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. 2020 Aug;50(4):776-790.
doi: 10.1016/j.semarthrit.2020.03.007. Epub 2020 Jun 1.

Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease

F Romero-Bueno  1 , P Diaz Del Campo  2 , E Trallero-Araguás  3 , J C Ruiz-Rodríguez  4 , I Castellvi  5 , M J Rodriguez-Nieto  6 , M J Martínez-Becerra  7 , O Sanchez-Pernaute  1 , I Pinal-Fernandez  8 , X Solanich  9 , T Gono  10 , M A Gonzalez-Gay  11 , M N Plana  12 , A Selva-O'Callaghan  13 ; MEDRA5 (Spanish MDA5 Register) group (listed contributors at the end of the article)
Affiliations

Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease

F Romero-Bueno et al. Semin Arthritis Rheum. 2020 Aug.

Abstract

Objectives: The study aimed to develop evidence-based recommendations for the treatment of rapidly progressive interstitial lung disease (RPILD) associated with the anti-Melanoma Differentiation-Associated Gene 5-positive dermatomyositis (DM) syndrome.

Methods: The task force comprised an expert panel of specialists in rheumatology, intensive care medicine, pulmonology, immunology, and internal medicine. The study was carried out in two phases: identifying key areas in the management of DM-RPILD syndrome and developing a set of recommendations based on a review of the available scientific evidence. Four specific questions focused on different treatment options were identified. Relevant publications in English, Spanish or French up to April 2018 were searched systematically for each topic using PubMed (MEDLINE), EMBASE, and Cochrane Library (Wiley Online). The experts used evidence obtained from these studies to develop recommendations.

Results: A total of 134 studies met eligibility criteria and formed the evidentiary basis for the recommendations regarding immunosuppressive therapy and complementary treatments. Overall, there was general agreement on the initial use of combined immunosuppressive therapy. Combination of high-dose glucocorticoids and calcineurin antagonists with or without cyclophosphamide is the first choice. In the case of calcineurin antagonist contraindication or treatment failure, switching or adding other immunosuppressants may be individualized. Plasmapheresis, polymyxin B hemoperfusion and/or intravenous immunoglobulins may be used as rescue options. ECMO should be considered in life-threatening situations while waiting for a clinical response or as a bridge to lung transplant.

Conclusions: Thirteen recommendations regarding the treatment of the anti-MDA5 positive DM-RPILD were developed using research-based evidence and expert opinion.

Keywords: Basiliximab; Cyclophosphamide; Cyclosporine; Dermatomyositis; Extracorporeal membrane oxygenation; Glucocorticoid; Intensive care; Intravenous immunoglobulins; Lung transplant; Mycophenolate; Plasmapheresis; Polymixyn B hemoperfusion; Rapidly progressive interstitial lung disease; Review; Rituximab; Tacrolimus; Tofacitinib; systematic.

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Conflict of interest statement

All members of the Expert Panel completed the disclosure form, which requires disclosure of financial and other interests, including relationships with commercial entities that are reasonably likely to experience direct regulatory or commercial impact as a result of promulgation of the guideline. Categories for disclosure include employment; leadership; stock or other ownership; honoraria, consulting or advisory role; speaker’s bureau; research funding; patents, royalties, other intellectual property; expert testimony; travel, accommodations, expenses; and other relationships. In accordance with the Policy, the majority of the members of the expert panel did not disclose any relationships constituting a conflict under the Policy.

Figures

Fig. 1.
Fig. 1.
Diagnosis of RPILD in patients with anti MDA5 antibodies. *Combined therapy with glucocorticoids and calcineurin antagonists is recommended specially if some risk factors are present (>60 years old, hyperferritinemia >500 nm/mL, C reactive protein > 1 mg/dl). HRCT, High Resolution CT Scan. PFT, Pulmonary Function Tests. RP-ILD, Rapidly Progressive Interstitial Lung Disease. GGO, Ground Glass Opacity.
Fig. 2.
Fig. 2.
Treatment of RPILD in patients with anti MDA5 antibodies.
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See this image and copyright information in PMC

References

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