Rheumatic diseases are associated various extra-articular manifestations, such as eye, nerve, pericardium, and pleura. The anterior part of the eye has distinguished anatomic structure resembling synovial joints, thus it is a common site of ocular manifestation in rheumatic disease. These changes include sicca syndrome, peripheral ulcerative keratitis, episcleritis, scleritis, and iritis. Some of these findings may be the clues for the diagnosis of the rheumatic diseases, and some ocular manifestations may represent the activity or prognosis of the rheumatic diseases. These ocular complications may leads to severe visual loss. It is crucial to rule out underlying systemic diagnosis in rheumatic disease associated ocular diseases, and when diagnosed, the coordination of the rheumatologist with the ophthalmologist in the treatment is imperative.
Corneal Ulcer ; Diagnosis ; Eye Diseases* ; Iritis ; Joints ; Pericardium ; Pleura ; Prognosis ; Rheumatic Diseases* ; Scleritis ; Sjogren's Syndrome

No abstract available.
Adult ; Female ; Humans ; Recurrence ; Retina/*pathology ; Scleritis/*complications/diagnosis ; Tomography, Optical Coherence ; Uveomeningoencephalitic Syndrome/complications/*diagnosis

A 52 year-old, contact lens-wearing man presented with progressive right eye pain and redness for one month. He had been evaluated and treated for necrotizing scleritis by multiple eye care specialists prior to presentation. He underwent a complete systemic work-up for both autoimmune and infectious causes of scleritis, including a culture. The culture revealed heavy growth of Nocardia asteroides complexes. The patient was treated with topical amikacin and oral Bactrim. Following several weeks of antibiotic treatment, the patient's infection resolved completely, and his visual acuity returned to baseline status. Nocardia is a rare but potentially devastating cause of necrotizing scleritis that may affect contact lens wearers without an associated keratitis. Prompt recognition and early treatment with appropriate antimicrobial agents are critical to achieve a favorable outcome.
Anti-Bacterial Agents/therapeutic use ; Contact Lenses/*adverse effects/*microbiology ; Humans ; Male ; Middle Aged ; Nocardia Infections/*diagnosis/drug therapy ; Nocardia asteroides/*isolation & purification ; Scleritis/drug therapy/*microbiology

PURPOSE: To report a rare case of episcleritis as the only manifestation of neurosyphilis. CASE SUMMARY: A 40-year-old female was referred to the outpatient clinic because of right episcleritis that was unchanged during the month of treatment. Her headache persisted, and slit lamp examination showed tortuous congestion of engorged episcleral vessels with swelling in the superior-temporal region of the right eye, but fundus and radiological studies showed normal findings. Serological tests were reactive for venereal disease research laboratory test, treponema pallidum hemagglutination assay test, and fluorescent treponemal antibody absorption test. Under the suspicion of persistent syphilis infection, cerebrospinal fluid examination was performed, and the diagnosis of neurosyphilis with episcleritis was diagnosed. Treatment consisted of intravenous injections of 5 million IU penicillin G potassium every 4 hours for 14 days. The ocular inflammation resolved within the first week of treatment and did not recur. CONCLUSIONS: We report a case of intractable episcleritis that required examination for syphilitic infection using serological and CSF tests, and the appropriate antimicrobial therapy for syphilis with follow-up examinations.
Adult ; Ambulatory Care Facilities ; Cerebrospinal Fluid ; Diagnosis ; Estrogens, Conjugated (USP) ; Female ; Fluorescent Treponemal Antibody-Absorption Test ; Headache ; Hemagglutination ; Humans ; Inflammation ; Injections, Intravenous ; Neurosyphilis* ; Penicillin G ; Scleritis* ; Serologic Tests ; Sexually Transmitted Diseases ; Syphilis ; Treponema pallidum

We report a case of posterior scleritis effectively managed with intravitreal bevacizumab. A 71-year-old woman was diagnosed with posterior scleritis. Although she was initially treated with systemic steroids, her clinical presentation deteriorated. She was then treated with a single intravitreal injection of bevacizumab and aqueous humor collection. The aqueous level of vascular endothelial growth factor prior to the intravitreal injection was 880.51 pg/mL, greater than that in the healthy control group (p < 0.001). One month later, the scleritis was completely resolved, and the patient remained stable during six months of follow-up. Intravitreal bevacizumab appears to be an effective adjuvant therapy for patients with posterior scleritis.
Aged ; Angiogenesis Inhibitors/administration & dosage ; Antibodies, Monoclonal, Humanized/*administration & dosage ; Aqueous Humor/metabolism ; Diagnosis, Differential ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Fundus Oculi ; Humans ; Interleukin-8/metabolism ; Intravitreal Injections ; Microscopy, Acoustic ; Scleritis/*drug therapy/metabolism/pathology ; Vascular Endothelial Growth Factor A/metabolism

We report on a case of necrotizing keratoscleritis in primary Sjogren's syndrome. A 66-year-old female patient who was complaining of ocular pain, tearing and decreased vision in her right eye for the previous two days was admitted to our hospital. Visual acuity in the right eye was hand movement, and initial examination showed a 3.0 x 1.8 mm uveal mass bulging through a corneoscleral melting site in the nasal region of the right eye. Positive anti-nuclear antibody was identified at a titer of 1:320 with a speckled pattern, and both Sjogren's syndrome A and Sjogren's syndrome B antibody tests were positive, with titers >200 U/mL. A technetium 99m pertechnetate salivary scan revealed chronic sialoadenitis in the submandibular glands. We diagnosed the lesion as necrotizing keratoscleritis due to primary Sjogren's syndrome. A corneoscleral patch graft was performed, followed by immunosuppression including oral cyclosporin and topical prednisolone. During a follow-up period of 12 months, the corneoscleral graft was well maintained with no recurrence.
Aged ; Diagnosis, Differential ; Disease Progression ; Female ; Follow-Up Studies ; Glucocorticoids/administration & dosage ; Humans ; Keratitis/drug therapy/*etiology/pathology ; Necrosis ; Ophthalmic Solutions ; Scleritis/drug therapy/*etiology/pathology ; Sjogren's Syndrome/*complications/drug therapy/pathology ; Visual Acuity

A 68-year-old woman presented with pain in her left eye. Necrosis with calcium plaques was observed on the medial part of the sclera. Aspergillus fumigatus was isolated from the culture of the necrotic area. On systemic work-up including serum and urine electrophoresis studies, the serum monoclonal protein of immunoglobulin G was detected. The patient was diagnosed with monoclonal gammopathy of undetermined significance and fungal scleritis. Despite intensive treatment with topical and oral antifungal agents, scleral inflammation and ulceration progressed, and scleral perforation and endophthalmitis developed. Debridement, antifungal irrigation, and tectonic scleral grafting were performed. The patient underwent a combined pars plana vitrectomy with an intravitreal injection of an antifungal agent. However, scleral and intraocular inflammation progressed, and the eye was enucleated. Aspergillus fumigatus was isolated from the cultures of the eviscerated materials. Giemsa staining of the excised sclera showed numerous fungal hyphae.
Aged ; Amphotericin B/administration & dosage ; Antifungal Agents/administration & dosage ; *Aspergillosis/therapy ; *Aspergillus fumigatus ; Disease Progression ; Eye Enucleation ; Female ; Humans ; Injections, Intraocular ; Paraproteinemias/*complications ; Sclera/pathology/ultrasonography ; Scleritis/*complications/diagnosis/*microbiology/physiopathology ; Vitrectomy

The ocular manifestations of rheumatoid arthritis (RA) are common and they can vary from patient to patient. However, necrotizing anterior scleritis without inflammation (scleromalacia perforans) is a rare and serious opthalmic complication, and it is typically associated with long-standing RA. Although the etiology and pathogenesis of scleromalacia perforans are diverse and they are not completely understood, ophthalmic surgery is one of the well known causes of scleromalacia perforans. Patients with systemic autoimmune disease such as RA have an especially higher risk of scleromalacia perforans after opthalmic surgery. Because scleromalacia perforans is a potential threat not just to eyesight, but to life as well, early diagnosis and prompt treatment are required for its successful management. We experienced a case of scleromalacia perforans that developed after scleral excision of pterygium in a 58 year old woman who had a 7 year history of RA, and this was well treated with an early screral graft. We report here on this case along with a review of the relevant literature.
Arthritis, Rheumatoid ; Autoimmune Diseases ; Early Diagnosis ; Female ; Humans ; Inflammation ; Polyenes ; Pterygium ; Scleritis ; Transplants

A case of idiopathic hypertrophic cranial pachymeningitis (IHCP) misdiagnosed as an acute subdural hematoma is reported. A 37-year-old male patient presented with headache following head trauma 2 weeks earlier. Computerized tomography showed a diffuse high-density lesion along the left tentorium and falx cerebri. Initial chest X-rays revealed a small mass in the right upper lobe with right lower pleural thickening, which suggested lung cancer, such as an adenoma or mediastinal metastasis. During conservative treatment under the diagnosis of a subdural hematoma, left cranial nerve palsies were developed (3rd and 6th), followed by scleritis and uveitis involving both eyes. Magnetic resonance imaging (MRI) revealed an unusual tentorium-falx enhancement on gadolinium-enhanced T1-weighted images. Non-specific chronic inflammation of the pachymeninges was noticed on histopathologic examination following an open biopsy. Systemic steroid treatment was initiated, resulting in dramatic improvement of symptoms. A follow-up brain MRI showed total resolution of the lesion 2 months after steroid treatment. IHCP should be included in the differential diagnosis of subtentorial-enhancing lesions.
Adenoma ; Adult ; Biopsy ; Brain ; Cranial Nerve Diseases ; Craniocerebral Trauma ; Diagnosis, Differential ; Eye ; Follow-Up Studies ; Headache ; Hematoma ; Hematoma, Subdural ; Hematoma, Subdural, Acute ; Humans ; Inflammation ; Lung Neoplasms ; Magnetic Resonance Imaging ; Male ; Meningitis ; Neoplasm Metastasis ; Scleritis ; Thorax ; Uveitis

PURPOSE: We report a case of orbital pseudotumor developed in an anophthalmic socket, presenting no typical symptoms or signs. METHODS: A 67-year-old woman was referred for treatment of necrotizing scleritis of her left eye and painful orbital pseudotumor of her right eye. The right eye had been removed 10 years previously. There was an irregular mass in superomedial portion of anterior orbit. Orbital MRI showed poorly defined T1 iso and low T2 signal intensity of a lesion in the medial anterior portion of the right orbit. Because systemic steroid administration was limited, 2 mg of betamethasone was injected locally, after which the size of the lesion was decreased and the orbital pain improved. CONCLUSIONS: The diagnosis of an orbital pseudotumor developed in an anophthalmic socket may be difficult because of the lack of typical eyeball-related signs or symptoms such as proptosis, red eye, or decreased vision. But, the easy access to the lesion can provide early and effective treatment through direct injection of glucocorticoid.
Aged ; Anophthalmos* ; Betamethasone ; Diagnosis ; Exophthalmos ; Female ; Humans ; Inflammation* ; Magnetic Resonance Imaging ; Orbit* ; Orbital Pseudotumor ; Scleritis