Uveitis associated with juvenile idiopathic arthritis (JIA) typically involves the anterior chamber segment, follows an indolent chronic course, and presents a high rate of uveitic complications and a worse outcome as compared to other aetiologies of uveitis. Disease assessment, treatment, and outcome measures have not been standardized. Collaboration between pediatric rheumatologists and ophthalmologists is critical for effective management and prevention of morbidity, impaired vision, and irreparable visual loss. Although the Standardization of Uveitis Nomenclature Working Group recommendations have been a great advance to help clinicians to improve consistency in grading and reporting data, difficulties arise at the time of deciding the best treatment approach in the individual patient in routine daily practice. For this reason, recommendations for a systematized control and treatment strategies according to clinical characteristics and disease severity in children with JIA-related uveitis were developed by a panel of experts with special interest in uveitis associated with JIA. A clinical management algorithm organized in a stepwise regimen is here presented.
Tamoxifen (TMX) has been related with the development of uterine sarcomas. Since the first reported case in 1988, 65 TMX-related cases have been referred to. Here we present three new cases of uterine sarcomas in patients with breast cancer treated with TMX and we comment on the outcome of the cases described in the literature. In the past 25 years, 60 uterine sarcomas have been diagnosed and treated in Hospital Clínic. Three patients have previously received TMX 20 mg/day for 3, 5, and 7 years for breast cancer. Uterine sarcoma appeared 5, 5, and 7 years, respectively, after the start of TMX treatment, and all of them had stage I (FIGO) disease. Two patients had a carcinosarcoma and one patient had an adenosarcoma. After treatment, the disease progressed in two patients and the third patient is alive having a follow-up of 42 months. The low incidence of uterine sarcomas makes it difficult to establish a relationship with TMX. Nevertheless, looking at the literature data, 20 mg/day of TMX over 1 year could be enough to develop uterine sarcoma; the sarcoma appears mainly during the first 8 years and seem to behave more aggressively. Although only 65 cases have been reported in the past 14 years, a strict follow-up is necessary in patients with breast cancer receiving TMX therapy.
To determine the efficacy of infliximab treatment in refractory posterior uveitis in Behçet's disease after withdrawal of infusions. Four patients with posterior uveitis secondary to Behçet's disease were treated with infliximab until complete remission and were followed after withdrawal of infusions. Intra-ocular inflammation was assessed using the binocular indirect ophthalmoscopy score, best-corrected visual acuity (BCVA) and foveal thickness measured by optic coherence tomography (OCT). All the patients included in the study were treated with infliximab for a minimum of 12 months and were in complete remission. None of the patients were taking steroids or immunosuppressants. Main follow-up after withdrawal of infusions was 7.5 months. Two out of four patients (50%) maintained complete remission of posterior uveitis. BCVA was stable in seven eyes. OCT showed worsening in macular edema in the two eyes of the patients with reactivation. Infliximab is an efficient long-term treatment for refractory posterior uveitis. Repeated infusions are required to maintain long-term remission which may be sustained on discontinuation of the drug.
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