scholarly journals CLINICAL FEATURES OF PATIENTS WITH CLINICALLY AMYOPATHIC DERMATOMYOSITIS WHO HAVE CIRCULATING ANTI-MDA5 AUTOANTIBODIES WITH INTERSTITIAL PNEUMONIA

Respirology ◽  
2018 ◽  
Vol 23 ◽  
pp. 154-155
Keyword(s):  
Interstitial Pneumonia ◽  
Clinical Features ◽  
Amyopathic Dermatomyositis ◽  
Clinically Amyopathic Dermatomyositis

Gottron Papules and Gottron Sign with Ulceration: A Distinctive Cutaneous Feature in a Subset of Patients with Classic Dermatomyositis and Clinically Amyopathic Dermatomyositis

2016 ◽  
Vol 43 (9) ◽  
pp. 1735-1742 ◽  
Author(s):  
Hua Cao ◽  
Qunli Xia ◽  
Meng Pan ◽  
Xiaoqing Zhao ◽  
Xia Li ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Amyopathic Dermatomyositis ◽  
Laboratory Findings ◽  
Clinically Amyopathic Dermatomyositis ◽  
Acute Interstitial Pneumonia ◽  
Cell Counts ◽  
Lower Baseline ◽  
Increased Risk ◽  
Positive Rate ◽  
White Blood Cell Counts

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.

scholarly journals Clinically Amyopathic Dermatomyositis with Interstitial Pneumonia That Was Successfully Treated with Plasma Exchange

2018 ◽  
Vol 57 (13) ◽  
pp. 1935-1938 ◽  
Author(s):  
Mizuki Yagishita ◽  
Yuya Kondo ◽  
Toshihiko Terasaki ◽  
Mayu Terasaki ◽  
Masaru Shimizu ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Interstitial Pneumonia ◽  
Amyopathic Dermatomyositis ◽  
Clinically Amyopathic Dermatomyositis
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