Hua Cao1, Qunli Xia1, Meng Pan1, Xiaoqing Zhao1, Xia Li1, Ruofei Shi1, Min Zhou1, Xiaoyi Ding1, Masataka Kuwana1, Jie Zheng2. 1. From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University. 2. From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University. jie-zheng2001@126.com.
Abstract
OBJECTIVE: Gottron papules and Gottron sign are characteristic and possibly pathognomonic cutaneous features of classic dermatomyositis and clinically amyopathic dermatomyositis (DM/CADM). However, the Gottron papules/Gottron sign with cutaneous ulceration (ulcerative Gottron papules/Gottron sign) are less common. We aimed to clarify the clinical characteristics of patients with DM/CADM who have ulcerative Gottron papules/Gottron sign. METHODS: Clinical features, laboratory findings, and prognosis of patients with DM/CADM who had Gottron papules/Gottron sign with or without ulceration were analyzed and compared. RESULTS: Occurrences of acute interstitial pneumonia/subacute interstitial pneumonia (AIP/SIP) were significantly higher in patients with ulcerative Gottron papules/Gottron sign (19/26) versus patients with Gottron papules/Gottron sign without ulceration (2/66, p < 0.001). We also observed that the white blood cell counts (mean ± SD 4.2 ± 1.6 vs 6.9 ± 2.9; p < 0.001) and creatine kinase (CK) levels (198.0 ± 377.7 vs 1364.0 ± 2477.0; p = 0.019) were significantly lower, whereas the positive rate of antimelanoma differentiation-associated gene 5 antibody (anti-MDA5; 88.5% vs 6.1%, p < 0.001) and serum ferritin levels (665.2 ± 433.5 vs 256.2 ± 279.0, p < 0.001) were significantly higher in the patients with ulcerative Gottron papules/Gottron sign. Moreover, the cumulative survival rate of the group with ulcerative Gottron papules/Gottron sign was significantly lower (p < 0.001). CONCLUSION: Patients with DM/CADM who have ulcerative Gottron papules/Gottron sign, positive anti-MDA5 antibody, and significantly lower baseline CK level are at increased risk of interstitial lung disease, especially AIP/SIP. A new designation for this subgroup of patients should be established to draw more attention to this clinical entity.
OBJECTIVE: Gottron papules and Gottron sign are characteristic and possibly pathognomonic cutaneous features of classic dermatomyositis and clinically amyopathic dermatomyositis (DM/CADM). However, the Gottron papules/Gottron sign with cutaneous ulceration (ulcerative Gottron papules/Gottron sign) are less common. We aimed to clarify the clinical characteristics of patients with DM/CADM who have ulcerative Gottron papules/Gottron sign. METHODS: Clinical features, laboratory findings, and prognosis of patients with DM/CADM who had Gottron papules/Gottron sign with or without ulceration were analyzed and compared. RESULTS: Occurrences of acute interstitial pneumonia/subacute interstitial pneumonia (AIP/SIP) were significantly higher in patients with ulcerative Gottron papules/Gottron sign (19/26) versus patients with Gottron papules/Gottron sign without ulceration (2/66, p < 0.001). We also observed that the white blood cell counts (mean ± SD 4.2 ± 1.6 vs 6.9 ± 2.9; p < 0.001) and creatine kinase (CK) levels (198.0 ± 377.7 vs 1364.0 ± 2477.0; p = 0.019) were significantly lower, whereas the positive rate of antimelanoma differentiation-associated gene 5 antibody (anti-MDA5; 88.5% vs 6.1%, p < 0.001) and serum ferritin levels (665.2 ± 433.5 vs 256.2 ± 279.0, p < 0.001) were significantly higher in the patients with ulcerative Gottron papules/Gottron sign. Moreover, the cumulative survival rate of the group with ulcerative Gottron papules/Gottron sign was significantly lower (p < 0.001). CONCLUSION:Patients with DM/CADM who have ulcerative Gottron papules/Gottron sign, positive anti-MDA5 antibody, and significantly lower baseline CK level are at increased risk of interstitial lung disease, especially AIP/SIP. A new designation for this subgroup of patients should be established to draw more attention to this clinical entity.
Authors: Peng Huang; Li Tang; Lu Zhang; Yi Ren; Hong Peng; Yangyang Xiao; Jie Xu; Dingan Mao; Lingjuan Liu; Liqun Liu Journal: Front Immunol Date: 2022-05-30 Impact factor: 8.786
Authors: Anca Bobirca; Cristina Alexandru; Anca Emanuela Musetescu; Florin Bobirca; Anca Teodora Florescu; Magdalena Constantin; Tiberiu Tebeica; Alesandra Florescu; Sebastian Isac; Mihai Bojinca; Ioan Ancuta Journal: Life (Basel) Date: 2022-07-23
Authors: Laurence Pacot; Jacques Pouchot; Nicolas De Prost; Marie Senant; Eric Tartour; Françoise Le Pimpec-Barthes; Dominique Israel-Biet; Marie-Agnes Dragon-Durey Journal: Front Med (Lausanne) Date: 2020-03-10