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Glucocorticoides na esclerose sistémica
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Authors
Abstract(s)
Introdução: A esclerose sistémica (ES) é uma doença heterogénea relativamente às suas manifestações clínicas e imunológicas, evolução e prognóstico. Apresenta elevada morbimortalidade, sobretudo quando estão presentes complicações de órgão major, pelo que a abordagem a estes doentes é um grande desafio médico. Ao contrário de outras doenças imunomediadas em que os glucocorticoides (GC) são amplamente usados com evidência clínica robusta, o seu papel na ES é controverso. Embora os GC possam ser úteis no controlo de algumas manifestações inflamatórias, o seu uso tem sido associado a um risco aumentado de Crise Renal Esclerodérmica (CRE). Como resultado, existe uma grande variabilidade na prescrição de corticosteroides entre os reumatologistas.
Objetivos: Caracterizar o padrão de utilização de GC nos doentes com ES, os motivos da sua prescrição, os seus benefícios e riscos, nomeadamente de desenvolvimento de CRE.
Métodos: Estudo retrospetivo de doentes com diagnóstico de ES de acordo com os critérios ACR/EULAR 2013, seguidos na consulta de reumatologia do Hospital Garcia da Orta e registados na plataforma Reuma.pt. Foi realizada uma análise descritiva para caracterização dos tratamentos realizados com GC. Os doentes com o diagnóstico de ES foram divididos em dois grupos, um de doentes que realizou pelo menos um tratamento com GC e outro de doentes nunca medicados com GC. A comparação entre os dois grupos foi realizada através do teste qui-quadrado, T-student ou de Mann-Whitney, conforme apropriado. A significância estatística foi determinada para valor-p inferior a 0.05.
Resultados: Foram incluídos 157 doentes com ES, dos quais 47.1% realizaram tratamento com GC sistémicos. Os doentes medicados com GC apresentavam mais frequentemente fenótipo de ES de síndrome de sobreposição, úlceras digitais, miosite e diabetes mellitus. Os doentes sob GC realizaram mais frequentemente outros tratamentos imunossupressores.
A maioria dos doentes realizou apenas um ciclo de tratamento com GC, contudo 36.6% dos doentes realizaram dois ou mais, totalizando 111 tratamentos. A formulação mais frequentemente utilizada foi a prednisolona oral e a indicação mais frequente para o tratamento foi o envolvimento musculoesquelético. Neste envolvimento bem como no cutâneo utilizaram-se preferencialmente doses baixas (≤7.5mg/dia) e intermédias (>7.5mg/dia e ≤30mg/dia) de GC, ao contrário do envolvimento pulmonar em que se utilizaram principalmente doses altas (>30mg/dia). A serosite foi a manifestação com melhor resposta clínica aos GC. Apenas se verificou um caso de CRE num doente não medicado com GC.
Conclusão: Apesar das preocupações com a segurança, os GC são frequentemente utilizados para tratar a ES, particularmente as manifestações musculoesqueléticas, cutâneas e pulmonares. Os nossos dados sugerem um benefício terapêutico dos GC no tratamento da ES e a sua utilização não se associou ao desenvolvimento de CRE nesta coorte.
Background: Systemic sclerosis (SSc) is a heterogeneous disease regarding clinical and immunological manifestations, progression, and prognosis. It has significant morbimortality, especially when major organ involvement is present, making its management a significant challenge. Unlike other immune-mediated diseases in which glucocorticoids (GC) are widely used with robust clinical evidence, their role in SSc is controversial. Although GCs may be useful to manage some inflammatory manifestations of SSc, their use has been associated with an increased risk of Scleroderma Renal Crisis (SRC). As a result, the use of GC in SSc patients in clinical practice is highly variable among rheumatologists. Objectives: To characterize the pattern of GC use in patients with SSc, as well as the reasons for GC prescription, its benefits and risks, particularly concerning SRC development. Methods: Retrospective study of patients with SSc diagnosis according to ACR/EULAR 2013 criteria, followed at Hospital Garcia de Orta rheumatology department and registered in Reuma.pt database. A descriptive analysis was conducted to characterize the treatments performed with GC. Patients with SSc were divided into two groups: those who received at least one GC treatment and those who never received GC treatment. The comparison between the two groups was performed using the chi-square test, Student’s T-test, or Mann-Whitney test, as appropriate. The threshold for statistical significance was a p-value inferior to 0.05. Results: 157 patients with SSc were included, of whom 47.1% were treated with GC. Patients exposed to GC more often had a SSc-overlap syndrome phenotype, digital ulcers, myositis, and diabetes mellitus. Those receiving GC were also more likely treated with other immunosuppressants. Most patients received only one course of systemic GC, however 33.6% were treated twice or more, with a total of 111 treatments performed. The most frequently used GC was oral prednisolone, and musculoskeletal involvement was the most frequent reason. Musculoskeletal and skin involvement were more frequently treated with low (≤7.5mg/day) and intermediate (>7.5mg/day and ≤30mg/day) doses while lung involvement was more frequently treated with high doses (>30mg/day). Serositis was the SSc manifestation with the best clinical response to GC. Only one case of SRC was reported in a patient that was never treated with GC. Conclusion: Despite safety concerns, GC are frequently used in clinical practice to treat SSc, particularly musculoskeletal, skin and lung manifestations. Our data suggest a therapeutic benefit of GC in the treatment of SSc and its use was not associated with the development of SRC in our cohort.
Background: Systemic sclerosis (SSc) is a heterogeneous disease regarding clinical and immunological manifestations, progression, and prognosis. It has significant morbimortality, especially when major organ involvement is present, making its management a significant challenge. Unlike other immune-mediated diseases in which glucocorticoids (GC) are widely used with robust clinical evidence, their role in SSc is controversial. Although GCs may be useful to manage some inflammatory manifestations of SSc, their use has been associated with an increased risk of Scleroderma Renal Crisis (SRC). As a result, the use of GC in SSc patients in clinical practice is highly variable among rheumatologists. Objectives: To characterize the pattern of GC use in patients with SSc, as well as the reasons for GC prescription, its benefits and risks, particularly concerning SRC development. Methods: Retrospective study of patients with SSc diagnosis according to ACR/EULAR 2013 criteria, followed at Hospital Garcia de Orta rheumatology department and registered in Reuma.pt database. A descriptive analysis was conducted to characterize the treatments performed with GC. Patients with SSc were divided into two groups: those who received at least one GC treatment and those who never received GC treatment. The comparison between the two groups was performed using the chi-square test, Student’s T-test, or Mann-Whitney test, as appropriate. The threshold for statistical significance was a p-value inferior to 0.05. Results: 157 patients with SSc were included, of whom 47.1% were treated with GC. Patients exposed to GC more often had a SSc-overlap syndrome phenotype, digital ulcers, myositis, and diabetes mellitus. Those receiving GC were also more likely treated with other immunosuppressants. Most patients received only one course of systemic GC, however 33.6% were treated twice or more, with a total of 111 treatments performed. The most frequently used GC was oral prednisolone, and musculoskeletal involvement was the most frequent reason. Musculoskeletal and skin involvement were more frequently treated with low (≤7.5mg/day) and intermediate (>7.5mg/day and ≤30mg/day) doses while lung involvement was more frequently treated with high doses (>30mg/day). Serositis was the SSc manifestation with the best clinical response to GC. Only one case of SRC was reported in a patient that was never treated with GC. Conclusion: Despite safety concerns, GC are frequently used in clinical practice to treat SSc, particularly musculoskeletal, skin and lung manifestations. Our data suggest a therapeutic benefit of GC in the treatment of SSc and its use was not associated with the development of SRC in our cohort.
Description
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2024
Keywords
Esclerose sistémica Glucocorticoides Crise renal esclerodérmica Reumatologia