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1.
Orphanet J Rare Dis ; 17(1): 381, 2022 10 21.
Artigo em Inglês | MEDLINE | ID: mdl-36271426

RESUMO

BACKGROUND: Our understanding of work productivity impairment among patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is limited. The purpose of this study was to provide an overview of work productivity loss in SAPHO syndrome patients through the use of the work productivity and activity impairment (WPAI) questionnaire, as well as to investigate the relationship between the WPAI and other disease-related indicators. METHODS: Patients for this cross-sectional study were recruited from Peking Union Medical College Hospital (Beijing, China). The questionnaires incorporating the WPAI were administered, along with the inclusion of demographic data, disease-specific measures, and general health variables. The construct validity of the WPAI was evaluated via the correlations between WPAI outcomes and other measures. Wilcoxon rank-sum tests and nonparametric Kruskal‒Wallis tests were used for the comparison of the WPAI outcomes between known groups. RESULTS: A total of 376 patients were included, and 201 patients (53.5%) were employed. The medians (interquartile range [IQR]) of absenteeism, presenteeism, work productivity loss, and activity impairment were 0% (0-13%), 20% (0-40%), 20% (0-52%), and 30% (0-50%), respectively. All of the WPAI outcomes showed moderate to strong correlations with other generic and disease-specific measures (|r| = 0.43-0.75), except for absenteeism. Increasing disease activity and worse health status were significantly associated with increased impairments of work productivity and activity. CONCLUSION: This study highlights the negative effects of SAPHO syndrome on the work productivity and activity of patients, thus indicating good construct validity and discriminative ability of the WPAI. To reduce the economic burden, it is important to improve the work productivity and daily activity of patients by ameliorating clinical care.


Assuntos
Síndrome de Hiperostose Adquirida , Humanos , Estudos Transversais , Eficiência , Presenteísmo , Absenteísmo , Inquéritos e Questionários
2.
Int J Rheum Dis ; 25(11): 1328-1332, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35938529

RESUMO

Pachydermoperiostosis (PDP) is a rare disorder characterized by skin thickening, acropachia, and periostosis. Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is also an orphan disease featured by different dermatological and osteoarthritic manifestations. Herein, we report the first case of an adolescent male diagnosed with both PDP and SAPHO syndrome, presenting with digital clubbing, polyarthralgia, ostealgia, pachydermia and acne on his face, chest and back. Furthermore, we distinguish the characteristics of both diseases and explore the potential pathological mechanism for this coexistence in one patient. Further investigations are needed to establish the detailed pathophysiological association of these 2 diseases.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Hiperostose , Osteíte , Osteoartropatia Hipertrófica Primária , Sinovite , Adolescente , Masculino , Humanos , Hiperostose/complicações , Hiperostose/patologia , Síndrome de Hiperostose Adquirida/complicações , Sinovite/diagnóstico , Acne Vulgar/complicações , Acne Vulgar/diagnóstico , Acne Vulgar/patologia , Doenças Raras/complicações
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