Infectious Scleritis: Pathophysiology, Diagnosis, and Management.

ABSTRACT: Infectious scleritis (IS) is a rare and severe ocular disorder responsible for approximately 5%-15% of all scleritis cases. It is often associated with a poor prognosis due to its similar clinical presentation to autoimmune scleritis, resulting in a delayed diagnosis and treatment. Therefore, differentiating between infectious and noninfectious entities is critical. Several details extracted from the patient's history and clinical examination can raise suspicion for infection. The most common predisposing factor is previous ocular surgery, especially pterygium, cataract, and vitreoretinal surgeries. Ocular trauma, poor contact lens hygiene, "eye-whitening" procedures, and subtenon triamcinolone injections have also been implicated. Clinical features of infection include the presence of scleral necrosis, hypopyon, unifocal or multifocal scleral abscesses, and mucopurulent discharge. Thorough diagnostic testing is essential before excluding infection as a possibility. Empiric broad-spectrum topical and systemic antibiotic therapy should be initiated while awaiting laboratory results and adjusted accordingly. Most IS cases require both aggressive medical and surgical treatment, and various studies have reported favorable outcomes with this combination. At this time, there is no consensus on the management of this severe ocular condition, and future studies are needed to establish clear treatment guidelines.

Clinical and pathological findings in three dogs with a corneocentric presentation of nodular granulomatous episcleritis.

PURPOSE: To describe the clinical signs, management, histopathologic findings, and outcome of three dogs with a corneocentric presentation of nodular granulomatous episcleritis (NGE). METHODS: Three dogs of varying breeds were presented for a unilateral, nonpainful, and infiltrative corneal lesion in the dorsal aspect of the eye. Clinical response to symptomatic topical treatment directed at a presumed inflammatory or immune-mediated cause was poor. Due to this, and concerns of neoplasia, ultrasonography (n = 1), incisional biopsy (n = 2), and/or enucleation (n = 2) were performed. RESULTS: The inflammatory infiltrate observed on histopathology was identical to that seen in nodular granulomatous episcleritis in all three cases. However, atypically the inflammation was confined to the cornea and limbus, without episcleral or conjunctival involvement. Inflammation of the cornea was full thickness to Descemet's membrane. Following enucleation (n = 2), there were no postoperative complications, and no reported ophthalmic disease in the remaining eye. Currently, the single non-enucleated case remains controlled with systemic and topical immunosuppression. CONCLUSION: To the best of the authors' knowledge, this is the first report of an NGE condition purely affecting the full thickness of the cornea, without episcleral or conjunctival involvement. The authors propose this to represent an atypical corneocentric variant of NGE. This clinical presentation can resemble neoplasia; incisional biopsy is recommended for a definitive diagnosis. Further research into the optimal treatment strategies for this variant of NGE is required.

Scedosporium apiospermum infectious scleritis following posterior subtenon triamcinolone acetonide injection: a case report and literature review.

BACKGROUND: Ubiquitous fungi of the Scedosporium apiospermum species complex (SASC) cause various opportunistic infections. Posterior subtenon triamcinolone acetonide (STTA) injection is a standard therapy for intraocular inflammation and macular edema. We report a case of Scedosporium apiospermum infectious scleritis after a posterior STTA injection. CASE PRESENTATION: A 75-year-old man received a posterior STTA injection to treat macular edema in his left eye. After 3 months, he complained of ocular pain and hyperemia in his left eye. Examination showed a subtenon abscess in the site corresponding with the STTA injection. After incising the abscess, the smear revealed numerous conidia-like structures. Although we suspected fungal infection and started topical voriconazole (VRCZ) and levofloxacin, the inflammation of the eye worsened. Fungal culture revealed filamentous fungus growth. Subsequently, we added systemic VRCZ and performed surgical debridement of the infected sclera and Tenon's capsule. Pathology of the sclera showed fungal hyphae. The antifungal susceptibility test revealed low minimum inhibitory concentrations for micafungin, VRCZ and miconazole (0.06, 0.25 and 0.5 µg/mL, respectively). After 2 months, the ciliary injection subsided and VRCZ therapy was stopped. However, subtenon abscess recurred 1 month after discontinuation of topical VRCZ. Surgical debridement and topical VRCZ were resumed, with the eye finally improving after 5 months of management. The fungal species was identified as Scedosporium apiospermum sensu stricto morphologically and by DNA sequencing. CONCLUSIONS: This case was successfully treated by topical and systemic VRCZ and repeated surgical debridement. Infectious scleritis caused by SASC rarely develops after posterior STTA. SASC can produce conidia in the enclosed subtenon space. Late-onset infectious scleritis after a posterior STTA injection suggests the presence of a fungal infection, including SASC, thereby requiring extensive and prolonged medical and surgical treatment.

[Scleritis - Progress in Diagnosis and Therapy]. / Skleritis ­ Fortschritte zur Diagnose und Therapie.

Inflammation of the sclera includes a wide range of morphological changes, which often occur in association with systemic diseases. While episcleritis is mainly harmless, scleritis often proceeds and is characterised by a destructive course. This may lead to important complications, that present as ulcerative keratitis, uveitis or secondary increases in intraocular pressure. As this form of inflammation is severe and there are often underlying diseases, this contribution focusses on differential diagnosis and current therapeutic approaches.

Severe fungal sclerokeratitis caused by Metarhizium anisopliae: a case report and literature review.

To date, there has been only one published report on the infectious sclerokeratitis caused by Metarhizium anisopliae, which is an entomopathogenic fungus. Regarding corneal infection, three reports have been published to date. Although the prognoses of the corneal infections are favourable, prognosis when scleral infection is involved is very poor. A 76-year-old patient presented with foreign body sensation in the left eye. Microscopic examination with Fungi Flora Y staining of the corneal scraping revealed fungal infection. The conjunctiva was melted by the infection over a wide area. Although intensive medications were administered, an emergency surgery was necessary because scleral thinning, corneal perforation and lens prolapse occurred. The fungal isolate was identified as M. anisopliae by sequencing the internal transcribed spacer region. Herein, we report the second known case worldwide of M. anisopliae sclerokeratitis, and we review the literature related to the ocular infections.

Red Alert: diagnosis and management of the acute red eye.

The acute red eye represents a broad spectrum of disease encompassing benign self-limiting conditions to potentially vision-threatening ophthalmic or system disease. This article will review clinical principles essential for the diagnosis and treatment of red eye relevant to all Armed Forces primary care and emergency medicine practitioners.

Update in the management of extraintestinal manifestations of inflammatory bowel disease.

Inflammatory bowel disease, comprised of Crohn's disease and ulcerative colitis, are chronic inflammatory disorders of the gastrointestinal tract. Up to 40 % of patients with inflammatory bowel disease can develop inflammation in other organ systems of the body. These extraintestinal manifestations (EIM) can affect the musculoskeletal, ocular, mucocutaneous, and hepatobiliary systems. Symptoms related to EIM can result in impaired quality of life, and complications of EIM can lead to disfigurement, functional deficits, and even life-threatening organ dysfunction. Some EIM parallel the activity of IBD, and respond to treatment of the underlying disease. Others, however, follow an independent course and require targeted treatment.

Possible Synergistic Role of Cryo-Alcohol Therapy in Infectious Scleritis-Scope and Rationale for Expanding Indications and Review of the Literature.

PURPOSE: The purpose of this study was to highlight the use of topical ethanol as an adjunct to cryotherapy, termed cryo-alcohol therapy, in the management of fungal/acanthamoeba scleritis along with a review of the literature. METHOD: Retrospective interventional case reports of fungal and acanthamoeba scleritis along with a review of the literature. RESULTS: The patient with circumferential necrotic fungal scleritis resolved in 6 weeks achieving a best-corrected visual acuity (BCVA) of 20/20, and the patient with acanthamoeba scleritis is awaiting optical keratoplasty after complete resolution in 8 weeks. The literature review from January 1990 to December 2020 revealed BCVA >20/200 in 50% of the eyes with a mean time to resolution being 4.16 ± 2.13 months in fungal scleritis, with 27.02% and 75% of the eyes requiring evisceration in fungal and acanthamoeba scleritis, respectively. CONCLUSIONS: Cryotherapy is a useful adjunct in managing refractory infectious scleritis, and its efficacy can be enhanced by combining the use of topical ethanol to aid in faster recovery and reduce visual morbidity.

[Scleritis and episcleritis]. / Sclérite et épisclérite.

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.

Surgically induced necrotising scleritis after pterygium excision.

BACKGROUND: Surgically induced necrotising scleritis (SINS) presents as a focal area of intense scleral inflammation next to the site of previous scleral or limbal excision. There may be or no associated connective tissue disease. It may also follow Pterygium excision with use of antimetabolites. OBJECTIVE: To report an uncommon complication following pterygium excision, one of the commonest surgeries performed by Ophthalmologists. CASE REPORT: Our patient was a 62-year-old retired teacher who had a left eye nasal primary pterygium excised with Mitomycin C (MMC) dab topically. He presented 20 months later with signs and symptoms of SINS in the left eye. Systemic and laboratory examinations were normal. The scleral defect was covered with mucous membrane graft (MMG) obtained from the lower lip. The choice of MMG was due to non-availability of amniotic membrane graft (AMG) in our centre. The patient was also placed on pulsed injection of methylprednisolone 1000 mg daily in slow intravenous (IV) infusion for three days, IV cyclophosphamide 500 mg on the 4th day and then on a monthly basis for six months; the graft remained intact thereafter with no further signs of inflammation over a period of six months of follow up. CONCLUSION: SINS should be recognised as a possible complication of pterygium excision. The use of MMG as an alternative for sclera patch graft in the absence of AMG is suggested.

Optimizing diagnosis and management of nocardia keratitis, scleritis, and endophthalmitis: 11-year microbial and clinical overview.

OBJECTIVE: To identify clinical factors and microbiological assays that facilitate a rapid diagnosis of Nocardia keratitis, scleritis, and endophthalmitis, and to determine optimal medical and surgical management strategies. DESIGN: Retrospective, consecutive case series. PARTICIPANTS: A total of 111 cases of keratitis, 11 cases of scleritis, and 16 cases of endophthalmitis, all culture-proven Nocardia infections, were identified between January 1999 and January 2010. INTERVENTION: The keratitis cases underwent intensive medical management, and the scleritis and endophthalmitis cases required concurrent surgical intervention for disease control. Corneal and scleral scrapings, as well as undiluted vitreous sample, were submitted for microbiologic evaluation (direct smear and culture). MAIN OUTCOME MEASURES: Historical points, clinical findings, and microbiologic assays that facilitated a prompt Nocardia diagnosis were identified, and management choices were examined for correlation with final acuity. RESULTS: Ocular exposure to soil or plant matter was a common historical point in cases of Nocardia keratitis (48%) and scleritis (45%), respectively. Nocardia keratitis often (38.7%) presented with "wreath"-shaped anterior stromal infiltrate or infiltrate interspersed with elevated, pinhead-sized, chalky lesions. Most patients with scleritis (63.4%) presented with nodular lesions demonstrating pointed abscesses. Nocardia endophthalmitis typically (75%) presented with endoexudates or nodular exudates surrounding the pupillary border. Gram stain and 1% acid-fast stain enabled prompt diagnosis of Nocardia in 64% and 63% of keratitis cases and 45% and 63% of scleritis cases, respectively. Direct smear was usually not revealing in cases of Nocardia endophthalmitis. Isolates from Nocardia keratitis, scleritis, and endophthalmitis demonstrated 97%, 100%, and 90% susceptibility to amikacin, respectively. Nocardia keratitis resolved with medical therapy alone in 82% of cases. Younger age and better initial acuity correlated with improved final acuity in keratitis cases. Outcomes were poor after Nocardia scleritis and endophthalmitis. CONCLUSIONS: Early appropriate treatment often results in visual recovery in eyes with Nocardia keratitis. Despite aggressive and prompt surgical intervention, the prognosis for Nocardia scleritis and endophthalmitis is more guarded. Nocardia isolated from ocular infections demonstrate high levels of susceptibility to amikacin. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

[Diagnosis and treatment of scleritis associated to inflammatory systemic diseases]. / Diagnose und Therapie der Skleritis bei entzündlichen Systemerkrankungen.

Because of potential vision-threatening complications and the association to inflammatory systemic diseases with an increased mortality, scleritis patients need an adequate diagnostic work-up and early and aggressive anti-inflammatory systemic treatment. New therapeutic options (such as biologicals) help to achieve remission in otherwise refractory cases.

[Diseases of the outer eye in rheumatoid arthritis]. / Erkrankungen des äusseren Auges bei rheumatoider Arthritis.

Eye involvement is a frequent finding in patients with rheumatoid arthritis and may represent the leading clinical manifestation of disease. In this context, all components of the visual organ might be affected. The main spectrum of eye involvement comprises keratoconjunctivitis sicca, episcleritis and scleritis as well as ulcerative keratitis. As with the underlying disease, autoimmune reactions based on a patient's genetic predisposition are assumed to be of significance in disease pathogenesis. Emerging evidence also points to additional morphological and physiological ocular characteristics in the pathogenesis of the various ocular pathologies. This article gives an overview of clinical aspects, pathogenetic background as well as therapeutic options for ocular involvement in rheumatoid arthritis.

Acanthamoeba sclerokeratitis.

BACKGROUND: Acanthamoeba scleritis is an uncommon but severe complication of acanthamoeba keratitis. We report the clinical and histopathologic features of a patient with acanthamoeba sclerokeratitis. METHODS: Review of the patient's clinical records and histopathologic examination of the globe including light microscopy and transmission electron microscopy. RESULTS: Review of the clinical record of the patient revealed a past ocular history of penetrating keratoplasty for persistent acanthamoeba keratitis. Later in the course of treatment, the patient developed nodular necrotizing scleritis with culture-proven viable acanthamoeba in this area. She underwent enucleation of the eye for persistent acanthamoeba sclerokeratitis. Histopathologic examination of the globe revealed no acanthamoeba cysts or trophozoites at the site of crotherapy. CONCLUSION: Our study provides evidence for the invasion of acanthamoeba organisms into the sclera in a case of acanthamoeba keratitis. The presence of trophozites in scleral tissue may exacerbate the immune response leading to nodular scleritis.

[Scleromalacia perforans. Rheumatoid arthritis--case report]. / Scleromalacie perforans. Poliartrita reumatoida--caz clinic.

The authors present the case of a 73-year-old patient who comes in our clinic because of the existence of a tumoral formation pigmented and elevated, situated in the temporal side of the right eye and because of decreasing of her visual acuity at both eyes, as well as foreign body eye sensation, tearing, light sensitivity redness of the eye. The ophthalmological examination establishes the diagnosis of the right eye: Scleromalacia perforans, scleral temporal nodule considering the chronic, systemic inflammatory invalidated disorder, rheumatoid arthritis fourth stage. The purpose of this case presentation is represented by the rarity of this type of ocular manifestation presented in late stages of rheumatoid arthritis.

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report.

BACKGROUND Systemic vasculitides constitute heterogenous conditions affecting many organs and systems through blood vessel inflammation. Although there are some classifications for vasculitis, several vasculitides are "unclassified" because they cannot be clearly assigned to one of the known entities. CASE REPORT We report an autopsy case of a 67-year-old Japanese man who presented with fever, ocular pain, erythema, chest pain, and headache. The disease caused episcleritis, thrombophlebitis, extensive deep vein thrombosis, multiple pulmonary nodules and masses, hypertrophic pachymeningitis, and hyper-intensity areas in brain parenchyma on magnetic resonance images. Histopathology of the pulmonary nodule confirmed vasculitis affecting medium-to-small veins and arteries without necrotizing vasculitis or granulomatous inflammation. We diagnosed the patient with unclassified vasculitis based on the clinicopathological characteristics. Steroids in combination with immunosuppressants were used, but the disease was refractory and relapsing. The disease activity was eventually controlled with rituximab, but the patient died of bronchopneumonia. On autopsy, lung and brain findings indicated healed vascular inflammation. CONCLUSIONS This is the first case report of unclassified vasculitis, which is characterized as medium-to-small-sized arteritis and phlebitis, causing episcleritis, thrombophlebitis, deep vein thrombosis, pulmonary vasculitis, and intracranial vasculitis. The clinical conditions share some similarities with granulomatosis with polyangiitis and Behçet's disease; however, they meet no classification criteria of any specific vasculitis. More cases need to be analyzed to confirm our findings.

Posttraumatic Stenotrophomonas maltophilia infectious scleritis.

PURPOSE: To describe the history, clinical presentation, and successful surgical and antibiotic management of a case of posttraumatic infectious scleritis secondary to Stenotrophomonas maltophilia. METHODS: A 51-year-old white man presented with worsening light sensitivity, localized conjunctival hyperemia, and a painful scleral nodule in his right eye that developed over a period of 1 month after minor ocular trauma. The patient was treated by his referring ophthalmologist for "episcleritis" with fluorometholone 0.1%, 1 drop 4 times a day, since injury onset without clinical improvement. Evaluation consisted of slit-lamp examination, ultrasound biomicroscopy, and surgical exploration with tissue cultures and histology. RESULTS: Ultrasound biomicroscopy of the right eye revealed the presence of a dome-shaped mass overlying an area of partial-thickness scleral laceration in the inferotemporal quadrant. The scleral nodule was surgically excised, and the scleral laceration was repaired with one 8-0 nylon suture. Culture results revealed infection by S. maltophilia, which was resistant to gentamicin, tobramycin, and trimethoprim-sulfamethoxazole. The patient experienced immediate pain relief after surgery, and treatment was continued with both topical ciprofloxacin 0.3% and prednisolone acetate 1% for 1 month with full recovery. CONCLUSIONS: S. maltophilia should be considered in the differential diagnosis of posttraumatic infectious scleritis. Submission of appropriate surgical specimens for microbiologic analysis and adequate antibiotic therapy may prevent the development of endophthalmitis in cases of suspected posttraumatic infectious scleritis.