Abstract Idiopathic inflammatory myopathies (IIMs) can initially present with interstitial lung disease (ILD) in a range of 20-80% of cases. IIM-related ILD is associated with high morbidity and mortality, as it is the leading cause of hospitalization and death in patients with inflammatory myositis. A 60-year-old female with a past medical history of migraines and hypertension presented with worsening neck pain and fatigue. She endorsed recent weight loss due to dysphagia and bilateral upper extremity weakness. Laboratory evaluation was significant for a positive antinuclear antibody (ANA) with a titer 1:640 in a speckled pattern, creatinine kinase elevated to 2,886 U/L, aldolase 39.6 U/L, and anti-PM/Scl-11(RD1) antibody. The patient was also found to be hypoxic on admission, requiring nasal cannula. A computed tomography (CT) scan displayed bilateral diffuse opacities, for which the patient was started on ceftriaxone and azithromycin with concern for community-acquired pneumonia. Further history elucidated two prior hospitalizations in the previous year for pneumonia with previous CTs showing nearly identical findings. A high-resolution CT (HRCT) was completed with scattered ground glass opacities within the bilateral upper lung lobes, with areas of reticulation in a perilobular predominant distribution within the lower lobes consistent with a nonspecific interstitial pneumonia (NSIP) pattern. Pulmonary function tests resulted in a moderately restrictive ventilatory defect with absent bronchodilator response and moderately reduced DLCO further confirming an undiagnosed ILD. Infection was excluded. The patient was treated with intravenous glucocorticoids, intravenous immunoglobulin, and azathioprine with resolution of dyspnea and extrapulmonary manifestations and discharged with outpatient pulmonary and rheumatology follow-up. ILD secondary to IIM can be difficult to diagnose or discern from multiple pulmonary pathologies, including infections or other subsets of ILD. Imaging can be non-specific, especially in the absence of extrapulmonary manifestations. Although more serologic studies are available, these tests can delay diagnosis and ultimately treatment, given the need for access to specialized testing and clinical suspicion of an IIM. Prompt diagnosis is important as there are variants of IIM that can rapidly progress if not treated and lead to death. This abstract is funded by: None
Romero et al. (Fri,) studied this question.