessels at approximately the same time, but, whereas fluorescein had disappeared completely from vessels by the 70th second, indocyanine green was observed within vessels for up to 23 min. Leakage of fluorescein occurred in all patients with diffuse episcleritis but staining occurred in only 1 patient with nodular scleritis. No leakage of indocyanine green, or staining, occurred in patients with diffuse episcleritis. However, leakage of indocyanine green was apparent in all nodular episcleritis and scleritis patients, staining the nodules in patchy form. The longer transit time, as well as leakage and staining patterns due to its protein-binding properties, make indocyanine green angiography a potentially useful technique in the investigation of patients with scleral inflammation, and in distinguishing diffuse from nodular variants. Further studies are necessary to correlate staining patterns with clinical findings.

Recurrent nodular scleritis associated with varicella zoster virus

PURPOSE: To describe a case of recurrent nodular scleritis that was apparently caused by reactivation of a varicella zoster virus infection. METHODS: Case report. Immunohistochemistry and polymerase chain reaction were used to detect viral antigen and DNA in the biopsy specimen of inflamed sclera of a patient with a history of recurrent nodular scleritis. RESULTS: Immunohistochemistry and polymerase chain reaction disclosed varicella zoster virus protein and DNA in the biopsy specimen of inflamed sclera from the patient. The scleritis resolved with oral famciclovir therapy. CONCLUSION: Reactivation of latent varicella zoster virus and direct viral invasion of sclera apparently caused the recurrent anti-inflammatory resistant scleritis in our patient.

Ultrasound biomicroscopy in scleritis

Objective This study aimed to investigate the usefulness of ultrasound biomicroscopy (UBM) for detecting and following up scleritis and episcleritis. Design The study design was a case series. Participants Patients with scleral inflammatory diseases (n = 16) were examined. Intervention Patient-reported problems and slit-lamp and UBM (50-MHz transducer) findings were compared retrospectively for signs of scleral inflammation. Main outcome measures Thickness, reflectivity, and homogeneity of the sclera and episclera were the criteria for discriminating between the different types of scleritis with the UBM technique. Results Scleral disease was associated with Wegener disease (n = 3), Cogan disease (n = 1), Hashimoto thyroiditis (n = 1), myositis (n = 1), or panuveitis (n = 1). Initial slit-lamp evaluation showed episcleritis (n = 3), diffuse scleritis (n = 9), nodular scleritis (n = 3), or necrotizing scleritis (n = 1). By means of UBM analysis, the diagnosis of episcleritis or scleritis was in agreement with the slit-lamp findings in 2 of 3 and 6 of 13 cases, respectively. In contrast to the slit-lamp diagnosis, UBM studies excluded scleritis in one patient, disclosed necrosis in four patients with scleritis, and detected nodular scleritis patterns in two further patients with diffuse scleritis. The determination of complete remission, improvement, or progression of disease by slit-lamp and UBM evaluation was in agreement in 11 of the 14 patients examined. However, UBM was superior to slit-lamp examination with respect to detecting scleral necrosis, scleral thinning, or the nodular type of scleritis. Conclusions The findings indicate that UBM is helpful in rapidly differentiating scleritis from severe episcleritis, detecting the diverse scleritis types with high accuracy, disclosing minimal disease progression, and judging treatment efficacy.

An Analysis of Therapeutic Decision for Scleritis

To compare the long-term efficacy of different systemic therapeutic regimens for patients with noninfectious anterior scleritis to establish guidelines for institution of therapy. Therapeutic failure of systemic nonsteroidal anti-inflammatory drugs (NSAIDs), systemic steroidal anti-inflammatory drugs, and systemic nonsteroidal immunosuppressive drugs was evaluated in 132 patients with noninfectious anterior scleritis (diffuse, nodular, or necrotizing types). In patients with diffuse scleritis, therapeutic failure for initial regimens occurred in 7% of patients treated with NSAIDs, in 16% of patients treated with steroids, and in 27% of patients treated with immunosuppressive drugs. In patients with nodular scleritis, therapeutic failure for initial regimens occurred in 9% of patients treated with NSAIDs, in 28% of patients treated with steroids, and in 25% of patients treated with immunosuppressive drugs. Addition or substitution of steroids or immunosuppressive drugs as second- or third-line therapies helped control the scleritis. In patients with necrotizing scleritis, therapeutic failure for initial regimens occurred in 100% of patients treated with NSAIDs, in 91% of patients treated with steroids, and in 26% of patients treated with immunosuppressive drugs. In patients with diffuse and nodular scleritis, NSAIDs should be the initial choice; in case of therapeutic failure, steroids should be added or substituted as second-line therapy, tapering and discontinuing them as soon as possible while maintaining remission with continued NSAIDs; in case of therapeutic failure, immunosuppressive drugs should be added or substituted as third-line therapy. In patients with necrotizing scleritis, immunosuppressive drugs should be the initial choice.

Scleritis and Streptococcus pneumoniae

We retrospectively review our experience with four patients with Streptococcus pneumoniae scleritis. Two of the patients had been exposed to beta irradiation after pterygium removal 4 and 13 years previously. One patient had a 3-year history of chronic anterior nodular scleritis, and one patient had severe rheumatoid arthritis. All were treated with intensive i.v. and topical fortified antibiotics. In two of the cases, the infection was controlled and visual acuity returned to 20/30 and 20/60. In one patient, infectious scleritis progressed to endophthalmitis. This eye ultimately became phthisical and required enucleation because of chronic pain. In the remaining patient, infectious scleritis led to perforation, which required a corneal-scleral patch graft. This patient had a final visual acuity of counting fingers. An infectious etiology should be suspected in cases of necrotizing scleritis associated with a purulent discharge, and appropriate smears and cultures should be obtained. Infectious scleritis can be caused by streptococcal organisms. Appropriate topical and intravenous antibiotic treatment is effective in some cases.

Progression of Scleral Disease

The clinical features of 290 patients with scleral inflammation were reviewed to determine whether a classification based on the anatomical site and clinical appearance of the disease at presentation reflected its natural history. The authors' results confirm that the majority of patients remain in the same clinical category throughout the course of their disease. Of the 104 (35.9%) patients who experienced a recurrence of their disease, only 12 had progressed from diffuse to nodular disease, and 10 patients who originally had nodular disease developed sclera) necrosis. Patients with necrotizing scleritis were older than patients in the other groups and more frequently had an associated systemic disease than patients with either diffuse or nodular disease; necrotizing scleritis was the most difficult disease to treat. Diffuse anterior scleritis had a lower incidence of visual loss (9%) than either nodular scleritis (26%) or necrotizing disease (74%), and, therefore, the authors consider nodular scleritis a disease of intermediate severity between diffuse scleritis and necrotizing disease. In this series, 12% of patients presented with posterior scleritis, and visual loss was most frequent in this group (84%).

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.

Treatment of Scleritis With Combined Oral Prednisone and Indomethacin Therapy

Six patients with diffuse or nodular scleritis that did not respond satisfactorily to oral indomethacin or prednisone used alone required a combination of these two drugs to achieve a complete clinical response. In our patients, the total daily doses of prednisone when used alone ranged from 40 to 100 mg, and the total daily doses of indomethacin when used alone ranged from 100 to 200 mg. Use of combination therapy with prednisone in total daily doses ranging from 10 to 60 mg as well as indomethacin in total daily doses ranging from 50 to 150 mg resulted in a complete clinical response. The corticosteroid-saving effect of nonsteroidal anti-inflammatory agents allowed the use of lower doses of corticosteroids, thereby decreasing the risk of serious systemic side effects.

Scleritis, Pericarditis, and Aortic Insufficiency in a Patient with Rheumatoid Arthritis

A 55-year-old black man developed anterior nodular scleritis in his left eye in November 1981. He had no symptoms of systemic disease, and initial laboratory tests revealed only a positive rheumatoid factor. Fourteen months later he presented with pericarditis and aortic insufficiency requiring aortic valve replacement. Examination of excised valvular and pericardial tissue showed changes compatible with rheumatoid disease. Shortly after the surgery he developed florid rheumatoid arthritis. The clinical course of this patient illustrates how scleritis can be the initial sign of severe systemic disease.

Bilateral Necrotizing Scleritis

A 53-year-old woman whose history was noncontributory for rheumatoid arthritis and collagen disease had bilateral necrotizing inflammation of the sclera. The left eye had necrotizing nodular scleritis and the right eye, massive granuloma of the sclera. In the right eye the orbital adnexae were also involved by granulomatous infiltration and developed a mass of fibroadipose tissue simulating an orbital tumor within a week after enucleation.

Scleritis and episcleritis.

The data from 159 patients (217 eyes) with episscleritis and 207 patients (301 eyes) with scleritis have been investigated in detail and the results analysed with the help of a computer. Of these patients, 91 per cent were followed-up during a period of one to eight years. A new classification is presented which is as follows: Episcleritis (217 eyes) Simple episcleritis (170 eyes) Nodular episcleritis (47 eyes) Scleritis (301 eyes) Diffuse anterior scleritis (119 eyes) Nodular anterior scleritis (134 eyes) Necrotizing scleritis (42 eyes). Of these, 13 were regarded as scleromalacia perforans. Posterior scleritis (6 eyes) The diagnosis is based on an exact clinical examination which is fully described. Episcleritis has been shown to be a benign recurring condition, a mild keratitis being the only occasional complication. Episcleritis does not progress to scleritis, except in the case of herpes zoster which sometimes starts as an episcleritis with the vesicular stage of the eruption, to reappear three months later as a scleritis in the same site. No clear conclusions could be drawn as to the aetiology of episcleritis.