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Indian Journal of Ophthalmology Sep 2020Scleritis is a rare painful ocular disorder, associated with severe ocular pain and tissue destruction. Although a majority of these cases are immune mediated and at... (Review)
Review
Scleritis is a rare painful ocular disorder, associated with severe ocular pain and tissue destruction. Although a majority of these cases are immune mediated and at least half of these are associated with systemic immune-mediated diseases, a smaller minority are due to infections of the sclera. The two conditions closely mimic each other, and a thorough knowledge of the subtle differences is necessary in order to reach a timely diagnosis. Diagnostic delay can lead to a poor outcome both due to the destruction caused by the uncontrolled infection and also due to propagation of the infection with the use of corticosteroids which may have been started for presumed immune mediated scleritis. In this review, we present the clinical features, etiological agents, and the differentiating features between immune and infectious scleritis. We also present diagnostic and management guidelines for managing scleral infection.
Topics: Adrenal Cortex Hormones; Delayed Diagnosis; Eye Pain; Humans; Sclera; Scleritis
PubMed: 32823398
DOI: 10.4103/ijo.IJO_2032_20 -
La Revue de Medecine Interne Dec 2023Scleritis and episcleritis are rare ocular inflammatory diseases but deserve to be known by internists because of their frequent association with systemic autoimmune... (Review)
Review
Scleritis and episcleritis are rare ocular inflammatory diseases but deserve to be known by internists because of their frequent association with systemic autoimmune diseases. It is important to distinguish them between because their prognosis, therapeutic management and potential complications are very different. Episcleritis represents a superficial ocular inflammation with usually benign visual prognosis, no complication with local treatment, and is associated with a systemic autoimmune disease in rare cases. In contrast, scleritis is a potentially serious ophthalmological condition that can threaten the visual prognosis in the absence of appropriate systemic treatment. It is associated with an underlying disease in 40-50% of cases, in particular a systemic autoimmune disease (25-35% of cases) or an infectious cause (5-10% of cases). Rheumatoid arthritis and systemic vasculitides, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, are the main autoimmune causes of scleritis and episcleritis. Scleritis can reveal the underlying autoimmune disease and requires systematic etiological investigations. Aggressive, complicated, refractory forms or those associated with a systemic autoimmune disease require glucocorticoids or even immunosuppressants, and close collaboration between ophthalmologists and internists is required. The development of biologic agents offers new effective therapeutic tools in the management of these difficult cases.
Topics: Humans; Scleritis; Inflammation; Arthritis, Rheumatoid; Prognosis; Autoimmune Diseases; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
PubMed: 37344292
DOI: 10.1016/j.revmed.2023.05.013 -
Journal of Clinical Pathology May 2019The sclera is an uncommon site of primary inflammation. Biopsy is infrequently employed in the evaluation of scleritis, but familiarity with its differential diagnosis... (Review)
Review
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.
Topics: Biopsy; Humans; Sclera; Scleritis
PubMed: 30723093
DOI: 10.1136/jclinpath-2018-205360 -
Ocular Immunology and Inflammation 2016
Topics: Cyclooxygenase Inhibitors; Female; Flurbiprofen; Humans; Male; Middle Aged; Scleritis
PubMed: 26872551
DOI: 10.3109/09273948.2016.1136190 -
Ocular Immunology and Inflammation May 2022To present a case of necrotizing sclerokeratitis in a patient with multidrug-resistant tuberculosis and study the challenges in diagnosis and management of anterior... (Review)
Review
PURPOSE
To present a case of necrotizing sclerokeratitis in a patient with multidrug-resistant tuberculosis and study the challenges in diagnosis and management of anterior tuberculous scleritis.
METHODS
Retrospective observational case report and review of anterior tuberculous scleritis.
RESULTS
A 28-year-old woman, previously diagnosed as presumed tubercular panuveitis, presented with necrotizing sclerokeratitis and progressed to develop panophthalmitis. Laboratory investigations revealed multidrug-resistant as the etiological agent. We reviewed cases of anterior tuberculous scleritis published in the literature, with regards to clinical features, microbiological investigations, treatment, and outcomes. Treatment includes standard antitubercular therapy, with or without systemic corticosteroids. Poor response to treatment is seen either due to delayed diagnosis or drug resistance, and the significance of the same is highlighted in our case.
CONCLUSION
Diagnosis of tuberculous scleritis is a challenge. Therapeutic failure must alert the clinician for drug resistance which is diagnosed early, can prevent the devastating outcomes.
Topics: Adult; Antitubercular Agents; Drug Resistance, Multiple; Female; Humans; Keratitis; Retrospective Studies; Scleritis; Tuberculosis; Tuberculosis, Ocular
PubMed: 33416427
DOI: 10.1080/09273948.2020.1853176 -
Journal Francais D'ophtalmologie Oct 2017Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of... (Review)
Review
Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly granulomatosis with polyangiitis (Wegener's) and polychondritis, and less often inflammatory bowel disease and systemic lupus erythematosus. Among the different types of scleritis, a clear distinction exists between necrotizing forms leading to decreased vision in 50 % of the cases and associated with systemic diseases in the vast majority, and non-necrotizing forms (either diffuse or nodular), with a better prognosis. However, recent publications show that necrotizing forms are much less frequent nowadays (around 5 % of the total), probably due to therapeutic innovations and progress made during the last 20 years. The medical management of scleritis requires collaboration between ophthalmologists and internists (or rheumatologists).
Topics: Autoimmune Diseases; Diagnosis, Differential; Diagnostic Techniques, Ophthalmological; Humans; Prognosis; Scleritis
PubMed: 28916114
DOI: 10.1016/j.jfo.2017.04.007 -
Survey of Ophthalmology 2005Scleritis is typically a severe painful inflammatory process centered in the sclera that may involve the cornea, adjacent episclera, and underlying uvea; it poses a... (Review)
Review
Scleritis is typically a severe painful inflammatory process centered in the sclera that may involve the cornea, adjacent episclera, and underlying uvea; it poses a significant threat to vision. Careful clinical history taking, detailed ocular examination, appropriate investigation for ocular disease with or without underlying systemic disease, and timely intervention with the use of immunosuppressant drugs when necessary, has improved the long-term outcome for patients with this disease.
Topics: Humans; Scleritis
PubMed: 15967190
DOI: 10.1016/j.survophthal.2005.04.001 -
Experimental Eye Research Aug 2020Scleritis is a sight-threatening inflammation characterized by severe pain and redness of the eye. It can cause blindness by severe complications like scleral and... (Review)
Review
Scleritis is a sight-threatening inflammation characterized by severe pain and redness of the eye. It can cause blindness by severe complications like scleral and corneal necrosis, keratitis, and uveitis. The pathogenesis of scleritis is largely unknown due to a combination of the rarity of the disease, the little available human tissue-based research material, and the lack of animal models. The immune system is assumed to play a crucial role in the pathogenesis of scleritis. Multiple clues indicate probable antigenic stimuli in scleritis, and the involvement of matrix metalloproteinases in the destruction of scleral tissue. In this article we review the current insights into the pathogenesis of scleritis, and we suggest new hypotheses by implementing knowledge of systemic autoimmune disease pathogenesis. Understanding the pathogenesis of scleritis is crucial to improve the clinical management, as well as to find novel treatment modalities.
Topics: Autoimmunity; Diagnostic Imaging; Humans; Matrix Metalloproteinases; Sclera; Scleritis
PubMed: 32504648
DOI: 10.1016/j.exer.2020.108078 -
The Veterinary Clinics of North... Mar 2023Episcleritis is synonymous with episclerokeratitis although the latter is most appropriate as the cornea is often affected as well as the episclera. Episcleritis is a... (Review)
Review
Episcleritis is synonymous with episclerokeratitis although the latter is most appropriate as the cornea is often affected as well as the episclera. Episcleritis is a superficial ocular disease characterized by inflammation of the episclera and conjunctiva. It responds most commonly to topical antinflammatory medications. In contrast scleritis is a granulomatous fulminant panophthalmitis that will progress rapidly and induce significant intraocular disease including glaucoma and exudative retinal detachments without systemic immune suppressive therapy.
Topics: Animals; Dogs; Scleritis; Inflammation; Dog Diseases
PubMed: 36813394
DOI: 10.1016/j.cvsm.2022.10.007 -
Current Rheumatology Reports Mar 2020The aim of this review is to provide the information necessary for recognizing scleritis in rheumatology patients, recognize associated systemic diseases in scleritis... (Review)
Review
PURPOSE OF REVIEW
The aim of this review is to provide the information necessary for recognizing scleritis in rheumatology patients, recognize associated systemic diseases in scleritis patients referred to rheumatology, and choose the best conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) or biologic therapy, based on the most recent data.
RECENT FINDINGS
Scleritis is most often associated with systemic conditions, particularly autoimmune conditions. Rheumatologists should be most vigilant about rheumatoid arthritis and ANCA-associated vasculitis. A recent systemic association has been found with IgG4-related disease as well. Most of the therapeutic decisions are based on observational data. Most recently, there is increasing evidence of the role of rituximab for refractory disease. Scleritis is a severe inflammatory ocular condition that leads to complications including vision loss. It can precede a systemic autoimmune disease diagnosis, especially in the case of vasculitis. Prompt recognition and adequate therapy are needed in order to avoid serious ocular complications. There is an unmet need for (1) head-to-heat trials on csDMARDs and biologic drugs for the treatment of idiopathic scleritis and (2) randomized controlled trials on the treatment of refractory cases.
Topics: Antirheumatic Agents; Autoimmune Diseases; Humans; Rituximab; Scleritis
PubMed: 32219644
DOI: 10.1007/s11926-020-0885-y