Gender and Ocular Manifestations of Connective Tissue Diseases and Systemic Vasculitides

Ocular manifestations are present in many connective tissue diseases which are characterized by an immune system that is directed against self. In this paper, we review the ocular findings in various connective tissue diseases and systemic vasculitides and highlight gender differences in each disease. In rheumatoid arthritis, we find that dry eyes affect women nine times more than men. The other extra-articular manifestations of rheumatoid arthritis affect women three times more commonly than men. Systemic lupus erythematosus can involve all ocular structures and women are nine times more affected than men. Systemic sclerosis is a rare disease but, again, it is more common in women with a female to male ratio of 8 : 1. Polymyositis and dermatomyositis also affect women more commonly than men but no gender differences have been found in the incidence or disease course in the systemic vasculitides associated with antineutrophil cytoplasmic antibody such as granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis). Finally, Behcet’s disease is more common in males, and male gender is a risk factor for Behcet’s disease. There is a slight female preponderance in sarcoidosis with female gender carrying a worse prognosis in the outcome of ocular disease

Relentless placoid chorioretinitis: A review of four cases in pediatric and young adult patients with a discussion of therapeutic strategies

IntroductionRelentless placoid chorioretinitis (RPC) is a rare, bilateral disease of the retinal pigment epithelium. The clinical course is prolonged and relapsing. No standard treatment has been established to date. The purpose of this case series is to report four cases of RPC in pediatric and young adult patients in which varying treatments were used, comparing them to previously published cases.MethodsA literature review was conducted to investigate currently published presentations and treatment options for RPC. A multicenter retrospective chart review was also performed on four consecutive patients. These patients were diagnosed with RPC because of new chorioretinitis lesions continuing to appear without or despite therapy for 5–36 months (2 patients), with a clinical course prolonged and relapsing, or because of the atypical location of the multiple lesions (>50) extending from the posterior pole to the equator and mid-peripheral retina (all four patients), which were not consistent with other entities like acute posterior multifocal placoid pigment epitheliopathy and serpiginous choroiditis.ResultsAll four cases of RPC received oral or IV steroids acutely, and three of these patients were transitioned to a steroid-sparing agent and biologic therapy: anti-TNF alpha or anti-IL-6. Quiescence of the chorioretinitis lesions was obtained after 7 months, 1 month, and 36 months; however, the latter had issues with treatment adherence. Mycophenolate mofetil was insufficient to control the disease in one patient, but tocilizumab and infliximab thereafter were effective after cessation of adalimumab due to side effects. Adalimumab when started the first month after the presentation was effective in controlling the disease in one patient. After the failure of interferon-alpha-2a, one patient displayed long-term control with infliximab. One patient did not require a steroid-sparing agent after oral prednisone taper as there was no evidence of progression or recurrence.ConclusionThis case series adds to the current knowledge regarding potential treatments for RPC, specifically the use of anti-TNF-alpha treatment and anti-IL-6 tocilizumab. In this case study, relapses of RPC were found among patients on mycophenolate mofetil and interferon-alpha-2a, and one case did not relapse on oral steroids without a steroid-sparing agent. Our findings suggest that adalimumab, infliximab, and tocilizumab may be useful medications to obtain quiescence of RPC

Uveítes anteriores associadas a doenças sistêmicas Anterior uveitis associated to systemic diseases

As uveítes anteriores são caracterizadas pela inflamação preponderante do segmento anterior do olho. Hiperemia conjuntival, reação de câmara anterior com células e "flare", precipitados ceráticos e sinéquias posteriores são sinais que compõem o quadro inflamatório. Aspectos clínicos de algumas doenças sistêmicas envolvidas na etiologia das uveítes anteriores serão abordados neste artigo.<br>Anterior uveitis describes a disease predominantly limited to the anterior segment of the eye. Conjunctival hyperemia, anterior chamber cell and flare, keratic precipitates, and iris abnormalities including posterior synechiae characterize the inflammation. This article discusses clinical aspects of the systemic diseases that cause anterior uveitis with review of the literature

Differential Effectiveness of Etanercept and Infliximab in the Treatment of Ocular Inflammation

Anti–tumor necrosis factor α (anti–TNF-α) agents are being used increasingly in refractory inflammatory eye diseases. We reviewed our patients on etanercept and infliximab to determine whether these medications are equally efficacious in controlling ocular inflammation. Exploratory retrospective analysis. Patients with ocular inflammatory disease on an anti–TNF-α agent (etanercept, infliximab). Case records of 22 patients treated with anti–TNF-α therapy were reviewed for demographic information, ocular and systemic diagnosis, duration and dose of anti–TNF-α treatment, concomitant ocular and systemic immunosuppressive medications, and treatment response. Uveitis recurrence rate, initial treatment response, treatment response, and medication use at 6 months, 1 year, and last visit. Patients treated with infliximab had a significant decrease in uveitis recurrences after starting therapy compared with those treated with etanercept (59% vs. 0%, P = 0.004). One year after treatment initiation and at final visit, more infliximab-treated patients had an improvement in their ocular inflammation (100% vs. 33%, P = 0.002, and 94% vs. 0%, P<0.001, respectively) and a decreased requirement for topical prednisolone acetate 1% (94% vs. 33%, P = 0.009, and 89% vs. 29%, P = 0.007, respectively) compared with those treated with etanercept. No significant differences in the use of oral corticosteroids and immunosuppressive agents were noted between the 2 groups at 6 months, 1 year, and final visit. Infliximab was more effective than etanercept in the treatment of recalcitrant uveitis and decreased the use of topical steroids

Visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus.

PurposeTo report a case of visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus.ObservationsA 15-year-old boy with a known diagnosis of systemic lupus erythematosus and a clinically significant anti-phospholipid panel presented with sudden vision loss in the left eye. Examination and ocular imaging revealed signs of vaso-occlusive retinopathy. The patient was immediately started on high dose intravenous steroids, followed by mycophenolate mofetil. He remained on aspirin. After showing no improvement in retinal arteriole and capillary perfusion he was started on therapeutic anti-coagulation with enoxaparin. He regained 20/20 vision. Intravenous fluorescein angiography demonstrated reperfusion of retinal arterioles. Optical coherence tomography angiography showed return of flow in the capillary networks.ConclusionsWe present a case of vaso-occlusive retinopathy in a patient with known systemic lupus erythematosus and a clinically significant anti-phospholipid panel, thus meeting criteria for anti-phospholipid syndrome. He was treated with intravenous methylprednisolone, mycophenolate motefil, aspirin, and enoxaparin. The patient not only had great recovery of visual acuity, but also demonstrated reperfusion of arterioles and reconstitution of flow in the retinal capillary network. These findings suggest that the vaso-occlusive disease is reversible if the diagnosis is made promptly and intensive therapy is initiated.ImportanceCurrently there are no reported cases of vaso-occlusive retinopathy from APLS and SLE with visual recovery, reperfusion, and return of capillary flow