RT Journal Article
SR Electronic
T1 Clinical, radiological and pathological features of anti-MDA5 antibody-associated interstitial lung disease
JF RMD Open
JO RMD Open
FD EULAR
SP e003150
DO 10.1136/rmdopen-2023-003150
VO 9
IS 2
A1 Chen, Xixia
A1 Jiang, Wei
A1 Jin, Qiwen
A1 Peng, Qinglin
A1 Zhang, Lu
A1 Lin, Sang
A1 Lu, Xin
A1 Liu, Min
A1 Wang, Yuli
A1 Song, Aiping
A1 Feng, Ruie
A1 Wang, Guochun
YR 2023
UL http://rmdopen.bmj.com/content/9/2/e003150.abstract
AB Introduction To investigate the clinical, radiographic and pathological features of interstitial lung disease (ILD) in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+DM).Methods We retrospectively analysed the medical records of patients with anti-MDA5+DM who had undergone radiological examination, and lung histopathology was performed on 17 of them.Results This study examined 329 patients with anti-MDA5+DM, of whom 308 (93.6%) were diagnosed with ILD and 177 (53.8%) exhibited rapidly progressive ILD (RPILD). The most common radiographic patterns were organising pneumonia (OP) (43.2%), non-specific interstitial pneumonia (NSIP) (26.4%) and NSIP+OP (18.5%). Histological analysis showed NSIP (41.2%) and NSIP+OP (47.1%) to be the predominant patterns. However, in the 17 patients who underwent lung histopathology, the coincidence rate between radiological and histopathological diagnoses was only 11.8%. Compared with patients without RPILD, those with RPILD showed a higher prevalence of NSIP+OP (26.6% vs 10.7%, p=0.001) and a lower prevalence of NSIP pattern (21.5% vs 37.4%, p=0.002) on high-resolution CT. Furthermore, patients with radiographic patterns of NSIP+OP or diffuse alveolar damage (DAD) had more risk factors for poor prognosis, with 12-month mortality rates of 45.9% and 100%, respectively.Conclusions RPILD was commonly observed in patients with anti-MDA5+DM. OP was identified as the predominant radiographic pattern, which corresponded to a histopathological pattern of NSIP or NSIP+OP. Notably, patients exhibiting radiographic patterns of NSIP+OP or DAD were shown to have a poor prognosis.All data relevant to the study are included in the article or uploaded as supplementary information.