A severe anti MDA-5 antibodies associated amyopathic dermatomyositis

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Mounira El Euch
Wifak Bani
Madiha Mahfoudhi
Fatima Jaziri
Khaoula Ben Abdelghani
Sami Turki
Taïeb Ben Abdallah

Abstract


Background: Lung damage during amyopathic dermatomyositis (ADM) associated with auto antibodies anti MDA-5 is serious. We report a rare observation of a severe ADM associated to anti MDA-5 antibodies complicated with diffuse interstitial lung disease (ILD) rapidly extensive and fatal.
Observation: We report the observation of a Tunisian adult who was admitted for ADM. The anti MDA-5 antibodies were detected. He developed an ILD rapidly extensive to
fibrosis. Corticosteroids were unefficient and he deceded of pneumomediastin and refractory respiratory distress.

Conclusion: This observation illustrates the severity of ADM with anti MDA-5 antibodies. Every clinician should detect any ILD to avoid the progression to fibrosis.

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References

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  2. Sato S, Hirakata M, Kuwana M et al. Autoantibodies to a 140- kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis and Rheumatism 2005;52:1571-6.
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