scholarly journals A Case of Polymyxin b-Immobilized Fiber Column Treatment for Rapidly Progressive Interstitial Pneumonia Associated with Clinically Amyopathic Dermatomyositis

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Oh Sasaki ◽  
Makoto Dohi ◽  
Hiroaki Harada ◽  
Mitsuru Imamura ◽  
Yumi Tsuchida ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Ferritin Level ◽  
Polymyxin B ◽  
Amyopathic Dermatomyositis ◽  
Clinically Amyopathic Dermatomyositis ◽  
Oxygen Fraction ◽  
Fraction Of Inspired Oxygen ◽  
Inspired Oxygen ◽  
Tomography Scan ◽  
Column Treatment

We report a case of rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis who responded to single course of polymyxin b-immobilized fiber column treatment. Initial treatment with pulsed corticosteroids and cyclophosphamide, intravenous immunoglobulin, and cyclosporine seemed to suppress the activity of interstitial lung disease temporarily, but signs of relapse were detected such as elevation of serum KL-6 level and progressing pulmonary shadows in chest computed tomography scan. After polymyxin b-immobilized fiber column treatment, the areas of pulmonary shadows drastically decreased. Gradually, arterial partial pressure of oxygen/fraction of inspired oxygen (PaO2/FiO2) ratio recovered, and serum ferritin level and KL-6 level decreased. These findings indicate that polymyxin b-immobilized fiber column treatment could be promising in combination with conventional therapy for rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis, especially at the early phase of relapse.

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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