Session Information
Title: Systemic Lupus Erythematosus - Clinical Aspects and Treatment: Treatment and Management Studies
Session Type: Abstract Submissions (ACR)
Background/Purpose
Pulmonary involvement is frequent in systemic lupus erythematosus (SLE) and can affect the pleura, pulmonary vasculature, and parenchyma. The prevalence of ILD is lower in SLE than in the other CTDs (3-13%). While ILD in SLE is usually mild, it can be progressive or severe in some patients. We aimed to determine clinical and serological characteristics of SLE patients with symptomatic ILD in an outpatient longitudinal lupus cohort.
Methods
The Oklahoma Lupus Cohort consists of a longitudinal cohort of patients who meet 1997 ACR criteria for SLE. Patients are enrolled after informed consent and their clinical and serological data are collected at routine clinic visits. Patients with pulmonary fibrosis or interstitial lung disease were identified using the database, and matched with 5-6 age and gender matched SLE controls without ILD. Data was collected using retrospective chart review. All patients included ( n=110) fulfilled 1997 ACR criteria for SLE
Results
Fifteen SLE patients with a concurrent diagnosis of pulmonary fibrosis or ILD were identified among 517 in SLE cohort giving a prevalence of 2.9%. Fourteen of 15 patients had imaging reports available for review and all patients had radiographic evidence of parenchymal lung involvement. Most commonly reported abnormalities were ground glass opacities and interstitial thickening or fibrosis mostly involving basilar regions followed by traction bronchiectasis, reticular pattern and fibrotic NSIP. Subpleural honeycombing and UIP were least common.
American Indians were 27% of those with ILD and 13% of controls. African Americans were evenly divided (37% cases, 37% controls). 53% of ILD patients had a diagnosis of anti-phospholipid syndrome vs. 32% of controls. Cases had a high rate of serositis history (78% vs. 38% controls, P value 0.006), anti-dsDNA (53% vs 23% p=0.02) and lymphopenia (47% cases vs. 17% controls, P value 0.016). There was a trend to increased frequency of anti-La (43%), anti-Sm (43%), RNP (53%) and lupus anticoagulant (33%) in ILD vs non-ILD (18%, 24%, 37% and 13% respectively, p= 0.07, 0.2, 0.2 and 0.06).
There were only 2 patients positive for anti-Jo1 among 517 SLE patients (0.4%); one by laser immunobead assay and immunodiffusion (patient A), the other by immunobead assay only (patient B). Both were among the cases with significant ILD. Both were African American females; had cytoplasmic ANA pattern seen on IFA and were positive for anti ds-DNA. They had marked impairment of lung function with FVC 51% (patient A) and 22% (patient B) respectively. Only patient A had clinically significant myositis.
Conclusion
Clinically significant ILD is seen in a small proportion of SLE patients but can cause significant morbidity. Patients with ILD are more likely to have American Indian heritage, earlier history of serositis and positive anti-ds DNA. They may have a higher prevalence of anti-La (SS-B), anti-Sm, RNP and lupus anticoagulant. Testing for overlap syndromes with antibodies associated with ILD may provide useful prognostic information for these patients.
Disclosure:
S. Vaseer,
None;
J. A. James,
None;
A. Thanou,
Exagen,
2;
J. T. Merrill,
Genentech, Roche,
5.
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