Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical center

Introduction: Still's disease is a rare autoinflammatory disorder that affects mostly young adults, with an unknown cause and difficult to diagnose. This study describes four cases of adult-onset Still's disease in Guatemala, providing valuable information for future research. Methodolo...

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Main Author: Bran Ordóñez A, Guevara Mejía JE, Bautista Esquivel YD, García Kutzbach A
Format: Article
Language:English
Published: Panamerican League of Associations of Rheumatology (PANLAR) 2023-05-01
Series:Global Rheumatology
Subjects:
Online Access:https://globalrheumpanlar.org/manuscrito-articulo-original/descripcion-clinica-de-pacientes-con-enfermedad-de-still-del-adulto-1?language=en
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author Bran Ordóñez A, Guevara Mejía JE, Bautista Esquivel YD, García Kutzbach A
author_facet Bran Ordóñez A, Guevara Mejía JE, Bautista Esquivel YD, García Kutzbach A
author_sort Bran Ordóñez A, Guevara Mejía JE, Bautista Esquivel YD, García Kutzbach A
collection DOAJ
description Introduction: Still's disease is a rare autoinflammatory disorder that affects mostly young adults, with an unknown cause and difficult to diagnose. This study describes four cases of adult-onset Still's disease in Guatemala, providing valuable information for future research. Methodology: A descriptive study was conducted based on patient records from 2021 to 2022. A questionnaire was designed to gather patient information. Results: Four patients (3 males, 1 female) with a mean age of 42 were included in the study. The most common symptoms were fever and joint pain, with all patients exhibiting leukocytosis, neutrophilia, and elevated liver enzymes. Two patients had positive antinuclear antibodies. Three patients had ferritin levels between 500-1000 ng/ml, while one patient had levels over 1000 ng/dl. All patients received steroids and methotrexate, with one patient also receiving leflunomide due to inadequate response. Adherence to treatment varied, with two patients attending regular appointments and two not. Conclusions: This is the first description of adult-onset Still's disease in Central America, providing valuable information for future research. Ferritin levels are useful markers of inflammation and may aid in the optimization of steroid treatment. The majority of patients adhered well to treatment and follow-up, highlighting the importance of consistent medical care for this rare disease.
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spelling doaj.art-894203825a6d4c4ea9ab28d9445ff9cb2023-06-20T20:26:22ZengPanamerican League of Associations of Rheumatology (PANLAR)Global Rheumatology2709-55332023-05-0141https://doi.org/10.46856/grp.10.e173Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical centerBran Ordóñez A, Guevara Mejía JE, Bautista Esquivel YD, García Kutzbach A0Asociación guatemalteca anti-enfermedades reumáticas (AGAR)Introduction: Still's disease is a rare autoinflammatory disorder that affects mostly young adults, with an unknown cause and difficult to diagnose. This study describes four cases of adult-onset Still's disease in Guatemala, providing valuable information for future research. Methodology: A descriptive study was conducted based on patient records from 2021 to 2022. A questionnaire was designed to gather patient information. Results: Four patients (3 males, 1 female) with a mean age of 42 were included in the study. The most common symptoms were fever and joint pain, with all patients exhibiting leukocytosis, neutrophilia, and elevated liver enzymes. Two patients had positive antinuclear antibodies. Three patients had ferritin levels between 500-1000 ng/ml, while one patient had levels over 1000 ng/dl. All patients received steroids and methotrexate, with one patient also receiving leflunomide due to inadequate response. Adherence to treatment varied, with two patients attending regular appointments and two not. Conclusions: This is the first description of adult-onset Still's disease in Central America, providing valuable information for future research. Ferritin levels are useful markers of inflammation and may aid in the optimization of steroid treatment. The majority of patients adhered well to treatment and follow-up, highlighting the importance of consistent medical care for this rare disease.https://globalrheumpanlar.org/manuscrito-articulo-original/descripcion-clinica-de-pacientes-con-enfermedad-de-still-del-adulto-1?language=enadult still's diseaseferritinsteroidsmethotrexatebiologics
spellingShingle Bran Ordóñez A, Guevara Mejía JE, Bautista Esquivel YD, García Kutzbach A
Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical center
Global Rheumatology
adult still's disease
ferritin
steroids
methotrexate
biologics
title Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical center
title_full Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical center
title_fullStr Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical center
title_full_unstemmed Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical center
title_short Clinical description of patients with adult still’s disease, report of 4 cases in a Guatemalan medical center
title_sort clinical description of patients with adult still s disease report of 4 cases in a guatemalan medical center
topic adult still's disease
ferritin
steroids
methotrexate
biologics
url https://globalrheumpanlar.org/manuscrito-articulo-original/descripcion-clinica-de-pacientes-con-enfermedad-de-still-del-adulto-1?language=en
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