title: [Dermatomyositis associated with anti-MDA5 antibodies and pneumocystis pneumonia: Two lethal cases].
abstract: BACKGROUND
  Dermatomyositis associated with anti-MDA-5 autoantibodies is a recently-described clinical entity. Herein we report two lethal cases involving pneumocystis pneumonia.
  
  
  METHODS
  Case no1. A 56-year-old male patient developed cutaneous symptoms consistent with dermatomyositis without muscular involvement. Antinuclear antibodies were present and anti-MDA5 auto-antibodies were identified. The scan showed interstitial lung disease without infection. Significant improvement was obtained with corticosteroids. One month later, the patient presented acute respiratory illness (hypoxemia: PaO260mmHg, exacerbation of lung disease evidenced by a scan, and diagnosis of pneumocystis pneumonia on bronchoalveolar lavage). He died despite appropriate antibiotic therapy and immunosuppressant therapy. Case no2. The second case concerned a 52-year-old Vietnamese man who developed more atypical cutaneous symptoms of dermatomyositis without muscular involvement. ANAb responses were positive (1/400) and MDA5 was present. The patient was treated with corticosteroids (40mg/d), hydroxychloroquine, and intravenous immunoglobulin. After significant improvement, the patient developed an acute respiratory illness due to superinfection with pneumocystis and he died despite specific treatment and cyclophosphamide bolus.
  
  
  CONCLUSIONS
  In dermatomyositis, anti-MDA5 antibody screening is essential for the prognosis since the disease carries a risk of complication with severe lung disease. Bronchial fibroscopy with bronchoalveolar lavage should be considered at the time of diagnosis. Our two cases suggest the need for early screening for pneumocystis pneumonia in the event of respiratory distress and possibly for prophylactic treatment at the start of immunosuppressant therapy.
authors.affiliation: Service de dermatologie, hôpital militaire Ste-Anne, 83800 Toulon, France.
authors.forename: M
authors.initials: M
authors.lastname: Aymonier
authors.affiliation: Service de dermatologie, hôpital militaire Ste-Anne, 83800 Toulon, France.
authors.forename: S
authors.initials: S
authors.lastname: Abed
authors.affiliation: Service de dermatologie, hôpital militaire Ste-Anne, 83800 Toulon, France.
authors.forename: T
authors.initials: T
authors.lastname: Boyé
authors.affiliation: Service de pneumologie, hôpital militaire Ste-Anne, 83800 Toulon, France.
authors.forename: H
authors.initials: H
authors.lastname: Barazzutti
authors.affiliation: Service de dermatologie, hôpital militaire Ste-Anne, 83800 Toulon, France.
authors.forename: B
authors.initials: B
authors.lastname: Fournier
authors.affiliation: Service de dermatologie, hôpital militaire Ste-Anne, 83800 Toulon, France. Electronic address: morandjj@aol.com.
authors.forename: J-J
authors.initials: JJ
authors.lastname: Morand
mesh_terms: D000974:Antibodies, Antinuclear / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D060085:Coinfection; D003882:Dermatomyositis / Q000150:complications* / Q000276:immunology; D004198:Disease Susceptibility; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000072640:Interferon-Induced Helicase, IFIH1 / Q000097:blood / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D018410:Pneumonia, Bacterial / Q000000981:diagnostic imaging / Q000209:etiology; D011020:Pneumonia, Pneumocystis / Q000000981:diagnostic imaging / Q000209:etiology*; D011379:Prognosis; D011552:Pseudomonas Infections / Q000000981:diagnostic imaging / Q000209:etiology; D012128:Respiratory Distress Syndrome / Q000209:etiology; D014057:Tomography, X-Ray Computed
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D007166:Immunosuppressive Agents; C452815:IFIH1 protein, human; D000072640:Interferon-Induced Helicase, IFIH1
keywords: Anti-MDA5 auto-antibodies; Anticorps anti-MDA5; Dermatomyosite; Dermatomyositis; Pneumocystis pneumonia; Pneumocystose pulmonaire