Jucier Gonçalves Júnior1, Naoki Mugii2, Pleiades Tiharu Inaoka3, Renata Miossi1, Fernando Henrique Carlos De Souza1, João Calvino Soares De Oliveira1, Fujiko Someya3, Yasuhito Hamaguchi4, Takashi Matsushita4 , Percival Degrava Sampaio-Barros1, Samuel Katsuyuki Shinjo1

1Division of Rheumatology, Universidade De São Paulo, São Paulo, Brazil
2Department of Rehabilitation, Kanazawa University Hospital, Ishikawa, Japan
3Kanazawa University, Division of Rehabilitation Science, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Ishikawa, Japan
4Department of Dermatology, Kanazawa University, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Ishikawa, Japan

Keywords: Dermatomyositis, inflammatory myopathies, myositis, polymyositis, systemic sclerosis.

Abstract

Objectives: This study aims to describe and compare the demographic, clinical, and laboratory characteristics and follow-up of representative samples of patients with myopathies and systemic sclerosis overlap syndromes (Myo-SSc) from two tertiary centers.

Patients and methods: This is a cross-sectional and retrospective study conducted between January 2000 and December 2020. Fourty-five patients were analyzed with Myo-SSc (6 males, 39 females; mean age: 50.2±15.4 years; range, 45 to 65 years) from two tertiary centers (n=30 from Brazil and n=15 from Japan).

Results: The median follow-up was 98 (range, 37 to 168) months. Muscle impairment started simultaneously with the diagnosis of systemic sclerosis in 57.8% (26/45) of cases. Muscle involvement occurred before the onset of systemic sclerosis in 35.5% (16/45) of cases, and after in 6.7% (3/45). Polymyositis was observed in 55.6% (25/45) of cases, followed by dermatomyositis in 24.4% (11/45) and antisynthetase syndrome in 20.0% (9/45). Concerning systemic sclerosis, the diffuse and limited forms occurred in 64.4% (29/45) and 35.6% (16/45) of the cases, respectively. Comparing the subgroups, Myo or SSc onset was earlier in Brazilian patients, and they had a higher frequency of dysphagia (20/45, [66.7%]) and digital ulcers (27/45, [90%]), whereas Japanese patients had higher modified Rodnan skin scores (15 [9 to 23]) and prevalence of positive anti-centromere antibodies (4/15 [23.7%]). The current disease status and mortality were similar in both groups.

Conclusion: In the present study, Myo-SSc affected middle-aged women, and its manifestation spectrum varied according to geographic distribution.

Citation: Gonçalves Júnior J, Mugii N, Inaoka PT, Miossi R, De Souza FHC, De Oliveira JCS, et al. Patients with inflammatory myopathies overlapping with systemic sclerosis: A Brazilian-Japanese bicentric study. Arch Rheumatol 2023;38(x):i-x.