Clinical Description of Patients with Systemic Lupus Erythematosus Evaluated in a Specialized Center for Rheumatic Diseases in Guatemala City

16 February, 2021
20 May, 2021
21 June, 2021

Autor(s)
  • Alex Noé López Martinez
    MD Centro médico AGAR
    AGAR( Guatemalan Association for Rheumatic Diseases)
    alexlopez@ufm.edu
    ORCID:
    0000-0003-1520-237X
  • Marvin Josué Bustamante Gutiérrez
    MD Centro médico AGAR
    AGAR(Guatemalan Association for Rheumatic Diseases)
    marvinbustamanteg@gmail.com
    ORCID:
    0000-0002-7126-480X
  • Idania Calixta Escalante Mendoza
    MD Centro médico AGAR
    AGAR(Guatemalan Association for Rheumatic Diseases)
    idaniaescalante@ufm.edu
    ORCID:
    0000-0003-0156-8098
  • Yeny Mariela Maldonado Contreras
    MD Centro médico AGAR
    AGAR(Guatemalan Association for Rheumatic Diseases)
    yenymarielamaldonado@gmail.com
    ORCID:
    0000-0002-5068-0134
  • Luis Adolfo Kramer Catalan
    MD Centro médico AGAR
    AGAR(Guatemalan Association for Rheumatic Diseases)
    luiskramer@ufm.edu
    ORCID:
    0000-0001-5873-8946
  • Juan Pablo Araica Gallo
    MD Centro médico AGAR
    AGAR ( Guatemalan Association for Rheumatic Diseases)
    pabloaraica@ufm.edu
    ORCID:
    0000-0003-4055-1264
  • Ariel Obregón-Ponce MD
    MD Centro médico AGAR
    AGAR(Guatemalan Association for Rheumatic Diseases - Guatemala)
    pabloaraica@ufm.edu
    ORCID:
    orcid.org/0000-0002-5498-7088
  • Abraham Garcia Kutzbach MD
    Rheumatologist, Rheumatology postgraduate director of the Guatemalan Association for Rheumatic Diseases - Guatemala, MACR, MRPANLAR,
    AGAR(Guatemalan Association for Rheumatic Diseases - Guatemala)
    abraham@garciakutzbach.org
    ORCID:
    000-0001-9214-2118
  • Views 1363Views

    License

    This is an open-access article distributed by the terms of the Creative Common Attribution License (CC-BY NC-4). The use, distribution or reproduction in other forms is permitted, provided the original author(a) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with this terms.

    6

    Clinical Description of Patients with Systemic Lupus Erythematosus Evaluated in a Specialized Center for Rheumatic Diseases in Guatemala City

    Summary

    Resumen /Abstract

    Resumen en Español

    El lupus eritematoso sistémico (LES) es una enfermedad autoinmune inflamatoria crónica, caracterizada por la producción de autoanticuerpos que generan inflamación sistémica y afectación multiorgánica. En este estudio realizamos una descripción de los criterios clasificatorios, índice de actividad, índice de daño de órgano y tratamiento de los pacientes atendidos en la sede de la Asociación Guatemalteca Anti enfermedades Reumáticas (AGAR) ubicada en la Ciudad Guatemala.

    Métodos

    Se incluyeron pacientes con diagnóstico de LES, según los criterios clasificatorios ACR 1997, que fueron evaluados en AGAR durante el primer trimestre de 2020, se describe el estado de actividad de la enfermedad utilizando la puntuación SLEDAI-2K e índice de daño de órganos SLICC/ACR.

    Resultados

    Hay un predominio del sexo femenino 10:1. La mayoría de los pacientes presentaban baja actividad de la enfermedad (58.8 %) y la afectación del sistema musculoesquelético fue la más frecuente (88.2 %). De forma global, la tasa de daño orgánico fue del 45 %. Los glucocorticoides orales (prednisona/deflazacort) a dosis bajas se usaron en el 92.1 % de los casos. Los antimaláricos (hidroxicloroquina) en el 85.7 % de los casos, el inmunosupresor más utilizado fue azatioprina (68.7 %), seguido de micofenolato mofetilo (31.3 %).

    Conclusiones

    La mayoría de pacientes estaban con una baja actividad al momento del examen. La artritis es la manifestación clínica más frecuente, y en la escala de daño de órgano predomina un solo elemento de daño (artritis). La mayoría de los pacientes se mantienen con dosis bajas de glucocorticoides, antimaláricos (hidroxicloroquina) y azatioprina.

    Palabras clave

    Lupus, índice de daño orgánico, RELES.

     

    Resumen en Inglés

    Abstract:

    Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease, characterized by the production of autoantibodies generating systemic inflammation and multi-organ involvement. In this study we carry out a description of the classification criteria, activity index, organic damage index and treatment of patients attended at the clinic of the Guatemalan Association for Rheumatic Diseases (AGAR) -Ciudad Guatemala.

    Methods

    Patients with a diagnosis of Systemic Lupus Erythematosus were included, according to the ACR 1997 classification criteria, who were evaluated in the AGAR clinic during the first quarter of 2020, the disease activity status is described using the SLEDAI-2K score and index of organic damage measured by SLICC.

    Results

    There is a 10: 1 predominance of the female to male ratio, most of the patients had low disease activity (58.8%) and the involvement of the musculoskeletal system was the most frequent (88.2%). The organic damage rate was 45% globally. Oral glucocorticoids (prednisone / deflazacort) at low doses were used in 92.1% of the cases, antimalarials (hydroxychloroquine) in 85.7% and the most used immunosuppressant was azathioprine (68.7%) followed by mycophenolate mofetil (31.3%).

    Conclusions

    Most of the patients were in low activity at the time of the examination, arthritis is the most frequent clinical manifestation, and in the scale of organic damage a single element of damage predominates. Most patients were maintained on low doses of glucocorticoids, antimalarials (hydroxychloroquine), and azathioprine.

    Resumen en Portugués

    Resumo:

    O lúpus eritematoso sistêmico (LES) é uma doença inflamatória crônica autoimune, caracterizada pela produção de autoanticorpos gerando inflamação sistêmica e envolvimento de múltiplos órgãos. Neste estudo realizamos uma descrição dos critérios de classificação, índice de atividade, índice de dano orgânico e tratamento de pacientes atendidos no ambulatório da Associação de Doenças Reumáticas da Guatemala (AGAR) - Ciudad Guatemala.

    Métodos

    Foram incluídos pacientes com diagnóstico de Lúpus Eritematoso Sistêmico, de acordo com os critérios de classificação do ACR 1997, que foram avaliados no ambulatório do AGAR durante o primeiro trimestre de 2020, o estado de atividade da doença é descrito usando o escore SLEDAI-2K e índice de dano orgânico medido pelo SLICC.

    Resultados

    Há uma predominância de 10: 1 do sexo feminino em relação ao masculino, a maioria dos pacientes apresentava baixa atividade da doença (58,8%) e o acometimento do sistema musculoesquelético foi o mais frequente (88,2%). A taxa de dano orgânico foi de 45% globalmente. Glicocorticóides orais (prednisona / deflazacort) em baixas doses foram utilizados em 92,1% dos casos, antimaláricos (hidroxicloroquina) em 85,7% e o imunossupressor mais utilizado foi a azatioprina (68,7%) seguida do micofenolato mofetil (31,3%).

    Conclusões

    A maioria dos pacientes estava em baixa atividade no momento do exame, a artrite é a manifestação clínica mais frequente, e na escala de dano orgânico predomina um único elemento de dano. A maioria dos pacientes é mantida com baixas doses de glicocorticoides, antimaláricos (hidroxicloroquina) e azatioprina.

    Key messages

    Introduction

    SLE is a chronic inflammatory autoimmune disease characterized by the increase in autoantibodies which generates systemic inflammation and multi-organ involvement. Genetic, hormonal, environmental factors intervene in the etiopathogenesis, and it presents as a set of highly variable clinical manifestations, so its diagnosis is a challenge for the treating doctor. This disease has a worldwide dominance and occurs in all ethnic groups with a higher prevalence in African Americans. According to The Lupus Foundation of America, 1,5 million Americans and at least 5 million people worldwide have some form of lupus. (1,2)

    The Spanish Registry of Patients with Systemic Lupus Erythematosus (RELES) is an observational, prospective, multicenter, cohort study that began in 2010 and is designed to study the prevalence of non-inflammatory complications in patients with SLE; 44 centers in Spain and institutions such as care centers for SLE patients in other countries, including Guatemala.

    Objective

    Create a prospective observational registry of SLE patients in the Guatemalan population, describing the classification criteria, disease activity index, organ damage index with special attention to non-inflammatory complications and treatment.

    Material and methods

    A total number of fifty-one SLE outpatients were included and followed-up for evaluation during the first quarter of 2020. The information was collected by direct clinical interview and analysis of the respective clinical records performed by the same researchers using a standard questionnaire for this purpose. The ACR 1997 classification criteria (3,4) was initially evaluated and in follow-up appointments, the indicators of disease activity SLEDAI-2K, organ damage index SLICC/ACR (5,6) were applied and the treatments were described.

    We defined disease activity, using the score The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2k), in: mild ≤6 points, moderate 7 - 12 points and high activity 12. The Organic damage index by SLICC/ACR includes the evaluation of >12 organ systems through 39 items that record the damage that has occurred in SLE patients. The data was collected through the RELES questionnaire and sent to the coordinating center of the S&H Medical Sciences Service based in Madrid where it was analyzed and was later forwarded and analyzed in statistical programs (SPSS), expressed in the descriptive analysis of frequency in 2 x 2 tables, as  appropriate for the calculation of ″p″ with statistical significance (<0.05).

    The RELES group authorized that the data be used for the analysis and publication of our cohort. Data confidentiality was guaranteed and the ethical principles established by the Helsinki Declaration were respected.

     

    Results

    Of the 51 patients analyzed, 92.2% were female, with a 10:1 ratio.

    Classification criteria

    Applying the ACR 1997 classification criteria, the most frequent manifestation in SLE patients was arthritis 94.1%, ANA (+) 92.2%, anti-dsDNA 82.4%, anti-Sm 19.6% and proteinuria 37.3% (Table 1). When compared with the Spanish RELES study, we observed similar behavior in the frequency of these manifestations (9-15); 7.8% of patients were ANA (-) (1,7-9).

    Disease activity

    The precise measurement of SLE activity remains a challenge, due to the complexity of this disease, and the great variability of manifestations between patients has been recognized and even within the same patient over time (16). Applying the SLEDAI-2k score (7,17,18) we found patients with mild activity 58.8%, with moderate activity 25.5% and with high activity 15.6% at the time of the examination (Table 1). Most of the patients with high activity were new cases, late visits or absenteeism, probably conditioned by economic and cultural factors. Our study population is heterogeneous and we did not investigate genetic factors related to ethnic groups. We do not have local studies to compare our population and its disease activity index.

    Organ damage

    Using the SLICC/ACR index (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus), the musculoskeletal system was the most affected (82.2%) with arthritis (72.5%), followed by neuropsychiatric conditions (17.7%) with peripheral neuropathies (13.7%) and the renal system (13.7%). The patients that presented an organ damage index ≤2 (70.6%), with an element of damage (43.1%) and no element of damage (15.7%) (Table 2). Comparative cohorts in South American countries like the evidenced studies of Medina and Londoño (21) informed a damage index of 51.1% with at least 1 element, the predominant clinical manifestation was musculoskeletal with arthritis, followed by cardiovascular affectations (myocardiopathy and history of heart attack); similar to our data (19-20,24).

    Treatment

    It is aimed at remission or low activity of the disease, prevention of accumulated damage, minimization of adverse effects of drugs and improvement of the quality of life of patients (10,20,23-24). In our cohort, the most widely used drugs were oral glucocorticoids (prednisone/deflazacort) (92.1%) and antimalarials (85.7%), followed by immunosuppressants: azathioprine (68.7%), mycophenolate mofetil (31.3%), methotrexate (23%) and oral cyclophosphamide (21.5%). The decreased use of hydroxychloroquine and mycophenolate mofetil is due to its cost. Of the patients treated with oral glucocorticoids, 64.6% received low doses (< 7.5 mg of prednisone or its equivalent), 21.8% moderate doses (7.5 to 30 mg) and 4% high doses (> 30 mg) (23,25).

     

    Conclusions

    Most of our patients with SLE are in low activity of the disease and are mostly maintaining low doses of corticosteroids, antimalarial drugs and one immunosuppressant. Arthritis is the most common clinical manifestation and ≤ 2 elements predominate on the organ damage scale. At AGAR, as a Guatemalan institution dedicated to the study and treatment of patients with rheumatic diseases, we are committed to achieving timely management, with the lowest rate of disease activity and the least organ damage in the medium and long term. Most of our population has low economic resources, little education and multiple comorbidities, which favors a high activity of the disease in some patients. However, most cases are diagnosed and treated early, which is reflected in a large proportion of patients with low activity and mild to moderate organ damage.

    Interest conflict

    The authors declare no conflicts of interest

    Financing

    Ninguna

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