Therapeutic plasma exchange for clinically amyopathic dermatomyositis ( CADM ) associated with rapidly progressive interstitial pneumonia

2020 ◽  
Vol 35 (5) ◽  
pp. 435-443
Author(s):  
Akira Yamagata ◽  
Machiko Arita ◽  
Ayaka Tanaka ◽  
Fumiaki Tokioka ◽  
Tomohiro Yoshida ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Interstitial Pneumonia ◽  
Therapeutic Plasma Exchange ◽  
Amyopathic Dermatomyositis ◽  
Clinically Amyopathic Dermatomyositis

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

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 A Case of Clinically Amyopathic Dermatomyositis that was Refractory to Intensive Immunosuppressive Therapy including Tofacitinib, but Successfully Treated with Plasma Exchange Therapy

2022 ◽  
Author(s):  
Daisuke Hiraoka ◽  
Jun Ishizaki ◽  
Kenta Horie ◽  
Takuya Matsumoto ◽  
Koichiro Suemori ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Immunosuppressive Therapy ◽  
Amyopathic Dermatomyositis ◽  
Poor Survival ◽  
Clinically Amyopathic Dermatomyositis ◽  
Poor Treatment ◽  
High Level ◽  
Additional Therapy ◽  
Intensive Immunosuppressive Therapy ◽  
Poor Treatment Response

ABSTRACT Clinically amyopathic dermatomyositis (CADM) patients often develop rapidly progressive interstitial lung disease (RP-ILD). A high level of anti-melanoma differentiation-associated gene 5 antibodies (anti-MDA5 Ab) before treatment is associated with RP-ILD development, a poor treatment response, and poor survival. The prognosis of CADM patients remains poor due to ILD even with combined intensive immunosuppressive therapy. Recently, several additional therapies, including tofacitinib (TOF) and plasma exchange (PE) therapy, have been reported to be effective. We herein report a case of CADM-ILD with a high level of anti-MDA5 Ab that was refractory to combined intensive immunosuppressive therapy including TOF, but successfully treated with PE. The following are possible reasons why TOF was ineffective: 1) cytokines that were not suppressed by TOF played an important role in RP-ILD; 2) TOF was administered later than previously reported; and 3) TOF did not suppress pathological substances such as antibodies. On the other hand, PE removes cytokines and various pathological substances. Therefore, PE may be a more reasonable additional therapy for intractable CADM-ILD.

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