Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

Abstract

BackgroundAnti-glycyl-tRNA synthetase (anti-EJ) antibody is occasionally positive in patients with interstitial lung disease (ILD). We aimed to define the clinical, radiological and pathological features of patients with anti-EJ antibody-positive ILD (EJ-ILD).MethodsWe retrospectively analyzed the medical records of 12 consecutive patients with EJ-ILD who underwent surgical lung biopsy.ResultsThe median follow-up time was 74 months (range, 17–115 months). The median age was 62 years (range, 47–75 years). Seven of 12 patients were female. Eight patients presented with acute onset. Six patients eventually developed polymyositis/dermatomyositis. On high-resolution computed tomography, consolidation and volume loss were predominantly observed in the middle or lower lung zone. Nine patients presented pathologically nonspecific interstitial pneumonia with organizing pneumonia, alveolar epithelial injury and prominent interstitial cellular infiltrations whereas the other three patients were diagnosed with unclassifiable interstitial pneumonia. Although all patients but one improved with the initial immunosuppressive therapy, five patients relapsed. When ILD relapsed, four of the five patients were treated with corticosteroid monotherapy. Four of the six patients without relapse have been continuously treated with combination therapy of corticosteroid and immunosuppressant.ConclusionsPatients with EJ-ILD often had acute onset of ILD with lower lung-predominant shadows and pathologically nonspecific interstitial pneumonia or unclassifiable interstitial pneumonia with acute inflammatory findings. Although the disease responded well to the initial treatment, relapse was frequent. Because of the diversity of the clinical courses, combination therapy of corticosteroid and immunosuppressant should be on the list of options to prevent relapse of EJ-ILD.

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

“…Previous studies have shown that combination therapy improved the findings of PFTs and chest HRCT scans in acute or subacute progressive PM/DM-ILD [22, 25-27]. We previously reported the importance of combination therapy, which should be on the list of options to prevent relapse in anti-EJ ILD [10]. However, it is controversial whether all ARS-ILD patients need corticosteroids in addition to the other immunosuppressive drugs they are taking.…”

“…Although some patients with rapidly progressive ARS-ILD have a poor prognosis in the short run, their overall prognosis is relatively good [6, 7]. However, because of frequent recurrence and ARS-ILD refractory to therapy, some patients progress to pulmonary fibrosis over the long run despite initial improvement [8-10]. Some patients are forced to use home oxygen therapy, while a few undergo lung transplantation in clinical practice.…”

Clinical significance of soluble CD163 in polymyositis-related or dermatomyositis-related interstitial lung disease

“…Interstitial lung disease (ILD) is a common complication seen in 20–78% of patients with PM/DM and can be a prognostic determinant [3–8]. Particularly, patients with DM and clinically amyopathic DM (CADM) frequently develop an acute and severe form of ILD [4, 5].…”

Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia

“…First, treatment response at 1 year (including at 1 year ± 6 months) based on changes in pulmonary function testing (PFT) results was defined as the %change of the initial value. Patients were assigned to the Improved or Worsened group based on increases or decreases, respectively, of >10% in FVC and >15% %diffusing capacity of lung carbon monoxide (%DL CO ) . Second, if PFT was repeatedly performed at least 12 months after the initial assessment, long‐term change in respiratory function was evaluated.…”

“…Other diagnoses in the remaining 30 patients included acute inflammatory findings such as organizing pneumonia and alveolar epithelial injury and interstitial cellular infiltration surrounded by hyaline membrane collapse, which was more prominent in unclassifiable IP than in f-NSIP as we reported previously. 15 We thought these subjects were comparable to a variant of organizing pneumonia with supervening fibrosis or a fibrosing variant of organizing pneumonia. 3,16 Patients with CTD fulfilled each of the standard criteria.…”