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Review
. 2018 Jul;38(7):1293-1296.
doi: 10.1007/s00296-018-3991-7. Epub 2018 Feb 7.

Rapidly progressive interstitial lung disease due to anti-MDA5 antibodies without skin involvement: a case report and literature review

Juan González-Moreno  1 , Manuel Raya-Cruz  2 , Ines Losada-Lopez  2 , Ana Paula Cacheda  3 , Cristina Oliver  4 , Bartomeu Colom  5
Affiliations
Review

Rapidly progressive interstitial lung disease due to anti-MDA5 antibodies without skin involvement: a case report and literature review

Juan González-Moreno et al. Rheumatol Int. 2018 Jul.

Abstract

Anti-MDA5 antibodies have been strongly associated with rapidly progressive interstitial lung disease (RP-ILD) in dermatomyositis (DM) patients, especially in the clinically amyopathic subset (CADM). We present a case of anti-MDA5 antibody-associated RP-ILD in a patient with arthritis but with no other clinical signs suggestive of DM or CADM successfully treated with a combination of cyclophosphamide, cyclosporine and corticoids. A review of the literature was also done. Despite its rarity, anti-MDA5 antibody-associated ILD should be suspected in cases of RP-ILD even without other signs of DM or CADM as prompt and aggressive treatment could improve prognosis.

Keywords: Anti-CADM-140 antibodies; Anti-MDA5 antibodies; Clinically amyopathic dermatomyositis; Dermatomyositis; Diffuse alveolar damage; Rapidly progressive interstitial lung disease.

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Figures

Fig. 1
Fig. 1
Thoracic CT showing peripheral ground-glass opacities predominantly in the lung bases (arrows)
Fig. 2
Fig. 2
Transbronchial biopsy. Periodic acid–Schiff (PAS) staining. ×200. Lung parenchyma with destructuring, inflammatory infiltrate and presence of hyaline membrane remnants (arrows)
See this image and copyright information in PMC

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