SHM Converge Scientific Abstract ePoster Gallery-Unusual Ulcerations: A Clue to Diagnosis of Unusual Anti-Mda-5 Dermatomyositis

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This case describes a 70-year-old Caucasian woman with progressive exertional dyspnea, fatigue, malaise, anorexia, mild proximal muscle weakness, nonproductive cough, and lip ulcerations. She had been repeatedly treated for presumed community-acquired pneumonia without improvement and was already taking prednisone for a prior rash that had been biopsied as DRESS syndrome from valsartan.<br /><br />On presentation, she was tachycardic, hypoxemic with exertion, and had scattered wheezes/rhonchi plus oral/lip ulcerations and 4/5 muscle strength in all extremities. Imaging showed bilateral interstitial and ground-glass lung opacities without pulmonary embolism. Infectious workup, including respiratory viral testing and cultures, was negative. Echocardiography was normal. Bronchoscopy revealed three bronchial submucosal ulcers, an unusual finding, and biopsy showed squamous dysplasia without malignancy. BAL studies were largely negative.<br /><br />Inflammatory and rheumatologic testing showed elevated CRP, mildly elevated ESR and aldolase, low complement levels, anti-SSA positivity, and ultimately a markedly elevated anti-MDA-5 antibody on myomarker panel. CK was normal. Repeat high-resolution CT demonstrated nonspecific interstitial pneumonia (NSIP), supporting anti-MDA-5 dermatomyositis with interstitial lung disease.<br /><br />She initially received antibiotics, inhalers, rate control, and anticoagulation, but persistent dyspnea led to discontinuation of antibiotics and treatment with IV furosemide and high-dose methylprednisolone. She transiently required high-flow oxygen, improved to 4 L/min, and was discharged on prednisone. Because of presumed anti-MDA-5 myositis, she later received rituximab, pulse steroids, and tacrolimus.<br /><br />The key teaching point is that unusual mucosal or bronchial ulcerations can be a clue to anti-MDA-5 dermatomyositis, especially in patients with progressive dyspnea and interstitial lung disease unresponsive to antibiotics. Early steroid use may mask classic rash and delay diagnosis.

Asset Subtitle

Cristine K. Arcilla

Meta Tag

Author List Cristine K. Arcilla, Daniel Goldsmith, Ruth Ramon Tapia, William Torelli

Category Clinical Vignettes

Concept Anti-MDA5 Dermatomyositis

Concept Anti-MDA5 Antibody

Concept Dyspnea

Concept Interstitial Lung Disease

Concept Mucosal Ulcer

Distinguished Non-Finalist

Presenter Organization Capital Health Regional Medical Center

Presenting Author Cristine K. Arcilla

Track Adult

Keywords

anti-MDA-5 dermatomyositis

interstitial lung disease

bronchial submucosal ulcers

dyspnea

ground-glass opacities

nonspecific interstitial pneumonia

oral ulcerations

prednisone

rituximab

tacrolimus

Anti-MDA5 Dermatomyositis

Anti-MDA5 Antibody

Dyspnea

Interstitial Lung Disease

Mucosal Ulcer