Evaluation of clinical prognostic factors for interstitial pneumonia in anti-MDA5 antibody-positive dermatomyositis patients

Mod Rheumatol. 2018 Jan;28(1):133-140. doi: 10.1080/14397595.2017.1318468. Epub 2017 May 11.

Abstract

Objectives: We retrospectively investigated clinical prognostic factors for interstitial pneumonia (IP) in anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab)-positive dermatomyositis (DM) patients.

Methods: Subjects comprised 18 patients with anti-MDA5 Ab-positive DM-IP (9 survivors; 9 deaths).

Results: Initial serum albumin levels, ferritin levels, and ground-glass opacity (GGO) scores in the right middle lobes were significantly higher in the death group than in the survivor group (p = .033, .013, and .005, respectively). Initial alveolar-arterial oxygen gradient (P[A-a]O2) was also higher in the death group than in the survivor group (p = .064). Initial serum ferritin, P[A-a]O2, and right middle lobe GGO score were found to significantly relate to death. Survival rates after 24 weeks were significantly lower among patients with an initial ferritin level of ≥450 ng/mL (25%), P[A-a]O2 of ≥30 mmHg (31%), and a right middle lobe GGO score of ≥2 (11%) than each of the others (p = .006, .020, and .002, respectively).

Conclusions: An initial serum ferritin level of ≥450 ng/mL, P[A-a]O2 of ≥30 mmHg, and right middle lobe GGO score of ≥2 (GGO ≥5% of the lobe) were identified as poor prognostic factors for anti-MDA5 Ab-positive DM-IP patients.

Keywords: Dermatomyositis; interstitial pneumonia; prognosis.

MeSH terms

  • Adult
  • Aged
  • Autoantibodies / blood*
  • Dermatomyositis / blood
  • Dermatomyositis / complications
  • Dermatomyositis / immunology
  • Dermatomyositis / mortality*
  • Female
  • Humans
  • Interferon-Induced Helicase, IFIH1 / immunology*
  • Lung Diseases, Interstitial / blood
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / immunology
  • Lung Diseases, Interstitial / mortality*
  • Male
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Survival Rate

Substances

  • Autoantibodies
  • IFIH1 protein, human
  • Interferon-Induced Helicase, IFIH1