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Abstract Prolonged steroid treatment has a suppressive effect on the immune system, however, its effect on the cellular response to mRNA vaccine is unknown. Here we assessed the impact of prolonged steroid treatment on the T-cell and humoral response to the SARS-CoV-2 spike (S) peptide following the third dose of the BNT162b2 vaccine in systemic autoimmune rheumatic disease patients. We found that CD4 T-cell response to the S peptide in patients on high-dose long-term steroid treatment showed significantly less S-peptide specific response, compare to low-dose or untreated patients. Remarkably, these results were not reflected in their humoral response, since almost all patients in the cohort had sufficient antibody levels. Moreover, S-peptide activation failed to induce significant mRNA levels of IFNγ and TNFα in patients receiving high-dose steroids. RNA-sequencing datasets analysis implies that steroid treatments' inhibitory effect of nuclear factor kappa-B signaling may interfere with the activation of S-specific CD4 T-cells. This reveals that high-dose steroid treatment inhibits T-cell response to the mRNA vaccine, despite having sufficient antibody levels. Since T-cell immunity is a crucial factor in the immune response to viruses, our findings highlight the need for enhancing the efficiency of vaccines in immune-suppressive patients, by modulation of the T-cell response.

Behçet disease (BD) is a multisystemic disease that commonly involves the eyes. Although it affects patients in all age groups, data on ocular disease by age of onset are limited. This retrospective, multicenter study aimed to compare epidemiology, systemic and ocular manifestations, treatments and outcomes between three age groups: juvenile (n = 25, 14.3%), adult-onset (n = 120, 68.6%) and late-onset (n = 30, 17.1%). Most patients in all groups were male. Systemic manifestations were similar in all groups. Systemic co-morbidities were more common in late-onset patients. Bilateral panuveitis was the most common ocular manifestation in all patients. Non-occlusive retinal vasculitis, peripheral vessel occlusions, cataract and elevated intraocular pressure were found more commonly among juvenile-onset eyes. Anterior uveitis and macular ischemia were most common among late-onset eyes, while branch retinal vein occlusion was most common in adult and late-onset eyes. All patients were treated with corticosteroids. Methotrexate, immunomodulatory combinations and biologic treatments were more commonly used for juvenile-onset patients. All groups had a similar visual outcome. Our study showed that patients with ocular BD have varied ocular manifestations and require different treatments according to age of disease onset, but visual outcome is similar.

Purpose: To report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cranial nerve (CN) III palsy. Observations: A 20-year-old woman developed bilateral anterior uveitis, which resolved with topical steroids. Three weeks later she exhibited posterior pole lesions in both eyes, corresponding with a diagnosis of APMPPE, as confirmed by multimodal imaging. Two days later the patient presented with right CN III palsy. The patient was started on oral prednisone, which was gradually tapered off. Signs and symptoms improved rapidly, with complete resolution within two months. Conclusion and importance: Though rare, APMPPE may present with neurological involvement, as in this previously unreported association with CN III palsy. Unlike uncomplicated APMPPE cases, in patients with neurological manifestations systemic therapy is advocated.

Purpose: To describe the clinical presentation and imaging features of a patient presenting with bilateral central retinal vein occlusion (CRVO), who was subsequently diagnosed with hyperviscosity retinopathy due to B cell lymphoproliferative disease, and had a good response to systemic immunosuppressive therapy. Observations: A clinical case report of an 87-year-old woman who presented with bilateral CRVO. Visual acuity, clinical examination, spectral domain optical coherence tomography (SD-OCT), color fundus photography and systemic evaluation were obtained. Ocular examination at presentation revealed bilateral CRVO, and OCT examination revealed significant central macular edema bilaterally. Six months after the diagnosis of hyperviscosity retinopathy and administration of systemic cyclophosphamide immunosuppressive therapy for B cell lymphoproliferative disease, most of the retinal hemorrhages resolved and partial resolution of the macular edema in the left eye was observed. Conclusion and Importance: This case describes the association between bilateral simultaneous CRVO and hyperviscosity. Under unique circumstances our patient received systemic therapy alone, without plasmapheresis. Although only limited therapy was applied, she didn't have deterioration or recurrent events; she had a small improvement in her macular edema and a significant improvement in her systemic functional state as well as reduction in her monoclonal IGM level. Keywords: Bilateral, CRVO, Hyperviscosity, Retinopathy, Waldenstrom's macroglobulinemia