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.
Highlights
We used data from high-resolution computed tomography (HRCT) and surgical lung biopsy (SLB)/autopsy specimens to evaluate details of IP patterns and classify them based on the international classification statement/guideline for idiopathic interstitial pneumonias (IIPs)[9] and idiopathic pulmonary fibrosis (IPF)[10]
We retrospectively studied data of patients with Systemic lupus erythematosus (SLE)-IP who had attended respiratory departments with a particular focus on radiologic and histopathologic patterns in these patients
According to IIPs/IPF classification statement/guidelines, “Unclassifiable” was the commonest pattern on both HRCT and SLB/autopsy specimens, and nonspecific interstitial pneumonia (NSIP) + organizing pneumonia (OP) pattern on HRCT was associated with a better prognosis than NSIP pattern
Summary
Lung involvement especially interstitial pneumonia (IP), occurs much less frequently in SLE than in other CTDs1. Many of the above-cited studies on lung involvement in SLE drew their patients from rheumatology departments; details of severe lung diseases in patients who have been managed in respiratory departments have not been well-established. Patients with SLE with neuropsychiatric[7] or renal involvement[8] are known to have poor prognoses, the prognostic significance of lung involvement has not been fully elucidated. In this multicentre retrospective study, we investigated patients with SLE and thoracic diseases who had been treated in respiratory departments and thoroughly evaluated those with SLE-related interstitial pneumonia (SLE-IP). To the best of our knowledge, this is the first study to comprehensively and precisely evaluate SLE-IP in a multicentre study by respiratory physicians, pulmonary radiologists, and pulmonary pathologists
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