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SAT0305 Interstitial Lung Disease (ILD) in Patients with Anti-Jo-1 Syndrome (AJS): A Descriptive Analysis
  1. B. Alhaddad1,
  2. C. O'Rourke2,
  3. M. Alalwani3,
  4. B. Zraik3,
  5. R. Yadav4,
  6. S. Chatterjee5
  1. 1Rheumatology, Metrohealth Medical Center
  2. 2Quantitative Health Sciences
  3. 3Internal Medicine
  4. 4Radiology
  5. 5Rheumatology, Cleveland Clinic, Cleveland, United States

Abstract

Objectives To evaluate the patterns, severity and prognostic parameters of ILD in AJS.

Methods We identified 51 anti-Jo-1 patients with ILD between 2003 and 2012. Clinical and laboratory data were obtained along with PFTs and pattern/scoring of thoracic HRCT scans based on Ooi scoring system (inflammation and fibrosis indices) (1). Progression of ILD was defined as follows: a decrease in DLCO and/or FVC (first to last reported) by >15%, an increase in fibrosis index or total HRCT score of ≥2, and death from respiratory failure.

Results Out of 51 patients; 20 were males and 15 had dermatomyositis. Median age at onset was 47 years, mean follow up duration was 6.6 years; 20% had ILD prior to AJS diagnosis. Arthritis, mechanic's hands, Raynaud phenomenon and fever were reported in 88%, 37%, 31%, and 23% respectively. Four patients had cancer: lung (1), prostate and gallbladder (1) and lymphoma (2). At diagnosis 3 patients had UIP, 13 (25%) had organizing pneumonia (3 with NSIP, 5 developed NSIP later); 36 (70%) had NSIP: 22 fibrotic, 2 mixed and 12 cellular; 75% of cellular NSIP became fibrotic on subsequent imaging. Mean DLCO and FVC at diagnosis were 58.9% and 67% respectively. Median initial inflammation and fibrosis indices at diagnosis were 5.6, 1.16 and at last follow up 4.8, 2.1 respectively. Among 45 patients with available follow up data; 17 had worsening ILD (39%) and 5 (11%) died of respiratory failure. Lower DLCO at diagnosis was a predictor of disease progression [50 vs. 65% (p=0.0290)]. All UIP patients progressed during follow up. Only 7 patients had a late diagnosis of ILD (≥3 years after onset of AJS). Of those; 5 (70%) had a DLCO ≤50% compared to 25% in those with early diagnosis. SSA positivity was detected in 53%; lower initial (53% vs. 62%) and last (56% vs. 67%) DLCO as well as higher fibrosis score at last follow up [3.5 vs. 0.9 (p=0.089)] were observed in SSA negative group. These differences did not reach statistical significance. Interestingly; 6 of 7 patients who died were SSA negative. Nine patients who developed pulmonary hypertension had higher median disease duration (10 years) and CT score (12.5) and all of them had DLCO ≤50%. Esophageal disease other than dysphagia was detected in 43% [GERD (33%), abnormal manometry or gastric emptying (15%) and dilatation on chest CT (10%)]. Esophageal disease had a trend towards lower DLCO at diagnosis [53 vs. 62% (p=0.084)], higher fibrosis score [2.7 vs. 1.6 (p=0.089)] and higher %change of total CT score [1.1 vs. -0.7 (p=0.007)].

Conclusions Lower DLCO at diagnosis was associated with progression of ILD. The lower DLCO observed in late onset ILD may suggest silent disease and re-emphasize the rule of screening for ILD in AJS. In contrast with previous studies; SSA co-positivity was not associated with adverse outcome. Esophageal disease seemed to be prevalent in anti-Jo-1 patients with ILD and may predict worse fibrosis. The association of malignancy in AJS was not significant.

References

  1. Ooi et al; Interstitial lung disease in systemic sclerosis. Acta Radiol. 2003 May;44(3):258-64.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.3984

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