HIba Hadid, MD
Assistant Professor of Medicine
Detroit VA Medical Center, United States
Disclosure information not submitted.
Amina Pervaiz
Detroit Medical Center- Wayne State University
Detroit, MI
Disclosure information not submitted.
Title: Raising awareness about myositis as a cause of hypoxic respiratory failure
Introduction: Myositis is a rare, underdiagnosed disease that presents in variable ways. Presentation is usually vague and non-specific. Physicians often attribute symptoms to deconditioning given the lack of objective findings at the first glance.
Description: A 56-year-old African American woman who was diagnosed late with antisynthetase syndrome after progressing to hypoxic respiratory failure. The patient initially presented to the hospital complaining of progressive symptoms of shortness of breath and fatigue. She is a life-long non-smoker and worked as a cook for 30 years with no significant occupational or environmental exposures. Initial vital signs were normal except for low oxygen saturation of 86% requiring 2 L oxygen. Physical exam was positive for trace lower extremity edema with no crackles or wheezing. Initial lab work was normal with negative COVID 19 test. Her CT scan revealed diffuse mild ground glass opacities. Echocardiogram showed grade I diastolic dysfunction. She was treated as heart failure and was started on diuretics without significant improvement. She was discharged on 2 L oxygen and diuretics. Her admissions recurred over the course of few months with progressive worsening of her imaging with diffuse GGO and increasing oxygen requirements for up to 5 L on exertion. Her symptoms of dyspnea progressed to the point where the patient was wheelchair bound and only able to walk a few feet. She was re admitted to the hospital and the pulmonary service was consulted. The patient was suspected of having a form of interstitial lung disease. Workup revealed normal ANA, RF, CCP, and ANCA. Her repeat echocardiogram revealed normal pulmonary artery pressures and normal ejection fraction. Her myositis panel showed positive PL1 antibodies and high CPK levels. A muscle biopsy was performed confirming necrotizing myopathy. The patient was diagnosed with antisynthetase syndrome. She was started on steroids then transitioned to Imuran with improvement in her symptoms. She is currently in pulmonary rehab and progressing as expected.
Discussion: This case helps highlight the importance of suspecting myositis as a cause of hypoxic respiratory failure that is otherwise unexplained. Undiagnosed, myositis may progress quickly and can involve the lungs.