Analysis of systemic lupus erythematosus-related interstitial pneumonia: a retrospective multicentre study

Sci Rep. 2019 May 14;9(1):7355. doi: 10.1038/s41598-019-43782-7.

Abstract

Thoracic diseases in patients with systemic lupus erythematosus (SLE), especially interstitial pneumonia (SLE-IP), are rare and have been poorly studied. The aims of this multicentre study were to evaluate SLE-IP and elucidate its clinical characteristics and prognosis. Fifty-five patients with SLE-IP who had attended the respiratory departments of participating hospitals were retrospectively evaluated in this multicentre study. Clinical information, high-resolution computed tomography (HRCT), and surgical lung biopsy/autopsy specimens were analysed by respiratory physicians, pulmonary radiologists, and pulmonary pathologists. IP patterns on HRCT and lung specimens were classified based on the international classification statement/guideline for idiopathic interstitial pneumonias. The most frequent form of SLE-IP at diagnosis was chronic IP (63.6%), followed by subacute (20.0%), and acute IP (12.7%). Radiologically, the most common HRCT pattern was "Unclassifiable" (54%). Histologically, "Unclassifiable" was the most frequently found (41.7%) among 12 patients with histologically proven IP. Interestingly, accompanying airway diseases were present in nine of these patients (75%). In multivariate analysis, current smoking (hazard ratio [HR] 6.105, p = 0.027), thrombocytopenia (HR 7.676, p = 0.010), anti-double-strand DNA titre (HR 0.956, p = 0.027), and nonspecific interstitial pneumonia (NSIP) + organizing pneumonia (OP) pattern on HRCT (vs. NSIP, HR 0.089, p = 0.023) were significant prognostic factors. In conclusion, chronic IP was the most frequent form of IP in patients with SLE-IP, and "Unclassifiable" was the commonest pattern radiologically and histologically.

Publication types

  • Multicenter Study

MeSH terms

  • Acute Disease
  • Adolescent
  • Adult
  • Aged
  • Chronic Disease
  • Female
  • Humans
  • Lung / pathology
  • Lung Diseases, Interstitial / complications*
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / pathology
  • Lupus Erythematosus, Systemic / complications*
  • Male
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Tomography, X-Ray Computed
  • Young Adult