Primary intraocular lymphoma: improving the diagnostic procedure.

OBJECTIVE: To analyze the clinical features of primary intraocular lymphoma (PIOL) and to describe cytochemical and immunocytochemical findings of the vitreous specimens as well as the reasons for delayed diagnosis of PIOL. DESIGN: Prospective noncomparative study. PARTICIPANTS: Eleven patients referred to the uveitis or medical retina units, Department of Ophthalmology, University of Helsinki, were diagnosed as having PIOL between 2000 and 2005. The median follow-up of the patients was 32 months. METHODS: Clinical features and diagnostic workup of uveitis were described. Twelve vitrectomies were performed on 9 patients. The first 5 biopsies were fixed in an equal volume of 50% alcohol. The specimens of the next 7 vitrectomies were handled without alcohol, and tissue culture medium was added to the samples. MAIN OUTCOME MEASURES: Clinical features of PIOL, intervals from ocular symptoms and from first ophthalmological examination to diagnosis, and the role of a proper handling of the vitreous sample in the diagnosis of PIOL. RESULTS: Six females (54%) and 5 males (46%) (median age, 61 years) were included. Ten patients had ocular symptoms for 1 to 30 months (median, 8) before the first contact with an ophthalmologist. Uveitis was bilateral in 9 patients. Vitreitis was seen in all patients, and it was severe in 8. Fundus lesions dominated in 3 patients. Six patients lost useful vision in one eye before the diagnosis of PIOL. Cytologic and immunohistochemical stainings prepared of the unfixed vitreous specimens showed PIOL in 6 patients. The samples fixed in alcohol were nondiagnostic in 4 patients, and in them, verification of diagnosis was based on brain biopsy (3) or cerebrospinal fluid (1) findings. Seven patients died due to primary nervous system lymphoma. CONCLUSIONS: Diagnosis of PIOL is difficult but can be improved. Severe bilateral vitreitis in an elderly patient is a characteristic finding of PIOL. Alcohol fixation may jeopardize the identification of PIOL cells in the vitreous sample. Optimal handling of the vitreous specimens and examination of the slides by an experienced cytopathologist are critical in the diagnostic workup of PIOL.

Recent advances in uveitis of juvenile idiopathic arthritis.

Chronic scarring-type uveitis is a frequent extra-articular manifestation of juvenile idiopathic arthritis. It occurs in about 20% of children with this disease, commencing typically within a few years from its onset. The risk of uveitis is greatest in antinuclear antibody-positive girls with early onset oligoarthritis. The classic clinical picture is chronic bilateral anterior uveitis, usually asymptomatic until substantial damage to intraocular structures occurs. In view of the asymptomatic nature of the condition, routine screening of juvenile idiopathic arthritis patients 2-4 times a year is crucial to prevent complications. The treatment consists of topical corticosteroids and mydriatics, in severe cases with immunosuppressive agents, and surgical management of complications. Although the prognosis of uveitis is improving, there are cases refractory to standard regimens. Patients in whom uveitis commences prior to the onset of arthritis present a special problem.

Involvement of intraocular structures in disseminated histoplasmosis.

PURPOSE: To describe ocular involvement and response to treatment in a patient with human immunodeficiency virus (HIV) infection with severe progressive disseminated histoplasmosis (PDH). METHODS: We report a 35-year-old HIV-infected patient seen in our clinics over a period of 4 years. During antiretroviral treatment (ART), the HIV load became undetectable at 3 months; however, CD4 T-cell count increased slowly and rose to 100 cells/microl. Histoplasma capsulatum was cultured from skin pustules, cerebrospinal fluid (CF) and aqueous humour. RESULTS: The patient developed central nervous system (CNS) involvement 2 months and panuveitis in both eyes 4 months after the initiation of ART. With intravenous liposomal amphotericin B followed by oral voricanozole, the chorioretinal lesions of the right eye (RE) became inactivated and magnetic resonance imaging (MRI) lesions of CNS disappeared. Relapse of the inflammation in the anterior segment of the left eye (LE) resulted in a total closure of the chamber angle and severe glaucoma. Despite medical therapy, two cyclophotocoagulations, total vitrectomy and repeated intravitreal amphotericin B injections, LE became blind. Histoplasma capsulatum was cultured from the aqueous humour after antifungal therapy of 16 months' duration. CONCLUSION: PDH with intraocular and CNS manifestations was probably manifested by an enhanced immune response against a previous subclinical disseminated infection. It seems difficult to eradicate H. capsulatum from the anterior segment of the eye in an immunocompromised patient.

Neurosarcoidosis mimicking Sjögren's syndrome.

PURPOSE: To describe a patient with a long disease history who was finally diagnosed with neurosarcoidosis and to discuss the reasons behind the delayed diagnosis. CASE REPORT: A 58-year-old man with sick sinus syndrome and bradycardia, which was treated with a pacemaker, developed first right and then left facial palsy. Subsequently, multiple cranial nerve palsies developed and later spontaneously resolved. Neurosarcoidosis was suspected at that stage, but excluded because the patient had no typical sarcoid lung changes, his serum and cerebrospinal fluid angiotensin converting enzyme activity levels were normal and a computed tomography scan disclosed no central nervous system changes. During follow-up, the patient developed extremely dry eyes and mouth, suggesting Sjögren's syndrome. Rheumatology consultation did not reveal any autoimmune or visceral features typical of Sjögren's syndrome and autoantibodies were negative. However, both labial salivary gland and conjunctival biopsies revealed non-caseating granulomas, and neurosarcoidosis was diagnosed. CONCLUSIONS: Neurosarcoidosis is a relatively rare disease with a somewhat poor longterm prognosis in one-third of cases, although the neurological manifestations often diminish or disappear in response to glucocorticoid treatment. Diagnosis is often a clinical challenge, especially in the absence of pulmonary changes or other features typical of sarcoidosis. The labial salivary gland and conjunctiva provide helpful biopsy sites for histopathological confirmation of the diagnosis.

High dose intravenous methylprednisolone pulse therapy versus oral prednisone for thyroid-associated ophthalmopathy.

PURPOSE: To compare the effectiveness of intravenous (i.v.) methylprednisolone pulse therapy and oral prednisone when used as the initial treatment of patients with mild or moderate thyroid-associated ophthalmopathy. METHODS: Thirty-three consecutive patients with thyroid-associated ophthalmopathy in Helsinki and Turku University Hospitals were randomly assigned either i.v. methylprednisolone pulse therapy (group A, n = 18) or oral prednisone (group B, n = 15). Treatment outcomes were measured by subjective changes in the grade of diplopia and quantitatively in several ophthalmic variables at 3 and 12 months. Any decision to proceed with additional treatment at 3 months was made on clinical grounds. The study was open in respect of both the initial treatment and the need for additional therapy. RESULTS: No significant differences in the grade of diplopia, proptosis or soft tissue activity scores were noted between groups A and B from 0 to 3 months. However, group A required additional forms of therapy at 3 months less frequently than did group B (p = 0.038). CONCLUSIONS: Our data suggest that i.v. methylprednisolone pulse therapy and oral prednisone are equally effective as initial treatments for thyroid-associated ophthalmopathy where diplopia, proptosis and signs of soft tissue inflammation are concerned. When additional treatment is required, i.v. methylprednisolone pulse therapy may be more effective than oral prednisone. However, the study's limitations meant that any decision to give additional treatment after the initial therapy was made on clinical grounds.