clinically amyopathic dermatomyositis
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Author(s):  
Daisuke Hiraoka ◽  
Jun Ishizaki ◽  
Kenta Horie ◽  
Takuya Matsumoto ◽  
Koichiro Suemori ◽  
...  

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.


Author(s):  
Saori Abe ◽  
Hiroto Tsuboi ◽  
Hirofumi Toko ◽  
Fumika Honda ◽  
Mizuki Yagishita ◽  
...  

2021 ◽  
Vol 8 ◽  
Author(s):  
Qiao Gu ◽  
MengYuan Diao ◽  
Wei Hu ◽  
Man Huang ◽  
Ying Zhu

Background: Clinically amyopathic dermatomyositis (CADM) presented with rapid progressive interstitial lung disease (RP-ILD) is rare. Here, we present a case of a post-partum female with CADM complicated by severe RP-ILD managed with venovenous extracorporeal membrane oxygenation (V-V ECMO).Case Summary: A 36-year-old woman was referred to a local hospital with cough and fever. She had a history of facial erythema and cough since an induction of labor for a stillborn fetus 2 months ago. Her status developed into RP-ILD with mediastinal emphysema and subcutaneous emphysema after admission, and V-V ECMO was initiated. After several failed attempts to wean the patient from ECMO, a decision was made to place the patient on the lung transplant waitlist. She underwent a double lung transplant on ECMO day 31 and received tacrolimus as an immunosuppressive regimen. The patient presented with positive anti-MDA5 and anti-Ro-52 antibodies and a high ferritin level, all of which indicated the presence of clinically amyopathic dermatomyositis (CADM). The patient was weaned from ECMO at 3 days after transplantation, but the patient's state of consciousness deteriorated, and head CT was considered for posterior reversible encephalopathy syndrome (PRES). After the temporary cessation of calcineurin inhibitors and a dosage reduction, the patient's state of consciousness returned to normal. Because of another disturbance of consciousness, the patient declined further treatment and was discharged 14 days after transplantation.Conclusion: Early recognition of CADM can effectively improve patients' prognosis. ECMO should be considered as a supportive therapy in patients in acute respiratory failure secondary to RP-ILD.


2021 ◽  
Vol 2021 (8) ◽  
Author(s):  
Masakazu Kitamura ◽  
Hiroshi Sugimoto

ABSTRACT An 84-year-old Japanese woman presented to our hospital with a month-long dry cough during the coronavirus disease 2019 (COVID-19) pandemic. She also had skin lesions on her fingers from 3 months prior. A chest computed tomography (CT) scan showed bilateral ground- glass opacities with a subpleural distribution, similar to the findings of COVID-19. The results of COVID-19 tests were negative. The titer of the anti-melanoma differentiation-associated gene 5 (MDA5) antibody was elevated. Consequently, we confirmed the diagnosis of clinically amyopathic dermatomyositis (CADM) and then administered oral prednisolone combined with tacrolimus. After the treatment, her symptoms, skin lesions and CT findings were gradually resolved.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 700.1-700
Author(s):  
E. Trallero-Araguás ◽  
F. Romero ◽  
I. Castellví ◽  
V. Ortiz-Santamaria ◽  
S. Castañeda ◽  
...  

Background:Idiopathic inflammatory myopathies are a heterogenous group of systemic autoimmune diseases. Several phenotypes have been linked to specific autoantibodies. Clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease, the most severe form of ILD, is associated with the anti-MDA5 antibodies. However not all the patients with dermatomyositis and anti-MDA5 positive antibodies develop this severe condition.Objectives:We aim to define different phenotypes from a large cohort of patients diagnosed with dermatomyositis who were positive to anti-MDA5 antibodies.Methods:We retrospective analyzed the clinical and immunological data of 90 anti-MDA5 patients [50 female, 55.6%, mean (SD) age at diagnosis 47 (15.4) yrs.] with dermatomyositis recruited from a multicenter register in Spain (MEDRA5) including 30 hospitals. All the patients fulfill de International Myositis Classification Criteria (EULAR/ACR) for dermatomyositis (score >90%). Anti-MDA5 were detected by means of commercial immunoblot (EUROIMMUN©). The chi-square test was used to assess the relationships between qualitative variables. The Kruskal-Wallis test was used to compared medians between groups.Results:Sixty-six patients (73.3%) were diagnosed with clinically amyopathic dermatomyositis. Three different phenotypes linked with the anti-MDA5 antibody were identified. Group 1: patients with rapidly-ILD phenotype (28 patients, 31.1%), group 2: antisynthetase-like phenotype (23 patients, 25.5%), and group 3: non-ILD phenotype (39 patients, 43.3%). Clinical and immunological comparison between the groups disclosed that age at disease onset was higher (median, IQR) in patients from group 1 [53 (43-60)] vs. group 2 [46 (40-56)] or group 3 [42(41-51)] (p=0.01); disease onset was more frequent in spring in patients from group 1 (46.5%) than in the rest of the groups (21.7% and 28.9%) (p<0.01). Cancer was detected in 7 patients, only associated with myositis in 3 cases (3 years interval between cancer and dermatomyositis) without significant differences between phenotypes. Vasculitis (one case ANCA positive) was detected in 9 cases (6 limited to skin, 1 renal and 1 intestinal), 6 of them in the group 3 (statistical significance, in comparison with group 1 and 2, p<0.01). Mortality rate was higher in group 1 (51.9%, 16 out of 17 due to refractory respiratory failure) vs group 2 (12.5%) or 3 (0%) (p<0.001). Anti Ro52 positivity was more frequent in group 1 (65.4%) vs. group 2 (25%) or 3 (35.5%) (p<0.017), although it did not reach statistical significance in terms of mortality (p=0.173) or patients admitted in the intensive care unit (p=0.173). Mechanic hands were more frequent in group 2 (40.6%) than in groups 1 (25%) and 3 (34.4%) (p=0.05). Fever was significantly most frequent in group 1(52.6%) than in group 2 (21.1%) and 3 (26.3%) (p=0.001). Other clinical or immunological features such as arthritis, myositis, or the number of characteristic skin lesions among others were not more frequent in one group or another.Conclusion:Three different phenotypes of patients positive to anti-MDA5 were identified. The presence or not of ILD, or the different type (rapidly progressive or not) of ILD were the main feature that allow to differentiate these phenotypes, which are relevant in clinical practice.References:[1]Allenbach Y, Uzunhan Y, Toquet S, et al; French Myositis Network. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases. Neurology. 2020;95: e70-e78.Acknowledgements:List of contributors of MEDRA5 group: Aguilar-García J (Internal Medicine, Hospital Costa del Sol, Marbella), Carrión-Barberá I (Rheumatology, Hospital del Mar, Barcelona), Cobo-Ibañez T (Rheumatology, Hospital Infanta Sofía, San Sebastián de los Reyes), de Escalante-Yangüela B (Internal Medicine, Hospital Clínico Lozano Blesa, Zaragoza), Fonseca-Aizpuru EM (Internal Medicine, Hospital de Cabueñes, Gijón), González-Cubillo L (Intensive Medicine, Hospital Universitario de Cruces, Barakaldo), González-Gay MA (Rheumatology, Hospital Marqués de Valdecilla, Santander), Prieto-González S (Internal Medicine, Hospital Clinic, Barcelona), Ruiz-Román A (Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla), Calero-Paniagua I (Internal Medicine, Hospital Virgen de la Luz, Cuenca), Callejas-Rubio JL (Internal Medicine, Hospital Clínico San Cecilio, Granada), Gil-Vila A (Internal Medicine, Hospital Vall d’Hebron, Barcelona), de Miguel-Campo B (Internal Medicine, Hospital Doce de Octubre, Madrid), García-Sevilla R (Pneumology, Hospital General Universitario de Alicante, Alicante), Iriarte-Fuster A (Internal Medicine, Hospital de Bellvitge, Hospitalet de Llobregat), Jovani-Casano V (Rheumatology, Hospital General Universitario de Alicante, Alicante), Lozano-Rivas N (Rheumatology, Hospital Virgen de la Arritxaca, Murcia), Martín-Gascón M (Internal Medicine, Hospital Morales Meseguer, Murcia), Martinez-González O (Rheumatology, Hospital Universitario de Salamanca, Salamanca), Monteagudo-Jiménez M (Internal Medicine, Hospital Parc Taulí, Sabadell), Mora-Ortega GM (Pneumology, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes), Moral-Moral Pedro (Internal Medicine, Hospital Universitari i Politecnic La Fe, Valencia), Pérez-De Pedro I (Interna Medicine, Hospital Regional Universitario de Málaga, Málaga), Picazo-Talavera MR (Rheumatology, Hospital del Sureste, Madrid), Rubio-Rivas M (Internal Medicine, Hospital de Bellvitge, Hospitalet de Llobregat)Disclosure of Interests:None declared


2021 ◽  
Vol 5 (3) ◽  
pp. 297-300
Author(s):  
Anna Eversman ◽  
Sayeeda Ahsanuddin ◽  
Joel Saltzman ◽  
Paula Silverman ◽  
Mara Beveridge

Background: Approximately 20% of patients displaying skin changes characteristic of dermatomyositis do not develop clinical or enzymatic evidence of muscle inflammation within 6 months of onset, leading to a diagnosis of clinically amyopathic dermatomyositis (CADM). While the association between dermatomyositis and malignancy is well-documented, the clinical significance of CADM is still being elucidated. Case Presentation: We report a case of new-onset CADM associated with recurrent esophageal adenocarcinoma for clinical interest and to add to the limited literature on CADM. Conclusion: To date, there is only one report of concurrent CADM and esophageal cancer. We present this case for clinical interest and to add to the limited literature on CADM.


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