MDA-5 dermatomyositis complicated by interstitial lung disease and cutaneous ulcers: successful treatment with corticosteroids, mycophenolate mofetil and intravenous immunoglobulin

Abstract

Antimelanoma differentiation-associated gene 5 (MDA-5) dermatomyositis is a subtype of dermatomyositis that is associated with rapidly progressive interstitial lung disease (RP-ILD), as well as with a variety of cutaneous manifestations. Patients with MDA-5 dermatomyositis tend to have a poor prognosis that is often attributed to the high rates of concurrent RP-ILD. Given the severity of disease, early diagnosis and aggressive management is pivotal. We present a case of a 40-year-old woman diagnosed with MDA-5 dermatomyositis who presented with weakness, painful cutaneous ulcerations and interstitial lung disease. She was treated with monthly intravenous Ig (IVIg), weight-based prednisone and mycophenolate mofetil (MMF). After approximately 2 years of treatment, her interstitial lung disease remains stable and she has had significant improvement in weakness and cutaneous ulcerations. Our case provides evidence for early and aggressive treatment of MDA-5 dermatomyositis with a combination of weight-based prednisone, MMF and IVIg.

Keywords: connective tissue disease; drugs: musculoskeletal and joint diseases; interstitial lung disease; musculoskeletal and joint disorders; rheumatology.

Figure 1

Ulceration on patient’s left elbow at time of presentation.

Figure 2

Ulceration on patient’s right elbow at time of presentation.

Figure 3

Images of patient’s high-resolution CT of her thorax.

Figure 4

Healed ulceration on patient’s left elbow at time of writing.

Figure 5

Healed ulceration on patient’s right elbow at time of writing.