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Brief Report
November 16, 2016

Dermatopulmonary Syndrome Associated With Anti-MDA5 Antibodies After Allogeneic Hematopoietic Stem Cell Transplantation

Author Affiliations
  • 1INSERM UMRS 976, Laboratory of Oncodermatology, Immunology and Cutaneous Stem Cells, Hôpital Saint-Louis, F-75010 Paris, France
  • 2Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France
  • 3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France
  • 4Service d’Immunobiologie, AP-HP, Hôpital Saint-Louis, Paris, France
  • 5Service d’Hématologie-Greffes, AP-HP, Hôpital Saint-Louis F-75010 Paris, France
  • 6Service de Pneumologie, AP-HP, Hôpital Saint-Louis, F-75010 Paris, France
  • 7UMR1153 CRESS, Biostatistics and Clinical Epidemiology research team, F-75010 Paris, France
  • 8INSERM UMRS 1160, Hôpital Saint-Louis, F-75010 Paris, France
JAMA Dermatol. Published online November 16, 2016. doi:10.1001/jamadermatol.2016.3976
Key Points

Question  Do anti-MDA5 antibodies have a clinical significance after allogeneic stem cell transplantation (AHSCT)?

Findings  Six of 83 patients who underwent AHSCT had anti-MDA5 antibodies; 4 of the 6 patients with anti-MDA5 antibodies demonstrated specific clinical features, including severe respiratory symptoms, of the anti-MDA5 syndrome.

Meaning  Patients who have undergone AHSCT with suggestive cutaneous or pulmonary symptoms should be screened for anti-MDA5 antibodies.

Abstract

Importance  Chronic graft-vs-host-disease (cGVHD) after allogeneic stem cell transplantation (AHSCT) may resemble autoimmune diseases. Anti-MDA5 (melanoma differentiation–associated gene 5) dermatopulmonary syndrome is a subset of dermatomyositis defined by specific clinical features and detection of anti-MDA5-antibodies in the serum.

Objective  To characterize the clinical features of patients who underwent AHSCT and screened positively for anti-MDA5 antibodies.

Design, Setting, and Participants  For this monocentric retrospective study, we exained 81 patients screened for anti-MDA5 antibodies at a specific dermatological or pulmonary postallograft consultation between January 2014 to September 2015 at a National Reference Center; 2 additional patients not seen at this consultation but having clinical features suggestive of anti-MDA5 syndrome were included. Twenty serum samples from of patients after AHSCT without cGVHD were used as controls.

Main Outcomes and Measures  Anti-MDA5 antibodies screened using an immunodot assay.

Results  Of 83 patients who underwent AHSCT (mean [SD] age, 47 [14] years), 6 patients tested positive for anti-MDA5 antibodies (mean [SD] age, 43 [16] years) including 4 patients with interstitial lung disease and 3 patients with cutaneous clinical features similar to anti-MDA5 skin symptoms encountered in patients who have not undergone AHSCT, namely finger pad inflammation, palmar violaceous papules, and digital ulcerations. Three patients had severe respiratory symptoms resistant to systemic steroids, and 1 patient died of severe interstitial lung disease.

Conclusions and Relevance  The clinical features and long-term prognosis of patients who underwent AHSCT and test positively for anti-MDA5 antibodies should be evaluated in large prospective studies.

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