QJM Advance Access published March 30, 2015 QJM: An International Journal of Medicine, 2015, 1–2 doi: 10.1093/qjmed/hcv068 Advance Access Publication Date: 17 March 2015 Case report

CASE REPORT

Anti-MDA5 dermatomyositis and progressive interstitial pneumonia M.G. Silveira1, A. Selva-O’Callaghan2, N. Ramos-Terrades3, K.V. Arredondo-Agudelo3, M. Labrador-Horrillo2 and C. Bravo-Masgoret1 From the 1Pneumology, 2Internal Medicine and 3Nephrology Department, Vall D’Hebron General Hospital, Universitat Autonoma de Barcelona, Barcelona 08035, Spain Address correspondence to A. Selva-O’Callaghan, C/ Siracusa N 12 Bis “A”, Barcelona 08012, Spain. email: [email protected]

In severe cases of anti-MDA5 positive clinically amyopathic dermatomyositis associated RP-ILD that do not respond to aggressive therapy, sequential therapy with hemoperfusion with polymyxin B and plasmapheresis may be a lifesaving strategy.

To the Editor, Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis characterized by the absence of myopathy. CADM patients testing positive to anti-MDA5 antibodies often have associated rapidly progressive interstitial lung disease (RP-ILD), which responds poorly to immunosuppressive treatment and has an unfavorable prognosis.1,2 Here, we report the case of a CADM patient with anti-MDA5 antibodies who developed severe RP-ILD that responded favorably to hemoperfusion with polymyxin B plus plasmapheresis and intravenous immunoglobulin administration. A 42-year-old man was admitted to hospital because of fever, cough and dyspnea, associated with a bilateral diffuse reticular pattern on chest X-ray. Broad-spectrum antibiotic therapy with azithromycin and ceftriaxone was started, with no clinical improvement. Sputum, blood cultures and serologies were negative. Empirical corticosteroid therapy was initiated at a dose of 1 mg/kg/day with a good response. Five months later, he had to be hospitalized again because of acute respiratory failure associated with cough and fever. His oxygen saturation (SO2) was low and required placement of an oxygen reservoir mask at flow rates of 10–12 liters per minute to maintain SO2 at

94%. He also showed considerable orthodeoxia, with an SO2 drop of up to 80% in sitting position or on minimal activity. Physical examination revealed fine crackles at the lung bases and signs of subcutaneous emphysema in chest, neck and upper limbs. Skin thickening was observed in both hands (mechanic’s hands) as well as Gottron’s papules. Chest highresolution computed tomography showed substantial diffuse pulmonary fibrosis, traction bronchiectasis and septal thickening, with significant pneumomediastinum. Cultures and viral serologies were negative. Despite antibiotic administration, his symptoms worsened. Based on a clinical suspicion of CADM with associated RP-ILD, anti-MDA5 antibodies were determined by ELISA and confirmed by blot, yielding highly positive results. Treatment with three pulses of methylprednisolone (500 mg) and tacrolimus 2 mg/12 h was initiated, but the patient continued to worsen and required intensive care unit admission. Because of his lack of response and need for high-flow oxygen therapy, we decided to apply hemoperfusion with polymyxin B (Toraymyxin 20R, Toray Medical Co., Tokyo, Japan) at a flow of 100 ml/h for 3 h once daily on two successive days. Although oxygen saturation improved slightly, the severe orthodeoxia persisted. Hence, a plasmapheresis scheme was carried out with replacement of 3.5 l of seroalbumin 5% followed by intravenous immunoglobulin infusion (0.4 mg/kg). Along the following 2 weeks, the patient gradually improved, oxygen requirements decreased and orthodeoxia resolved. Two months later, high-resolution computed tomography showed resolution of the pneumomediastinum and subcutaneous and interstitial emphysema, and a considerable improvement in lung fibrosis (Figure 1). The patient was discharged without oxygen therapy: SO2 in room air was 95%, forced vital capacity (FVC) 42% and

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marker of this severe manifestation is a valuable aid to reaching a prompt diagnosis and enabling the start of aggressive therapy.1,2 To date, outcomes have been poor with various immunosuppressive drugs. Several case reports of patients with CADM-associated RP-ILD have shown that hemoperfusion with polymyxin B use dramatically improves the prognosis of this severe disease.3 In the case reported here, initial administration of hemoperfusion with polymyxin B yielded only a slight transient improvement; plasmapheresis together with intravenous immunoglobulin and tacrolimus administration was needed to achieve a good outcome.

Funding This study was funded in part by a grant (FIS/2012 PI12–01320) from the Spanish Ministry of Health and Consumer Affairs. Conflict of interest: None declared.

References

diffusing capacity for carbon monoxide (DLCO) was non-evaluable. Six months later, his FVC had increased to 67% and DLCO was 25%. Management of CADM patients with associated RP-ILD is a challenge for clinicians. The recent discovery of anti-MDA5 as a

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Figure 1. Chest high-resolution computed tomography images at onset (a) and 2 months after therapy (b).

1. Gono T, Kawaguchi Y, Satoh T Kuwana M, Katsumata Y, Takagi K, et al. Clinical manifestation and prognostic factor in anti-melanoma differentiation associated gene antibodyassociated interstitial lung disease as a complication of dermatomyositis. Rheumatology 2010; 49:1713–9. 2. Labrador-Horrillo M, Martinez MA, Selva-O’Callaghan A, Trallero-Araguas E, Balada E, Vilardell-Tarres M, et al. AntiMDA5 antibodies in a large Mediterranean population of adults with dermatomyositis. J Immunol Res 2014; 2014:290797. 3. Teruya A, Kawamura K, Ichikado K, Sato S, Yasuda Y, Yoshioka M. Successful polymyxin B hemoperfusion treatment associated with serial reduction of serum antiCADM-140/MDA5 antibody levels in rapidly progressive interstitial lung disease with amyopathic dermatomyositis. Chest 2013; 144:1934–6.

Anti-MDA5 dermatomyositis and progressive interstitial pneumonia.

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