Publications by authors named "Perla Ayumi Kawakami-Campos"

Background: VEXAS syndrome, a recently identified systemic autoinflammatory disorder, poses new diagnostic and management challenges. Based on experience with other autoinflammatory diseases, anti-interleukin (IL)-1, anti-IL-6, anti-tumor necrosis factor (TNF) biotechnological agents, and Janus kinase inhibitors (JAKis) have been widely employed in VEXAS patients. The aim of this study is to evaluate the global effectiveness and safety of biotechnological agents and JAKis using data from the real-world context.

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During the last decade, spondyloarthritis (SpA) has increasingly been considered a disease at the crossroads between autoimmunity and autoinflammation. Some patients may even present with autoinflammatory-related manifestations, including fever, hidradenitis suppurativa, other neutrophilic dermatoses, and an unusually high increase in inflammatory markers. Therefore, a subgroup of SpA patients may be identified, and specific details about this cluster need to be investigated.

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  • The study aimed to explore gender differences in the phenotypical expression of Behçet's disease (BD) using data from the International AIDA Network Registry, focusing on damage index, disease manifestations, and cardiovascular risk.
  • A total of 1024 patients (567 males and 457 females) were examined, revealing that males had a significantly higher overall damage index and more frequent occurrences of uveitis and vascular involvement, while females showed higher instances of arthralgia, arthritis, and CNS involvement.
  • Key factors associated with major organ involvement included male gender, treatment with biologic agents, origin from endemic regions, and longer disease duration, indicating a more severe course of BD in males compared to females.
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  • Dry eye disease (DED) may be associated with axial spondylarthritis (axial-SpA), as this study aims to investigate the correlation between the two conditions and identify related variables.
  • Out of 71 axial-SpA patients, 64.8% reported DED symptoms compared to only 15.8% of 19 healthy controls, suggesting a significantly higher prevalence of DED in axial-SpA patients.
  • Various ocular tests revealed that axial-SpA patients exhibited poorer tear production and ocular surface health, indicated by lower Schirmer test scores, shorter tear break-up times, and higher staining levels, reinforcing the link between axial-SpA and DED.
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Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.

Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.

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Article Synopsis
  • - VEXAS syndrome is a new autoinflammatory disease that often affects various organs, with noticeable inflammatory issues in the eyes and orbits.
  • - In a study of 59 VEXAS patients, 45.8% exhibited orbital/ocular problems, with periorbital edema and episcleritis being the most common conditions observed.
  • - There is a significant link between relapsing polychondritis and eye involvement in VEXAS, and patients with eye issues showed higher mortality rates, indicating the need for closer monitoring by healthcare providers.
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  • This study evaluated the effectiveness of a systemic score in predicting severe outcomes in patients with Still disease, including the risk of mortality and severe complications like macrophage activation syndrome.
  • Involving 597 patients, the study found that a higher systemic score significantly correlated with life-threatening outcomes, with scores of 7 or above indicating a greater risk.
  • Key components that were most predictive of severe outcomes included liver and lung involvement, emphasizing the importance of these factors in managing Still disease.
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Introduction: The effectiveness of canakinumab may change according to the different times it is used after Still's disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment.

Methods: Patients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still's disease.

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Background: To report a case of unilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in a Hispanic convalescent COVID-19 female patient. Case presentation A 35-year-old Hispanic female with exposure to the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was evaluated due to unilateral visual loss. Ophthalmic examination and diagnostic tests were consistent with APMPPE.

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