Dramatic improvement of anti-SS-A/Ro-associated interstitial lung disease after immunosuppressive treatment

Rheumatol Int. 2016 Jul;36(7):1015-21. doi: 10.1007/s00296-016-3463-x. Epub 2016 Mar 28.

Abstract

The aim of the study was to report three patients affected by interstitial lung disease associated with positive anti-SS-A/Ro autoantibody who showed a dramatic improvement after immunosuppressive treatment. Medical charts were reviewed to obtain clinical data, laboratory parameters, lung function tests, high-resolution computed tomography results and response to immunosuppressive treatment. The three patients showed a clinical picture of a lung-dominant connective tissue disease characterized by a sudden onset with dyspnea, cough and subtle extrathoracic features together with positive anti-SS-A/Ro antibody and weak titer antinuclear antibodies. All three patients responded favorably to immunosuppressive therapy: Two cases were treated with a combination of corticosteroid and cyclophosphamide followed by mycophenolate mofetil; in the third patient, clinical benefit was obtained after rituximab was added to corticosteroid and immunosuppressant drug. In spite of an abrupt onset with significant lung function impairment, all three patients had a favorable clinical response to immunosuppressive therapy. This report may be useful in making therapeutic decisions in case of interstitial lung disease associated with anti-SS-A antibody.

Keywords: Anti-SS-A/Ro autoantibody; Immunosuppressive treatment; Interstitial lung disease.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged, 80 and over
  • Antibodies, Antinuclear / blood*
  • Biomarkers / blood
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Lung / drug effects*
  • Lung / immunology
  • Lung / physiopathology
  • Lung Diseases, Interstitial / blood
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / immunology
  • Lung Diseases, Interstitial / physiopathology
  • Middle Aged
  • Mycophenolic Acid / therapeutic use
  • Recovery of Function
  • Remission Induction
  • Rituximab / therapeutic use
  • Time Factors
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Antibodies, Antinuclear
  • Biomarkers
  • Immunosuppressive Agents
  • SS-A antibodies
  • Rituximab
  • Cyclophosphamide
  • Mycophenolic Acid