Clinical features of patients with anti-melanoma differentiation-associated gene-5 antibody-positive dermatomyositis complicated by spontaneous pneumomediastinum

• Published in: Clinical rheumatology Vol. 38; no. 12; pp. 3443 - 3450
• Main Authors: Yamaguchi, Koichi, Yamaguchi, Aya, Itai, Miki, Kashiwagi, Chiharu, Takehara, Kazutaka, Aoki, Shuhei, Sawada, Yuri, Taguchi, Kohei, Umetsu, Kazue, Oshima, Kazuma, Uchida, Megumi, Takemura, Masao, Hara, Kenichiro, Motegi, Sei-ichiro, Muro, Yoshinao, Nakasatomi, Masao, Sakairi, Toru, Hiromura, Keiju, Kurabayashi, Masahiko, Maeno, Toshitaka
• Format: Journal Article
• Language: English
• Published: London Springer London 01-12-2019; Springer Nature B.V
• Subjects: Adult; Aged; Autoantibodies; Dermatomyositis; Dermatomyositis - complications; Dermatomyositis - diagnosis; Dermatomyositis - immunology; Emphysema; Female; Fibrosis; Humans; Immunosuppression; Immunosuppressive agents; Interferon-Induced Helicase, IFIH1 - immunology; Lung diseases; Male; Mediastinal Emphysema - etiology; Medicine; Medicine & Public Health; Melanoma; Middle Aged; Original Article; Patients; Prognosis; Respiratory failure; Retrospective Studies; Rheumatology; Pneumomediastinum; Anti-melanoma differentiation-associated gene 5 antibodies; Rapidly progressive interstitial lung disease; Dermatomyositis
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-019-04729-5

Background
Dermatomyositis (DM) with autoantibody against melanoma differentiation-associated gene-5 (MDA5) is characterized by elevated risk of rapidly progressive interstitial lung disease (RP-ILD) with a potentially fatal course. Pneumomediastinum (PNM) is a common pulmonary manifestation which accompanies ILD. However, the clinical features of the patients with anti-MDA5 antibody-positive DM who develop PNM remain unclear.
Methods
We retrospectively examined 31 patients with DM having anti-MDA5 antibody and compared the clinical features between patients with PNM (PMN(+)) (
n
 = 11) and those without (PNM(−) (
n
 = 20). In addition, we evaluated the treatment-related prognoses in PNM(+) group.
Results
CT score (total ground-glass opacity (GGO) score,
P
 = 0.02; total fibrosis score,
P
 = 0.02) before treatment, and mortality (
P
 = 0.04) were significantly higher in PNM(+) group. The cumulative survival rate as assessed by Kaplan–Meier method was significantly lower for the PNM(+) group (
P
 = 0.02). Among 11 PMN(+) patients, 9 patients (9/11, 81.8%) underwent intensive immunosuppression therapy for RP-ILD, and 5 patients (5/11, 45.5%) did not respond to it and died from the respiratory failure. At the time of diagnosis of PNM, nonsurvivors had worse liver function (ALT,
P
 = 0.03; LDH,
P
 = 0.01), worse respiratory status (A-aDO2,
P
 = 0.01), and worse CT score (total GGO score,
P
 < 0.01).
Conclusions
A subgroup of patients with DM having anti-MDA5 antibody complicated by PNM as well as RP-ILD did respond to intensive immunosuppression therapy. Initial aggressive immunosuppressive therapy should be considered for these patients.
Key Points
•
This study clearly demonstrate the presence of PNM was associated with elevated risk of death due to respiratory failure from RP-ILD among patients with DM having circulating anti-MDA5-antibody.
•
This study demonstrate evaluation of CT image may be helpful to find patients with better response to the intense immunosuppression therapy for the patients with DM having circulating anti-MDA5-antibody and PNM.