Clinical and HRCT features of amyopathic dermatomyositis associated with interstitial lung disease: A retrospective study of 128 patients with connective tissue disease-related interstitial lung disease.

BACKGROUND

This study retrospectively analyzed the laboratory data and chest images of patients with amyopathic dermatomyositis associated with interstitial lung disease (ADM-ILD) and patients with other connective tissue disease-related ILDs (CTD-ILDs) to find a characteristic index for the early recognition of ADM-ILD and help clinicians consider the possibility of ADM-ILD as soon as possible.

METHODS

In our cohort study, the records of 128 Chinese patients with CTD-ILD, including 33 ADM-ILD patients, 37 rheumatoid arthritis (RA)-ILD patients, 33 primary Sjogren's syndrome (pSS)-ILD patients, 14 systemic sclerosis (SSc)-ILD patients and 11 systemic lupus erythematosus (SLE)-ILD patients. The patients' clinical features, laboratory parameters, and chest HRCT findings were analyzed.

RESULTS

ADM-ILD patients generally had significantly higher LDH (333.52±160.21 U/L), AST (66.21±83.66 U/L), and CK-MB (18.23±8.28 U/L) levels than other CTD-ILD patients. A total of 90.91% (30/33) of ADM-ILD patients had elevated LDH. Patients with ADM-ILD were more prone to organizing pneumonia radiologic patterns on chest HRCT scans than patients with other CTD-ILDs (χ=37.39, p < 0.001) and were found in 18 of 33 ADM-ILD patients. Anti-MDA5 (45.45%) was the most commonly detected autoantibody in ADM-ILD patients, followed by anti-PL-7 (21.21%), anti-Jo-1 (12.12%), and anti-PL-12 (9.09%), and levels of ALT (96.93±119.79 vs. 17.50±6.218 U/L), AST (113.00±106.13 vs. 23.56±6.91 U/L), LDH (415.00±198.51 vs. 261.94±67.75 U/L) and CK-MB (22.57±5.91 vs. 14.61±8.36 U/L) were significantly higher in anti-MDA5-positive patients, but these patients had significantly lower WBC counts (4.82±2.61 vs. 7.14±3.00 × 10/L), lymphocyte counts (0.72±0.20 vs. 1.23±0.53 × 10/L), and ALB levels (31.90±4.76 vs. 35.49±4.71 g/L).

CONCLUSIONS

ADM-ILD patients have higher serum LDH, AST and CK-MB levels, especially serum LDH levels, and are more prone to organizing pneumonia radiologic patterns on chest HRCT scans than other CTD-ILD patients. A high level of serum LDH with ILD may be a useful characteristic index for recognizing ADM-ILD.