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01.12.2015 | Research article | Ausgabe 1/2015 Open Access

BMC Pulmonary Medicine 1/2015

Interstitial lung disease in clinically amyopathic dermatomyositis with and without anti-MDA-5 antibody: to lump or split?

Zeitschrift:
BMC Pulmonary Medicine > Ausgabe 1/2015
Autoren:
Satoshi Ikeda, Machiko Arita, Mitsunori Morita, Satoshi Ikeo, Akihiro Ito, Fumiaki Tokioka, Maki Noyama, Kenta Misaki, Kenji Notohara, Tadashi Ishida
Wichtige Hinweise

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

Ikeda S, Arita M, and Morita M were involved in the acquisition of the data; Ikeda S, Arita M, Morita M, Ikeo S, Ito A, Tokioka F, Noyama M, and Misaki K were involved in the analysis and interpretation of the clinical data; Notohara K were involved in the analysis and interpretation of the pathological findings; Ikeda S and Arita M were involved in the drafting of the manuscript; Ishida T was involved in the study supervision. All authors read and approved the final manuscript.

Abstract

Background

Interstitial lung disease (ILD) associated with clinically amyopathic dermatomyositis (CADM-ILD) is often refractory and rapidly progressive. Although the anti-melanoma differentiation-associated gene 5 (anti-MDA-5) antibody is associated with rapidly progressive ILD (RP-ILD), differences in clinical features and prognosis of anti-MDA-5 antibody-positive and -negative CADM-ILD remain unclear.

Methods

To clarify the differences in the clinical features and prognosis between anti-MDA-5 antibody-positive and -negative cases, we retrospectively reviewed the medical records of patients diagnosed with CADM-ILD with and without anti-MDA-5 antibody at Kurashiki Central Hospital from January 2005 to September 2014.

Results

Anti-MDA-5 antibody was found in 10 of 16 patients (63 %). The levels of Krebs von den Lungen-6 (KL-6) and surfactant protein D (SP-D) at the first visit were significantly lower in positive patients than in negative patients, whereas the levels of aspartate aminotransferase (AST), γ-glutamyl transpeptidase (γ-GTP), and the CD4+/CD8+ ratio in the bronchoalveolar lavage (BAL) fluid were significantly higher in positive patients than negative patients. Subpleural ground-glass opacity (GGO) or irregular linear opacity was predominant in positive patients. Peribronchovascular consolidation was predominant in negative patients. Positive patients had significantly lower survival rates than negative patients, with all six fatal cases occurring in positive patients who died of refractory ILD within 92 days from the first visit despite intensive treatment.

Conclusions

There are clear differences in the clinical features and prognosis of anti-MDA-5 antibody-positive and -negative CADM-ILD. Low serum KL-6 and SP-D levels, high serum AST and γ-GTP levels, high CD4+/CD8+ ratio in BAL fluid, and predominance of subpleural GGO or irregular linear opacity in HRCT may help to discriminate anti-MDA-5 antibody-positive CADM-ILD with poor prognosis.
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