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Scleritis is a severe and painful ophthalmic disorder, in which a pathogenic role for collagen-directed autoimmunity was repeatedly suggested. We evaluated the presence of sclera-specific antibodies in a large cohort of patients with non-infectious scleritis. Therefore, we prospectively collected serum samples from 121 patients with non-infectious scleritis in a multicenter cohort study in the Netherlands. In addition, healthy (n = 39) and uveitis controls (n = 48) were included. Serum samples were tested for anti-native human type II collagen antibodies using a validated enzyme-linked immunosorbent assay (ELISA). Further, sclera-specific antibodies were determined using indirect immunofluorescence (IIF) on primate retinal/scleral cryosections. Lastly, human leukocyte antigen (HLA) typing was performed in 111 patients with scleritis. Anti-type II collagen antibodies were found in 13% of scleritis patients, in 10% of healthy controls and in 11% of uveitis controls (p = 0.91). A specific reaction to scleral nerve tissue on IIF was observed in 33% of patients with scleritis, which was higher than in healthy controls (11%; p = 0.01), but similar to uveitis controls (25%; p = 0.36). Reactivity to the scleral nerve tissue was significantly associated with earlier onset of scleritis (48 versus 56 years; p < 0.001), bilateral involvement (65% versus 42%; p = 0.01), and less frequent development of scleral necrosis (5% versus 22%; p = 0.02). HLA-B27 was found to be twice as prevalent in patients with scleritis (15.3%) compared to a healthy population (7.2%). In conclusion, scleral nerve autoantibody reactivity was more common in scleritis and uveitis patients in contrast to healthy controls. Further research is needed to characterize these scleral-nerve directed antibodies and assess their clinical value., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

The aim of this study was to alert the ophthalmic community to an atypical manifestation of ocular surface squamous neoplasia, which may delay diagnosis and treatment and result in a guarded visual prognosis and significant sequelae. A 61-year-old immunocompetent man presented with an initial diagnosis of necrotizing scleritis in the right eye for 3 months. He was treated with systemic prednisone but experienced persistent pain and low visual acuity. Conjunctival biopsy of the affected region confirmed the diagnosis of invasive ocular surface squamous neoplasia, which progressed with intraocular and orbital invasion; thus, exenteration was performed. Masquerade syndrome should be suspected in patients with nodulo-ulcerative lesions of the conjunctiva and sclera. This clinical can be more aggressive, with a greater likelihood of intraocular and orbital involvement. The earlier the diagnosis and treatment, the better the patient prognosis.

Purpose: To analyze the morphological outcomes of the posterior corneal opacity or "semilunar sign" in noninfectious anterior scleritis using multimodal imaging., Methods: This was a prospective observational case series. Patients with anterior scleritis from January 2018 to January 2019 were included. Clinical and demographic data were collected. Posterior cornea was visualized using the digital slit lamp photography (Elite, mega digital vision), spectral domain optical coherence tomography (MS39), and specular count analyzer (EM-3000). "Semilunar sign" was defined by the (1) presence of posterior corneal opacity, (2) concave semilunar pattern, (3) absence of blood vessels, and (4) normal anterior cornea. Incidence, clinical characteristics and significance, correlation with Mantoux sensitivity, and role of multimodal valuation were assessed., Results: Overall 76 eyes of 72 patients were recruited with anterior scleritis. Fifteen eyes of 11 patients (15.3%) presented with semilunar sign. The scleritis was both nonnecrotizing (n = 8) and necrotizing (n = 7). The semilunar configuration appeared as isolated (n = 9) and continuous lesion (n = 6). The extent was directly related to the scleral disease extent (P = 0.002). The mean thickness measured 212.5 ± 129.3 μm. The mean central endothelial cell density (ECD) was 2540.8 ± 351.7 cells/mm 2 , which was significantly higher than the involved peripheral cornea (P = 0.05). The mean surface area of the semilunar sign was 7.7 ± 5.2 mm 2 . There was no significant correlation between the opacity thickness and the best-corrected visual acuity (P = 0.895, r = -0.39), ECD (P = 0.52, r = -0.188), and Mantoux (P = 0.696, r =- 0.142)., Conclusion: Corneal semilunar sign of scleritis affected the peripheral cornea and caused no functional abnormality in early presentation. Multimodal analysis can aid in clinical assessment and severity., Competing Interests: None

Scleritis involves inflammation of the sclera, which constitutes 75% of the wall of the eye. This pathology is often seen as an ocular lesion associated with systemic inflammatory diseases. Severe types of scleritis such as posterior scleritis require urgent immunosuppressive treatments, including molecularly targeted therapies to avoid permanent visual impairment. Which molecules should be selected as targets has remained unclear. To clarify the pathogenesis of scleritis and propose appropriate target molecules for therapy, we have established novel animal model of scleritis by modifying the Collagen-II Induced Arthritis (CIA) model. Immunization twice with collagen II emulsified with complete Freund's adjuvant (CFA) caused arthritis and scleritis. The clinical appearance resembled human diffuse scleritis. Histopathological analysis suggested that macrophages, plasma cells, deposition of immune complexes, and growth of blood and lymphatic vessels are involved in the pathogenesis of CIA-associated scleritis. In addition, we analysed the background diseases of posterior scleritis and responses to molecularly targeted therapies as a case series study. We inferred from both the animal model and case series study that targets should not be T cells, but factors inhibiting macrophage activity such as tumor necrosis factor (TNF) and interleukin (IL)-6, and molecules suppressing antibody-producing cells such as CD20 on B cells should be targeted by molecularly targeted therapies.

Purpose: To share our initial experience with the innovative use of topical erythropoietin for the treatment of necrotizing scleritis manifesting immediately after pterygium excision surgery., Methods: This study enrolled 3 patients who developed necrotizing scleritis immediately after undergoing pterygium excision. All cases with pterygium were primary, and topical mitomycin C and conjunctival autografts were used at the time of surgery. Noninvasive therapy that included ophthalmic lubricants and topical and systemic corticosteroids failed to improve the avascular scleral lesions. The patients were prescribed erythropoietin-containing drops (3000 U/mL) every 6 hours in addition to topical antibiotics and lubricant. The effect of topical erythropoietin on the healing process of avascular scleral lesions was investigated, and its ocular and systemic side effects were evaluated., Results: The mean age of the participants was 69.0 ± 14.8 years, and 2 of the 3 eyes belonged to male subjects. The time between pterygium surgery and presentation to our clinic was 33.0 ± 14.7 days. There were no infectious causes or underlying systemic diseases in any of the cases. After treatment with topical erythropoietin for an average of 34.3 ± 20.3 days, the lesions were completely vascularized in all 3 eyes without any ocular or systemic adverse effects. The patients were followed up for an average of 126 ± 94 days after discontinuation of erythropoietin. There was no evidence of recurrence during the last examination in any of the eyes., Conclusions: Topical erythropoietin might be a safe and an effective method for treating cases of necrotizing scleritis that manifests immediately after pterygium surgery., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

The onset of scleral necrosis after ocular surgery may have catastrophic ocular and systemic consequences. The two most frequent surgeries causing surgically-induced scleral necrosis (SISN) are pterygium excision and cataract extraction. Several pathogenic mechanisms are involved in surgically induced scleral necrosis. All of them are poorly understood. Ocular trauma increasing lytic action of collagenases with subsequent collagen degradation, vascular disruption leading to local ischemia, and immune complex deposition activating the complement system represents some of the events that lead to scleral necrosis. The complex cascade of events involving different pathogenic mechanisms and the patient's abnormal immune response frequently leads to delayed wound healing that predisposes the development of scleral necrosis. The management of SISN ranges from short-term systemic anti-inflammatory drugs to aggressive immunosuppressive therapy and surgical repair. Therefore, before performing any ocular surgery involving the sclera, a thorough ophthalmic and systemic evaluation must be done to identify high-risk patients that may develop SISN., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Infectious scleritis is a rare but important cause of scleral inflammation. It is usually associated with an underlying ocular (prior ocular surgery or trauma) or systemic risk factor. A 53-year-old apparently systemically healthy woman presenting with spontaneous-onset pain, redness and watering in the left eye for 10 days was diagnosed with culture-proven Pseudomonas aeruginosa anterior scleritis. However, she was non-responsive to organism-sensitive antibiotics and scleral graft was performed twice, which showed graft re-infection. On repeated extensive systemic evaluations, the patient was diagnosed with biopsy-proven granulomatosis with polyangiitis (GPA). The patient was started on mycophenolate mofetil for both induction and maintenance phases and showed dramatic improvement with no recurrence till 1 year follow-up. High index of suspicion for autoimmune disorders, especially GPA, must be maintained for unilateral relentless infective scleritis masquerading as autoimmune necrotising scleritis. Mycophenolate mofetil holds a promising role for inducing as well as maintaining disease remission in ocular GPA., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

A 63-year-old woman came with complaints of decreased vision and minimal pain on extraocular movements in the left eye and, after investigations, was diagnosed with posterior scleritis. Systemic steroid treatment helped resolve the symptoms and signs. One year later, she presented with right eye involvement. She was evaluated for systemic illness and, based on clinical and investigational findings, it was deduced that probably a sarcoidosis was predisposing her to the eye problem. She was provisionally diagnosed with bilateral sequential posterior scleritis secondary to sarcoidosis. She was prescribed oral steroids, which helped resolve her eye problem. She was next referred to a rheumatologist and a pulmonologist for further evaluation and treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To evaluate the reduction of conjunctival and episcleral hyperemia and bleeding in pterygium surgery following the use of topical brimonidine preoperatively. Methods: In this study, 45 patients who had undergone pterygium surgery under topical anesthesia were enrolled. Brominidine tartrate 0.15% eye drops were applied topically to 25 eyes of 25 patients at the sixth and third minutes preoperatively, and no drops were applied to 20 eyes of 20 patients for vasoconstriction. Preoperative and postoperative photographs and video images were taken. Results: Vasoconstriction effects of topical brimonidine tartrate on surface vessels were observed. The surface area of blood vessels was reduced 60% within 5 min; this effect was observed with the help of Photoshop and ImageJ programs and it lasted for ∼20 min. The surgery lasts for 7 min, on average, so the conjunctival whitening formed by brimonidine tartrate provides a safe and comfortable operative area throughout the surgery. Conclusion: We recommend applying brimonidine tartrate before pterygium surgery due to its conjunctival whitening effect to provide a safe and comfortable operative area throughout the surgery.

Purpose: : To present histopathological and immunohistochemical analysis of idiopathic inflammatory diseases of orbit and ocular adnexa., Methods: Design- A retrospective laboratory-based study. The study was carried out in an ocular pathology laboratory in a tertiary institute of northeast India where analysis of 93 cases was done in 5 years, during the period from 2011 to 2016. Hematoxylin--eosin and special stains were done for the diagnoses. Immunohistochemistry (IHC) panel was also carried out. For infectious pathology, Grocott's methenamine silver (GMS) stain for fungus, tissue Gram's stain for bacteria's, and acid-fast stains for tubercular bacilli were done. IHC panels were done for CD 20 (B-cells), CD-3 (T-cells), CD-45 (Leukocyte common antigen, LCA), BCL-2, CD-138 (Plasma cells), Kappa, Lambda, IgG-4 in tissue, IgG-4 in serum, etc. IHCs were done using kit methods (standardized) and adequate controls were taken for each sample., Results: 93 cases of nonspecific orbital inflammation were reported out of 1,467 specimens. Orbital pseudotumors (idiopathic orbital inflammatory disease, IOID) were seen in 27 cases (sclerosing variety-6); benign lymphoid hyperplasia in two cases; reactive lymphoid hyperplasia in 10 cases; atypical plasma lymphoproliferative reactive (polyclonal immunophenotypically, IgG4 negative) lesions in four cases; IgG-4 related disease in one case; nonspecific inflammatory reactions (conjunctiva, sclera, and lid) in 49 cases. In all the diagnoses, infections and lymphomas were excluded., Conclusion: Biopsy supported study on nonspecific orbital inflammation was important to know the pattern., Competing Interests: None

The patient is a 19-year-old female who presented with 3 weeks of right eye pain, eyelid swelling, blurry vision, and headache. Visual acuity was counting fingers at 1 foot. Intraocular pressure was normal, and there was diffuse scleral injection on anterior examination. She had a mild anterior chamber reaction with 15 cells/high-powered field and a mild vitreous inflammatory reaction. Fundus examination revealed diffuse choroidal thickening with multilobulated serous retinal detachments worse inferiorly (Figures 1 and 2). Fluorescein angiography demonstrated severe optic disc leakage. Ultrasonography demonstrated diffuse choroidal thickening, a serous retinal detachment, and a prominent "T-sign" (Figure 3). The patient was diagnosed with posterior scleritis and treated with 80 mg of oral prednisone. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:660.]., (Copyright 2019, SLACK Incorporated.)

In patients with inflammatory bowel disease (IBD), ocular extraintestinal manifestations (EIM) are less common than EIM of other systems, but they are clinically important because they can lead to complications that can cause catastrophic damage to the visual acuity and ocular structure. Anterior uveitis and episcleritis are the most common ocular EIM. Involvement of the orbit, posterior segment, and optic nerve can also occur. A variety of treatments are available ranging from topical steroids to systemic immunosuppressive therapies. The treatment of IBD is also essential if the activity of inflammatory bowel disease affects the ocular symptoms.

The sclera is an uncommon site of primary inflammation. Biopsy is infrequently employed in the evaluation of scleritis, but familiarity with its differential diagnosis is instrumental in ensuring efficient histological evaluation. This review provides a clinical overview of scleritis and describes the context in which scleral biopsy might arise. Most cases are associated with systemic autoimmune disease, but a sizeable proportion occur as an isolated disorder. Conditions mimicking autoimmune scleritis include infection and neoplasm. Histological patterns of inflammation in eyes removed surgically or at autopsy have been placed into three groups: (1) autoimmune scleritis characterised by varying mixtures of palisading granulomas, necrosis and vasculitis; (2) infectious scleritis, characterised by acute inflammation and necrosis; and (3) idiopathic scleritis, characterised by chronic non-specific inflammation with follicles and varying amounts of fibrosis. This traditional system of classification may be oversimplified. Aetiological or categorical classification is not always possible on small biopsies given the histopathological overlap of infectious and non-infectious scleritis., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

A 65-year-old man presented with transient neurological symptoms, followed by rapid cognitive decline, myoclonus and fevers. He had evidence of scleritis and an arthropathy. This paper reports the clinicopathological conference discussed at the Association of British Neurologists Annual Meeting 2017., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To characterize the clinical correlates and outcome of inflammatory ocular disease (IOD) among patients with ANCA-associated vasculitides (AAV)., Methods: Medical records of potential cases of AAV seen at Mayo Clinic from 2003 to 2013, inclusive, were reviewed to identify confirmed cases meeting the diagnosis of AAV using the Chapel Hill Consensus Conference 2012 descriptors. Records of confirmed cases of AAV were then further reviewed for IOD, and clinical characteristics, treatment and outcomes abstracted., Results: A total of 1171 confirmed cases of AAV were identified of which 183 patients (mean age 49.0 years; 51% female; 95% Caucasian) had IOD. The most common manifestation of IOD was injection of the eye (57%) followed by eye pain (46%) and visual acuity loss (18%). Scleritis was the most common type of IOD (22%) followed by episcleritis (21%), orbital inflammation (18%), lacrimal duct stenosis (10%) and uveitis (9%). Oral glucocorticoids were used to treat IOD in the majority of patients (96%). CYC and rituximab were the most frequently used immunosuppressive agents (54 and 36%, respectively). Of those with orbital inflammation, 52% underwent therapeutic surgical intervention. Clinical remission of IOD was achieved in 91% of patients but relapses were seen in 23%. Significant visual acuity loss was observed in only six patients., Conclusion: IOD is a common manifestation of AAV and seen in about 16% of patients with AAV. Scleritis, episcleritis and orbital inflammation are the most common subtypes. Most patients respond well to glucocorticoids and immunosuppression, but relapse of IOD is common., (© The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com)

Background/aims: To determine the morphological features of anterior scleral inflammation using swept-source optical coherence tomography., Methods: In this retrospective observational study, we examined 17 eyes of 14 patients with diffuse anterior scleral inflammation and 13 eyes of 13 young unaffected patients. We compared cross-sectional images of the conjunctiva, episclera and sclera obtained using swept-source optical coherence tomography equipped with a multiple B-scan averaging process between normal eyes and those with episcleritis and scleritis., Results: Optical coherence tomography showed that the conjunctival stroma/episclera layer was notably swollen in diseased eyes. The eyes with diffuse anterior scleral inflammation had a significantly thicker conjunctival stroma/episclera than normal eyes (403.0 μm vs 288.0 μm, p=0.002). There was no significant difference in scleral stroma thickness between eyes with anterior scleral inflammation and normal eyes (464.7 μm vs 434.2 μm, p=0.11). We separately analysed 11 eyes with diffuse scleritis and 6 eyes with diffuse episcleritis. While the conjunctival epithelium and conjunctival stroma/episclera were thicker in eyes with diffuse scleritis than in those with diffuse episcleritis (78.9 μm vs 50.4 μm, p=0.003 and 445.5 μm vs 308.8 μm, p=0.033, respectively), the scleral stroma thickness in eyes with diffuse scleritis was comparable with normal eyes (465.5 μm vs 434.2 μm, p=0.43)., Conclusions: The swelling of diffuse scleritis occurred within the episclera rather than in the scleral stroma. Since optical coherence tomography visualises the morphology of the episclera and sclera, it can be useful for evaluating inflammation activity and therapeutic effects in diffuse scleritis., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

The authors report a rare case of ocular surface squamous neoplasia with intraocular involvement that had an initial masquerade presentation of recurrent anterior nodular scleritis. A 35-year-old male patient presented with right eye recurrent anterior nodular scleritis for which a lamellar patch graft was done. Two months later, the patient presented with recurrence of symptoms. Histopathology review revealed the presence of well-differentiated squamous cell malignancy. A high index of suspicion for malignancy is required in such cases when they do not respond to conventional therapy.

Purpose: To report ocular manifestations of idiopathic primary myelofibrosis in a patient with relapsed primary myelofibrosis., Methods: Observational case report., Results: A 57-year-old African American male with history of primary myelofibrosis, diagnosed by bone marrow aspirate and biopsy, believed to be in remission was referred to us for bilateral angle-closure glaucoma refractory to medical treatment and laser peripheral iridotomy. His fundus examination revealed serous retinal detachments, choroidal effusions, and Roth spots, and B-scan revealed his angle closure was due to annular anterior ciliochoroidal effusions. The patient was taken to surgery for a pars plana vitrectomy with radial sclerotomies performed to relieve the patient's eye pressure in the left eye. Cytologic analysis of the choroidal effusions revealed neutrophilic infiltrates with immature forms and erythroid precursors, suggesting a neoplastic infiltration from the patient's primary myelofibrosis. The patient's vision and ocular symptoms significantly improved after chemotherapy. Repeat bone marrow aspiration and biopsy confirmed the patient's primary myelofibrosis with no progression to acute leukemia despite his high leukocyte count., Conclusion: Neoplastic infiltration of primary idiopathic myelofibrosis into the eye and surrounding structures, which is scantly described in the current literature, may be an early sign of new onset or relapsing primary myelofibrosis. Chemotherapy can be very effective in the ultimate treatment and remission of these ocular symptoms.

Posterior scleritis, a severe and painful inflammation of the sclera, is an often misdiagnosed pathology due to its clinical polymorphism. An accurate diagnosis is however needed in order to propose an appropriate treatment of the ophthalmologic symptoms and to exclude an associated systemic inflammatory or auto-immune pathology.

A 3.5-year-old adult female rhesus macaque (Macaca mulatta) manifested swelling of the left upper eyelid and conjunctiva and a decline in clinical condition 18 days following intramuscular challenge with Ebola virus (EBOV; Kikwit-1995), after apparent clinical recovery. Histologic lesions with strong EBOV antigen staining were noted in the left eye (scleritis, conjunctivitis, and peri-optic neuritis), brain (choriomeningoencephalitis), stomach, proximal duodenum, and pancreas. Spleen, liver, and adrenal glands, common targets for acute infection, appeared histologically normal with no evidence of EBOV immunoreactivity. These findings may provide important insight for understanding sequelae seen in West African survivors of Ebola virus disease., (Published by Oxford University Press for the Infectious Diseases Society of America 2015. This work is written by (a) US Government employee(s) and is in the public domain in the US.)

Purpose: To correlate the clinical course of sympathetic ophthalmia with the histological and immunohistochemical characteristics of the enucleated inciting eye., Methods: A consecutive case series with baseline clinical features and subsequent histopathologic findings., Results: Evaluation of the 16 enucleated inciting eyes (blind and painful) disclosed that 9 of the 16 had typical histology, fulfilling the criteria for sympathetic ophthalmia of diffuse granulomatous inflammation. Among the 16, 11 sustained previous penetrating trauma, 4 underwent previous eye surgery, and 1 patient presented with an unknown etiology. Patients with atypical histology (7 of 7) were taking corticosteroids at the time of enucleation. Only 2 of 9 patients with typical histology were taking corticosteroids at the time of enucleation. At 6 months after enucleation of the inciting eye, 4 of the 7 patients with atypical histology had a visual acuity of ≥20/40 compared with 8 of 8 patients (100%) with typical histology. On a 4-point scale (0-3+), the choroidal infiltrate of the 9 histopathologically typical eyes showed an average of 2.5+ CD68 (macrophages), 2.5+ CD20 (B cells), and 1.5+ CD3 (T cells)., Conclusion: Histopathologic findings had minimal correlation with the clinical course of sympathetic ophthalmia. Corticosteroid treatment before enucleation may influence the pathologic confirmation of sympathetic ophthalmia. The predominance of B lymphocytes and macrophages over T lymphocytes may represent different stages of the disease process.

A 48-year-old woman who is a contact lens wearer presented with unilateral ACANTHAMOEBA keratitis, confirmed by PCR, which responded initially to topical polyhexamethylene biguanide (PHMB) and brolene. Three months later, despite continued treatment, she developed diffuse anterior scleritis with severe pain and marked scleral injection but without evidence of recurrence keratitis. Oral non-steroidal anti-inflammatories and oral high-dose corticosteroids were added without success. Subsequent treatment with intravenous methylprednisolone and high-dose cyclosporine led to a temporary improvement. Re-presenting with signs of recurrent scleritis and severe pain, the antitumor necrosis factor monoclonal antibody adalimumab, and later oral cyclophosphamide, were added. This led to complete quiescence of the scleritis. Unfortunately, frequent recurrences of ACANTHAMOEBA keratitis and anterior uveitis occurred on immunosuppression requiring continued treatment with PHMB, brolene and topical corticosteroids. This is the first case of severe refractory ACANTHAMOEBA scleritis requiring the concomitant use of four immunosuppressive agents to achieve continued disease control. The challenges in managing this case are discussed., (2015 BMJ Publishing Group Ltd.)

Intravitreal fluocinolone acetonide implant (Retisert) has a high potency, a low solubility, and a very short duration of action in the systemic circulation, enabling the steroid pellet to be small and reducing the risk of systemic side effects. Scleral melt has not been reported as a possible complication of Retisert implant. The authors describe the occurrence of scleral melt 18 months after the implantation of fluocinolone acetonide implant in a 42-year-old Caucasian woman. To the authors' knowledge, this is the first report of this possible complication.

To investigate the choroidal morphologic changes in two patients with posterior scleritis. We used high-penetration optical coherence tomography (HP-OCT) in vivo with a long-wavelength light source (1,060 nm) to view the choroidal changes. In patient 1 with unilateral scleritis, the subfoveal choroidal thickness of the right eye was 418 μm with a serous retinal detachment (SRD) at the initial visit. The treatment regimen was prednisolone 30 mg/day, and the posterior scleritis resolved. Follow-up HP-OCT showed the following choroidal thicknesses-266 μm on day 27 with no SRD, 245 μm on day 69, and 200 μm on day 216. In patient 2 with bilateral scleritis, the subfoveal choroidal thickness of the left eye was 279 μm at the initial visit. The inflammation was more severe in the left eye compared to the right eye on day 99. The choroidal thickness again increased markedly with recurrent disease in the left eye despite treatment. The posterior scleritis resolved with treatment. Follow-up HP-OCT showed the following choroidal thicknesses of the left eye--321 μm on day 99, 257 μm on day 176, and 228 μm on day 358. Significant choroidal changes underlie posterior scleritis. HP-OCT can show these deep choroidal pathologies in patients with posterior uveitis. The choroidal thickness recovers following treatment.

Recent advances in the understanding of the initiation and perpetuation of the immune response strongly suggest that all forms of noninfective immunologically induced scleral inflammation have a common origin. Analysis of the progress of patients with scleritis corroborates the current clinical classification that, together with studies of the immunohistology fluoresceine/ICG angiography, 3D proteoglycan, and keratan sulphate electron microscopy of scleritis, strongly suggests that from the initiation of the inflammatory process, necrotizing scleritis and diffuse and nodular scleritis not only pursue a different course but also have a different pathogenesis; nonnecrotizing scleritis being the consequence of an auto immune response, whereas necrotizing scleritis being the complication of an already present (if not always manifest), systemic immune-mediated systemic disease and its associated vasculitis. The increasing imaging capacity of anterior segment ocular coherence tomography (OCT) and en face OCT enables the changes occurring in the sclera during the course of the disease to be observed for the first time. These observations suggest that the inflammatory changes involve the potential suprachoroidal space between choroid and sclera, an observation supported by the presence of subscleral granulomas on histopathology. New imaging techniques have also been able to explain the changes seen in the cornea as a complication of scleritis. These findings have implications for investigation and the treatment of these conditions.

To report the challenges in management in a case of scleritis with extensive staphylomas with secondary glaucoma. A 35-year-old one eyed female, a known case of scleromalacia perforans, presented with raised intraocular pressure on maximum medical treatment. She underwent successful Ahmed glaucoma valve surgery with exposure of the implant following a repeat episode of necrotizing scleritis after 3 months. Management of eyes with scleritis and secondary glaucoma can be challenging with unexpected complications and postoperative course.

A 49-year-old female patient previously treated for scleritis and uveitis-induced cataract in the right eye presented with a subretinal white lesion in the same eye. With a preliminary diagnosis of choroidal tumor, enucleation of the eyeball was performed in accordance with the patient's request. Histologic and immunohistologic examinations were consistent with immunoglobulin G4-related disease. The case demonstrates that it is important to consider IgG4-related disease in the differential diagnosis of an intraocular tumor.

A 57-year-old man presented to the ophthalmology clinic with a red right eye. He denied pain, diplopia, tearing, and blurred vision. His medical history included asymptomatic annular plaques on the trunk and extremities for at least a decade. Ophthalmological examination revealed a necrotizing scleritis of the right eye. Examination of the skin demonstrated variable sized annular plaques with central atrophy, some with prominent indurated border and yellow discoloration. No periorbital lesions were present. The ocular lesion rapidly progressed and areas of scleral melting developed in the right eye, which eventually required a scleral patch graft. The left eye also developed necrotizing scleritis with areas of scleral melting. Two sets of skin biopsies were performed a few weeks apart. An initial set of skin punch biopsies revealed extensive palisading granulomatous inflammation throughout the dermis, extending into the subcutis. The accompanying perivascular mononuclear infiltrate contained the collections of plasma cells. Scattered multinucleated giant cells were noted. The possibility of necrobiosis lipoidica diabeticorum was suggested. Subsequent skin biopsies showed more prominent and extensive necrobiosis, raising the possibility of necrobiotic xanthogranuloma. Protein electrophoresis was performed, which revealed an IgG λ monoclonal protein.

Objective: The objective of this study was to characterize the clinical features of relapsing polychondritis (RPC) within the Department of Defense beneficiary population and determine the utility of echocardiography, imaging studies, and pulmonary function testing for diagnosis and monitoring disease., Methods: We performed a retrospective Electronic Medical Record chart review of all patients diagnosed with RPC within the Department of Defense between January 2004 and December 2009., Results: Thirty patients met McAdam's diagnostic criteria and an additional 13 met our criteria for partial RPC. Auricular chondritis (88%), inflammatory eye disease (57%), and arthritis (60%) were the most common clinical manifestations. Pulmonary involvement was seen in 16 (37%) patients. Methotrexate (42%) and corticosteroids (21%) were the most conventional therapies. Thirty (70%) patients had pulmonary function tests with flow volume loop abnormalities observed in 33%. Chest computed tomography was performed in 63%, with abnormalities in 48%. Abnormalities on echocardiography were observed in 12 of 25 (48%) patients., Conclusions: The incidence, demographic data, and organ involvement in our RPC patients were similar to previous studies. The diagnosis of RPC was determined primarily on physical examination and symptom-driven diagnostic testing. There was no notable pattern by rheumatologists for monitoring the progression of tracheobronchial tree or large vessel involvement. Interpreting flow volume loops is recommended with pulmonary function testing to detect early laryngotracheal involvement. Computed tomography of the chest is also recommended to monitor for vascular and tracheobronchial tree involvement., (Published by Elsevier Inc.)

Case Report: We describe the findings on optical coherence tomography before and after treatment with corticosteroids in a 51 year old patient with recurrent episodes of bilateral posterior scleritis without associated systemic disease., Discussion: We believe that optical coherence tomography allows the pathological changes that occur in posterior scleritis to be monitored by objective and quantitative comparison of images, and even for therapeutic decisions., (Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.)

Purpose: To describe the clinical, histological, and immunohistochemical manifestations of canine necrotizing scleritis., Methods: A retrospective examination of the clinical records and samples of ocular tissues from five dogs with a histological diagnosis 'necrotizing scleritis' was completed. Archived, formalin-fixed, paraffin-embedded samples and two control globes were stained with hematoxylin and eosin, Gram, periodic acid-Schiff (PAS) and Masson trichrome stains, and they were immunohistochemically labeled for CD3, CD18, and CD20., Results: Of the five cases reviewed, only two could be confirmed as idiopathic necrotizing scleritis. The other three cases were retrospectively diagnosed as unilateral focal, non-necrotizing scleritis, one as episcleritis and the third was scleritis secondary to a proptosed globe based on our retrospective clinical, histological, and immunohistochemical evaluations. In these two cases, idiopathic necrotizing scleritis manifested as a bilateral, progressive, inflammatory disease of the sclera and cornea that induces significant uveitis. Light microscopic examination confirmed collagen degeneration and granulomatous inflammation. There was no evidence for an infectious etiology based on Gram's and PAS stainings. Immunohistochemical labeling revealed a predominance of B cells in idiopathic, bilateral necrotizing scleritis. Tinctorial staining abnormalities with Masson's trichrome stain were present in scleral collagen of the two cases with idiopathic necrotizing scleritis as well as a case of secondary traumatic scleritis., Conclusions: Based on a limited number of cases, idiopathic canine necrotizing scleritis shares similar histopathological features with non-necrotizing scleritis and episcleritis; however, necrotizing scleritis is B-cell-dominated and bilateral, and significant collagen alterations manifest with Masson's trichrome stain., (© 2011 American College of Veterinary Ophthalmologists.)

Rheumatic conditions affect a wide variety of tissues, including the eye, and can cause significant visual loss. Early diagnosis coupled with appropriate management, using immunosuppression where necessary, can significantly improve the outcome. This article reviews the most common manifestations seen in clinical practice.

A 2-year-old male castrated Springer Spaniel with a 3-week history of waxing and waning right sided exophthalmos, periorbital swelling, chemosis, and fundic changes was examined. Ophthalmic examination of the right eye revealed slight chemosis and nictitating membrane protrusion. Indirect ophthalmoscopy showed an approximately 8 mm peripapillary hyporeflective tapetal lesion with subretinal edema consistent with a bulbar/retrobulbar disease process. Inflammatory or neoplastic diseases were the main differential diagnoses. Ancillary diagnostics (ocular ultrasound and computed tomography) showed a focal, poorly defined dense tissue involving the caudomedial aspect of the right globe, adjacent to the optic nerve head without apparent orbital involvement. Considering the duration, location, and severity of the clinical findings, enucleation of the right globe was performed. Histopathology revealed focal thickening of the posterior wall with severe pyogranulomatous inflammation, surrounding a foreign body most consistent with plant material embedded between the sclera and the choroid (suprachoroidal). This report describes a rare case of a scleral/suprachoroidal foreign body (plant material), and outlines the difficulty of establishing an etiological diagnosis using standard ocular imaging., (© 2011 American College of Veterinary Ophthalmologists.)