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      Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis

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          Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management.

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          Most cited references 21

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          The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM.

          Interstitial lung disease (ILD), especially rapidly progressive ILD (RPILD), is a major poor prognostic factor in patients with DM. We investigated the association of anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab) with clinical characteristics and mortality in Japanese patients with DM. Seventy-nine DM patients, comprising 58 classic DM and 21 clinically amyopathic DM (CADM) patients, were enrolled. Serum Abs were screened by immunoprecipitation assays, and an immunosorbent assay (ELISA) was used for MDA5. The relationships of clinical characteristics and mortality with each Ab were investigated. Anti-MDA5 Ab was detected in 17 patients. Anti-clinically amyopathic DM 140  kDa polypeptide Abs (anti-CADM-140 Abs) were found in 16 of the 17 anti-MDA5 Ab(+) patients. Skin ulcers, palmar papules, CADM, RPILD and mediastinal emphysema were widely distributed in anti-MDA5 Ab(+) patients. Mortality at 6 months as well as 5 years was also significantly higher in anti-MDA5 Ab(+) patients than in anti-MDA5 Ab(-) patients. In a multivariable Cox regression analysis, mortality was independently associated with anti-MDA5 Ab (relative hazard 6.33; 95% CI 1.43, 28.0). All of the deaths in anti-MDA5 Ab(+) patients were attributed to respiratory failure of RPILD; however, RPILD did not worsen in any of the anti-MDA5 Ab(+) patients who survived the first 6 months. The presence of anti-MDA5 Ab identifies the characteristic skin, musculoskeletal, pulmonary and prognostic features in patients with DM. In addition, anti-MDA5 Ab seems to predict a group of patients with CADM-complicated fatal RPILD.
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            Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis.

            Acute/subacute interstitial pneumonia (A/SIP) in patients with polymyositis/dermatomyositis (PM/DM) is frequently fatal within months despite high dose prednisolone (PSL) therapy. Our objective was to improve the survival rate of patients with A/SIP associated with PM/DM; and to characterize patients with PM/DM who are at high risk of developing A/SIP. We conducted a pilot trial of combined immunosuppressive therapy with high dose PSL, 10-30 mg/kg of intravenous pulse cyclophosphamide (IVCYC) every 3-4 weeks, and 2-4 mg/kg/day of cyclosporin A (CSA) for patients with A/SIP. A/SIP was diagnosed based on a history of rapidly worsening respiratory symptoms, progressive radiological findings or hypoxemia, and compatible findings in high resolution computed tomography images. Before December 2000, 12 patients with DM among 83 PM/DM patients developed A/SIP, and 9 patients died despite treatment using high dose PSL with or without a choice of CSA, cyclophosphamide, or azathioprine. Thereafter, 10 patients with DM among 27 PM/DM patients developed A/SIP, and they were given combination therapy with PSL, CSA, and IVCYC. Five patients survived and are doing well for more than 2 years, although the remaining 5 patients died of respiratory failure within 3 months. DM patients with A/SIP showed the following characteristic features: mild myositis, palmar papule, fever, and negative or low titer of antinuclear antibody. Immediate institution of intensified immunosuppressive therapy should be considered for patients with A/SIP complicating DM. However, even early recognition of A/SIP and immediate commencement of a regimen including CSA and IVCYC in addition to high dose PSL may not be sufficient for some of those patients.
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              Increased ferritin predicts development and severity of acute interstitial lung disease as a complication of dermatomyositis.

              Acute/subacute interstitial pneumonia (A/SIP) is an intractable and fatal complication of DM. Since a useful indicator predicting the complication of A/SIP has not been found, the aim of this study was to determine whether serum ferritin is a potential predictive indicator of the occurrence of A/SIP in 64 patients with DM. Of the total patients enrolled, 19 had A/SIP, 24 had chronic interstitial pneumonia and 21 were without interstitial lung disease (ILD). Clinical manifestations and laboratory data were obtained from medical records on admission. Serum ferritin levels were extremely high in patients with DM with A/SIP. It was significantly higher in DM with A/SIP than that in DM without A/SIP (median 790 vs 186 ng/ml; P or =1500 ng/ml than the rate in the group with ferritin levels 1500 ng/ml.

                Author and article information

                Clin Pract
                Clin Pract
                Clinics and Practice
                22 April 2021
                June 2021
                : 11
                : 2
                : 235-240
                [1 ]Department of Medicine, Pneumology Unit, Cayenne Hospital Center Andrée Rosemon, F-97306 Cayenne, France
                [2 ]Department of Pathology and Centre of Biological Resources (CRB Amazonie), Cayenne Hospital Center Andrée Rosemon, F-97306 Cayenne, France; Kinan.drak.alsibai@ 123456gmail.com
                [3 ]Centre of Biological Resources (CRB Amazonie), Cayenne Hospital Center AndréeRosemon, F-97306 Cayenne, France
                [4 ]Chest Diseases Department, Strasbourg University Hospital, F-67000 Strasbourg, France; naji.khayath@ 123456chru-strasbourg.fr
                Author notes
                [* ]Correspondence: anesayyoub@ 123456gmail.com ; Tel.: +33-766-833-526; Fax: 33-594-395-351

                Current address: Department of Medicine, Pulmonology Unit, Cayenne Hospital Center, Avenue des Flamboyants BP 6006, F-97306 Cayenne, France.

                © 2021 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

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