Systemic Vasculitis and Its Association with the Eye.

Vasculitis is a group of diseases characterized by the inflammation of the blood vessel walls. They are classified according to the size of the main vessel involved: large vessel, medium vessel, and small vessel vasculitis. Ophthalmic manifestations are quite common in most of these diseases. Episcleritis and scleritis are the most prevalent manifestation of vasculitis. However, there are certain ocular features characteristic of specific vasculitis entities. Given the severity and potential life-threat of these diseases, knowledge of the ocular manifestations is mandatory for the ophthalmologists.

Diagnosis and Treatment of Central Serous Chorioretinopathy in Patients with Scleritis.

Central serous chorioretinopathy (CSCR) is characterized by central neurosensory retinal detachment from the retinal pigment epithelium. While the association between CSCR and steroid use is widely recognized, it is difficult to distinguish whether the subretinal fluid (SRF) in ocular inflammatory disease results from steroid use or an inflammation-related uveal effusion. We report the case of a 40-year-old man who presented to our department with intermittent redness and dull pain in both eyes that had persisted for three months. He was diagnosed with scleritis with SRF in both eyes and steroid therapy was started. Inflammation improved with steroid use, but SRF increased. This indicated that the fluid was not caused by the posterior scleritis-related uveal effusion but by steroid use. SRF and clinical symptoms subsided after steroids were discontinued completely and immunomodulatory therapy was initiated. Our study highlights that steroid-associated CSCR must be considered in the differential diagnosis of patients with scleritis, and prompt diagnosis with an immediate shift from steroids to immunomodulatory therapy can resolve SRF and clinical symptoms.

Pulmonary nodules and nodular scleritis in a teenager with superficial granulomatous pyoderma gangrenosum.

Superficial granulomatous pyoderma gangrenosum, a rare variant of pyoderma gangrenosum, has been considered to be the most benign form of the disease. We present the case of a 15-year-old boy with pulmonary involvement and nodular scleritis associated with this unusual type of pyoderma gangrenosum and discuss its differential diagnosis.

Necrotising Scleritis and Peripheral Ulcerative Keratitis Associated with Rheumatoid Arthritis Treated with Rituximab.

Background Rheumatoid arthritis is a chronic and common inflammatory autoimmune disease. This primarily involves the synovia of the joints, but can cause many extra-articular manifestations as well, including peripheral ulcerative keratitis (PUK) and necrotising scleritis. These are often a threat to vision; they significantly compromise not only the eye's structural integrity but are also important for prognosis and need urgent management. History and signs Three cases of peripheral ulcerative keratitis associated with rheumatoid arthritis were recorded in the electronic databank of the Jules Gonin Uveitis Clinic, two with necrotising scleritis and peripheral ulcerative keratitis and one with only peripheral ulcerative keratitis. They were all followed at Jules Gonin Eye Hospital (Lausanne, Switzerland), conjointly with the Department of Rheumatology at the Centre Hospitalier Universitaire Vaudois (Lausanne, Switzerland). Therapy and Outcome Good initial therapeutic response was observed in the two patients who received rituximab therapy. The patient who received only high dose corticosteroid developed massive colon perforation as well as acute renal insufficiency a few days after her ocular event. Conclusion From our limited number of patients, we found that the two patients who received the induction therapy with rituximab were stabilised from an ocular standpoint; however, rituximab had to be switched to other molecules, either due to other systemic symptoms from the disease itself or due to adverse effect of this treatment. This contributes to the increasing number of reports that rituximab can be an effective treatment for refractory ocular complications of rheumatoid arthritis (RA), at least as an induction therapy.

Idiopathic Orbital Inflammation Associated With Necrotizing Scleritis and Temporal Bone Inflammation.

The authors present a case of aggressive idiopathic orbital inflammation producing necrotizing scleritis along with synchronous tumefactive fibroinflammatory lesion of the temporal bone. A young woman with no medical history presented with sectoral scleritis and mildly reduced vision. Response to initial treatment, which included topical and systemic corticosteroids, as well as systemic nonsteroidal anti-inflammatory drugs, was limited. Over the following months, signs of orbital inflammation developed, including ptosis, proptosis, and limited extraocular motility. MRI revealed both orbital and ipsilateral temporal bone masses. An orbital biopsy was performed revealing a mixed inflammatory infiltrate, whereas a biopsy of the temporal bone mass revealed a tumefactive fibroinflammatory lesion. Biopsy showed no histopathologic evidence of infection nor neoplasm. The patient eventually responded to treatment with systemic prednisone, azathioprine, and rituximab.

[Clinical characteristics of 4 cases of scleritis associated with systemic lupus erythematosus].

Episcleritis and scleritis are relatively rare ocular diseases, which are commonly associated with rheumatic diseases including systemic lupus erythematosus (SLE). To investigate clinical and laboratory features of SLE-associated episcleritis and scleritis, we now report 4 cases of inpatients who were diagnosed with episcleritis or scleritis secondary to SLE from September 2005 to July 2016 in the Department of Rheumatology and Immunology in Peking University People's Hospital. Demographic, clinical and laboratory characteristics were summarized together with the treatment regimen and the prognosis; the literature was reviewed. There were 3 female and 1 male patients. The average age was (49.0±23.8) years and the mean duration of SLE at the onset of episcleritis or scleritis was (2.1±1.4) years. In addition to the eye involvement, the patients had mucocutaneous manifestations, serositis, lupus nephritis and interstitial pneumonia simultaneously; in the past, 1 patient experienced arthritis, 2 presented Raynaud's phenomenon, and 2 had hematologic involvement. All the patients had antinuclear antibody (ANA) of high titer. The anti double-stranded DNA (ds-DNA) antibody titers were increased in 2 patients. Three patients had positive anti-nucleosome antibody (ANuA) while the other 1 patient did not test it. The complement levels were decreased in 3 patients. The systemic lupus erythematosus disease activity index (SLEDAI) scores were more than 4 points in all the patients (ranging from 7-16), suggesting active disease. Ocular symptoms included pain, redness of the eye and tears. Ophthalmic examinations revealed 3 cases of episcleritis and 1 case of scleritis. Among the 4 patients, 2 patients experienced ocular complications including decrease in vision and uveitis. All the patients were treated with systemic corticosteroids combined with hydroxycloroquine; 3 patients were treated with immunosuppressants (cyclophosphamide in 2 patients and leflunomide in 1 patient). All of the 4 patients received topical steroid and 1 patient received periocular injection of triamcinolone acetonide; 1 patient received topical nonsteroidal anti-inflammatory drug (NSAID).No recurrence of episcleritis or scleritis was observed during the follow-ups. As a conclusion, scleritis and episcleritis, although uncommon, may occur in patients with autoimmune rheumatic diseases including SLE. The occurrence of episcleritis and scleritis may suggest active disease of SLE. Ocular complications need to be aware of in the patients. Prompt diagnosis and treatment was associated with good visual outcomes in the follow-ups.

Trigeminal autonomic cephalgia caused by recurrent posterior scleritis.

A 40-year-old woman presented with a side-locked headache with autonomic features, which then switched sides before reverting to the original side. The atypical features of side swapping, partial response to indometacin and abnormal optic disc appearances ultimately led to a diagnosis of recurrent posterior scleritis. We discuss the differential diagnosis of trigeminal autonomic cephalgias and its secondary causes, and provide practical pointers for its investigation and management.

[Posterior scleritis]. / La sclérite postérieure : cas clinique.

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.

La sclérite postérieure est une inflammation sévère et douloureuse localisée au niveau de la sclère. Cette pathologie méconnue est souvent sous-diagnostiquée en raison de son polymorphisme clinique. Il est néanmoins important de la reconnaître de façon à proposer un traitement adéquat des symptômes oculaires et de rechercher les différentes pathologies systémiques inflammatoires ou auto-immunes pouvant lui être associées.

Autoimmune lymphocytic hypophysitis in association with autoimmune eye disease and sequential treatment with infliximab and rituximab.

INTRODUCTION: Autoimmune lymphocytic hypophysitis associates predominantly with other autoimmune endocrinopathies and is most commonly treated with glucocorticoids and/or decompressive pituitary surgery. Here we report a new association and treatment modality for lymphocytic hypophysitis. METHODS: A 52-year-old woman presented with scleritis, uveitis, facial palsy, and central diabetes insipidus, accompanied by thickened pituitary stalk and enlarged pituitary on cranial MRI. Neurosarcoidosis was suspected and treatment with glucocorticoids and methotrexate initiated. Since symptoms persisted, infliximab (a monoclonal antibody that antagonizes tumor necrosis factor alpha) was added to her regimen. The patient initially improved but after 6 months developed recurrent pituitary enlargement, bilateral optic neuritis, and panhypopituitarism. To ascertain the nature of the pituitary lesion, she underwent transsphenoidal biopsy, which revealed lymphocytic hypophysitis with numerous CD20 positive B lymphocytes. The pathological finding suggested to us that administration of rituximab (a monoclonal antibody that lyzes B cells expressing CD20) could be useful. Following two courses of rituximab, the pituitary mass resolved and the corticotroph axis partially recovered. The patient has remained in remission during 3 years of follow up. CONCLUSION: This is the first report of hypophysitis occurring with the triad of scleritis, uveitis, and optic neuritis, as well as the first immunotherapy based on the sequential use of infliximab and rituximab.

Posterior scleritis with retinal pigment epithelium rip: an unusual presentation.

Posterior scleritis is a great mimicker and can cause irreversible visual loss because of late or misdiagnosis. We report a case of retinal pigment epithelial rip in the event of nodular posterior scleritis that is hardly reported in the literature. The authors hypothesize the rip to be a result of inflammation, exudation and continuing pressure by the fluid or granuloma on the pigment epithelium.

Five-year outcome in immune-mediated scleritis.

BACKGROUND: Immune-mediated scleritis is a rare condition, and the information on the clinical course and complications is scarce. The aim of this study was to identify prognostic factors, complications, and therapeutic effects in patients with immune-mediated scleritis. METHODS: Patients with diagnosis of scleritis and a follow-up time of 5 years were identified. Systemic disease, laboratory investigations, type of scleritis, disease activity, therapy, and complications were recorded. The study design was a retrospective, non-comparative, interventional case series. RESULTS: Systemic disease was identified in 15 (37%) patients at presentation and in 18 (45%) after 5 years. Rheumatoid arthritis (15%), granulomatosis with polyangiitis (7.5%), and polychondritis (7.5%) were the most predominant disorders. Persistent scleritis (>5 years) was associated with systemic disease (66 vs. 6%; p < 0.05) and positive auto-antibodies (48 vs. 23%; p = 0.18). Control of ocular inflammation was achieved in 38 of 40 (95%). Prednisone (14 patients) and/or methotrexate (8) were the predominant drugs to control persistent disease. Complications included interstitial keratitis (2), inflammatory astigmatism (2), corneal melt (3), macular edema (6), and severe systemic disease (5). CONCLUSION: The presence of systemic disease and positive auto-antibodies are associated with persistent scleritis. Immunosuppressive agents allow control of scleritis, but may contribute to severe systemic complications.

Cytomorphological changes and susceptibility of clinical isolates of Acanthamoeba spp. to heterocyclic alkylphosphocholines.

The treatment of diseases caused by pathogenic strains of Acanthamoeba spp. is to date limited and frequently unsuccessful. Alkylphosphocholines (APCs) are promising agents with interesting results of antiparasitic activity in experimental and clinical conditions. In the present study susceptibilities of two clinical isolates of Acanthamoeba spp. to four heterocyclic APCs were investigated. The isolates showed high degrees of susceptibility to studied APCs and all the tested concentrations inhibited the growth with the highest concentrations of 500-1000µM causing 100% eradication of the trophozoites and cysts. The highest susceptibility was noted in IF16-P-4-Pip with EC50 values of 28.62-43.73µM, and EC90 values of 30.70-63.16µM after 48h of incubation. The cytomorphological changes of trophozoites after the exposure to APCs included rounding up of cells, resorption of acanthopodia and subsequent lysis. The remains of cells were typical with oval shape and identifiable nucleus. After the application of IF16-P-4-Pip, IF16-P-2-MetPip, and IF16-P-Azep, at concentrations of 62.5-125µM to trophozoite suspension, a formation of pseudocysts was detected. The single-layered coat covering the surface of pseudocyst stained positively with a fluorescence brightener, Rylux. Destroyed cysts were characteristic with shrinkage of the cytoplasm and separation of the cytoplasmic membrane from the endocyst. IF16-P-2-MetPip at the highest concentration formed large spherical vesicles which frequently enclosed inactivated cysts. Heterocyclic APCs used in the study demonstrated strong amoebicidal activity and the cytotoxic effect of IF16-P-4-Pip similar to that of miltefosine indicates its possible therapeutic potential.

Ahmed glaucoma valve surgery for necrotizing scleritis with secondary glaucoma.

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.

[Study on the clinical manifestation of 90 cases with scleritis and episcleritis].

OBJECTIVE: To evaluate the demographic characteristics, clinical features, ocular complications, and disease associations of patients who were diagnosed as scleritis and episcleritis in clinic. METHODS: A retrospective case series study.Ninety patients were diagnosed clinically as scleritis and episcleritis in Beijing Tongren Hospital from Jan 2010 to Jan 2013. Analyzed the general states of health, case history, the clinical manifestation, clinical features, ocular complications (decrease in vision, anterior uveitis, keratitis, ocular hypertension, cataract, and diseases of retina), and disease associations, to summarize the clinical characteristics of scleritis and episcleritis. RESULTS: Ninety patients were recorded, from 4 to 83 years old, and the average was (48 ± 15) years old. 30 cases (33.3%) were male, and 60 (66.7%) were female. There were no significant difference between male and female (χ² = 10.000, P < 0.01).In the 90 cases, 36 (40.0%) were episleritis and 54 cases (60.0%) were scleritis. Children group (0-16 years old) had 2 cases (2.2%), which all were episleritis.Young adult group (17-44 years old) had 39 cases (43.4%), including 13 were episleritis and 26 cases were scleritis. Middle adult group (45-59 years old) had 30 cases, including 9 cases episleritis and 21 cases scleritis. There were 19 cases (21.2%) in aged group ( ≥ 60 years), including 12 cases episleritis and 7 scleritis.Young adult group, Middle adult group and Aged group were significantly more than Children and aged group (χ² = 33.390, 24.500 and 13.762, P < 0.01).Young group was significantly more than Aged group Children group (χ² = 6.889, P < 0.01). Ocular complications were more frequent overall in patients with scleritis versus in those with episcleritis (χ² = 30.044, P < 0.01) , including decrease in vision, keratitis, and ocular hypertension (χ² = 130.200, 67.200, 54.444, P < 0.01) .Nineteen cases (21.1%) had different types of arthritis, and 10 cases (11.1%) had infection diseases in local. There were difference between episcleritis and scleritis (χ² = 145.644, P < 0.01) . CONCLUSIONS: Scleritis occurs in all ages, but mainly in adults.It is uncommon, and potentially life-threatening diseases. It is needed pay attention to diagnosis and treatment of the patients with systemic disease and ocular complications by Ophthalmologist.

A Case of Refractory Posterior Scleritis with Marked Retinochoroidal Detachment Associated with Panuveitis.

An 84-year-old man presented with decreased right-eye visual acuity. Upon initial examination, the rightand left-eye visual acuities were 0.03 and 1.2, respectively; moreover, the right- and left-eye intraocular pressure was 12 mmHg and 13 mmHg, respectively. Examination revealed a shallow anterior chamber of the right eye, anterior chamber inflammation, vitreous opacity, and marked retinochoroidal detachment. Optical coherence tomography (OCT) revealed retinal detachment (RD) and choroidal folds; moreover, B-scan ultrasonography (B-scan) showed RD as well as thickened sclera with fluid in Tenon's space. Fluorescent fundus angiography revealed hyperfluorescence in the optic disc and vascular hyperpermeability in the right eye. The left eye lacked extra-ocular symptoms or abnormalities. The right ocular axis measured 23.4 mm with no apparent subretinal fluid migration due to positional changes. Accordingly, the patient was diagnosed with panuveitis associated with posterior scleritis and immediately started on 40 mg prednisolone, which improved his symptoms. However, at 3 post-treatment months, choroidal folds were observed and was restarted on 20 mg prednisolone. The choroidal folds subsequently disappeared, with a current visual acuity of 0.3 in the right eye and no recurrence. Our findings indicated the utility of accurate diagnosis of posterior scleritis by B-scan and prompt systemic steroid administration.