title: Dermatopulmonary Syndrome Associated With Anti-MDA5 Antibodies After Allogeneic Hematopoietic Stem Cell Transplantation.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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 patients after AHSCT without cGVHD were used as controls.
  
  
  METHODS
  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
  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.
authors.affiliation: INSERM UMRS 976, Laboratory of Oncodermatology, Immunology and Cutaneous Stem Cells, Hôpital Saint-Louis, F-75010 Paris, France2Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Clémence
authors.initials: C
authors.lastname: Lepelletier
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France4Service d'Immunobiologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Djaouida
authors.initials: D
authors.lastname: Bengoufa
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France4Service d'Immunobiologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Zeltni
authors.initials: Z
authors.lastname: Lyes
authors.affiliation: INSERM UMRS 976, Laboratory of Oncodermatology, Immunology and Cutaneous Stem Cells, Hôpital Saint-Louis, F-75010 Paris, France2Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Adèle
authors.initials: A
authors.lastname: de Masson
authors.affiliation: INSERM UMRS 976, Laboratory of Oncodermatology, Immunology and Cutaneous Stem Cells, Hôpital Saint-Louis, F-75010 Paris, France2Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: François
authors.initials: F
authors.lastname: Chasset
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Jachiet
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France5Service d'Hématologie-Greffes, AP-HP, Hôpital Saint-Louis F-75010 Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Michonneau
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France5Service d'Hématologie-Greffes, AP-HP, Hôpital Saint-Louis F-75010 Paris, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Robin
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France5Service d'Hématologie-Greffes, AP-HP, Hôpital Saint-Louis F-75010 Paris, France.
authors.forename: Régis
authors.initials: R
authors.lastname: Peffault de Latour
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France5Service d'Hématologie-Greffes, AP-HP, Hôpital Saint-Louis F-75010 Paris, France.
authors.forename: Flore
authors.initials: F
authors.lastname: Sicre de Fontbrune
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France6Service de Pneumologie, AP-HP, Hôpital Saint-Louis, F-75010 Paris, France.
authors.forename: Yacine
authors.initials: Y
authors.lastname: Tandjaoui-Lambiotte
authors.affiliation: INSERM UMRS 976, Laboratory of Oncodermatology, Immunology and Cutaneous Stem Cells, Hôpital Saint-Louis, F-75010 Paris, France2Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France.
authors.forename: Armand
authors.initials: A
authors.lastname: Bensussan
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Rybojad
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France6Service de Pneumologie, AP-HP, Hôpital Saint-Louis, F-75010 Paris, France7UMR1153 CRESS, Biostatistics and Clinical Epidemiology research team, F-75010 Paris, France.
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Tazi
authors.affiliation: INSERM UMRS 976, Laboratory of Oncodermatology, Immunology and Cutaneous Stem Cells, Hôpital Saint-Louis, F-75010 Paris, France2Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France5Service d'Hématologie-Greffes, AP-HP, Hôpital Saint-Louis F-75010 Paris, France8INSERM UMRS 1160, Hôpital Saint-Louis, F-75010 Paris, France.
authors.forename: Gérard
authors.initials: G
authors.lastname: Socié
authors.affiliation: Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France6Service de Pneumologie, AP-HP, Hôpital Saint-Louis, F-75010 Paris, France7UMR1153 CRESS, Biostatistics and Clinical Epidemiology research team, F-75010 Paris, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Bergeron
authors.affiliation: INSERM UMRS 976, Laboratory of Oncodermatology, Immunology and Cutaneous Stem Cells, Hôpital Saint-Louis, F-75010 Paris, France2Université Paris Diderot-Paris VII, Sorbonne Paris Cité, France3Service de Dermatologie, AP-HP, Hôpital Saint-Louis, Paris, France.
authors.forename: Jean-David
authors.initials: JD
authors.lastname: Bouaziz