A CHALLENGING CASE OF DIFFUSE ALVEOLAR HEMORRHAGE

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SESSION TITLE: Fellows Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is commonly seen in the intensive care unit and has a variety of causes including vasculitis. Most commonly, a vasculitis is linked to either antineutrophil cytoplasmic antibodies (ANCA) or an anti-glomerular basement membrane antibody. In rare cases, it manifests in a pauci-immune state. CASE PRESENTATION: 52-year-old male with a past medical history of biopsy confirmed IgA nephropathy, who presented with fatigue and hemoptysis. The patient was noted to be from Cambodia and recently visited his home country. During his hospitalization, patient was noted be hypoxic requiring oxygen supplementation. A CT chest was obtained revealing a left upper lung mass concerning for infection verses malignancy (Figure 1). CT guided biopsy by IR was performed, however, while waiting for the studies to return, patient decompensated and required ICU level of care secondary to massive hemoptysis. He was intubated and bronchoscopy revealed serial aliquots consistent with DAH (Figure 2). Transthoracic echocardiogram revealed severe mitral stenosis secondary to calcifications. Cardiology consultation recommended mitral valve replacement however, patient was in critical condition at this time. The patient’s CT guided left upper lobe biopsy returned positive for alveolar hemorrhage. There was no bacterial or viral infection noted. Additionally, vasculitis workup including ANA, ANCA, MPO, PR3, hepatitis panel, and HIV were all negative. Thoracic surgery was consulted and VATS of the left upper and right upper and lower lobe biopsy was performed. During his hospital course, the patient developed progressive AKI requiring hemodialysis. Open lung biopsies returned positive for focal active capillaritis. There was no specific staining noted for IgA, IgM, or IgG. The patient was started on pulse dose steroids and received seven days of plasma exchange therapy. His clinical status improved and he was discharged from the ICU. DISCUSSION: DAH can be caused from a variety of diseases. Interestingly, our patient had a history of IgA nephropathy and severe mitral stenosis, both of which can cause DAH, however, at the end of his workup and hospital course his diagnosis was pauci-immune vasculitis. This is a rare systemic disease that can cause pulmonary renal syndrome. Pulmonary renal syndrome is most commonly associated with ANCA associated vasculitis, however, in 10% this may be absent. Diagnosis is confirmed by open lung biopsy and immunosuppression is the mainstay of treatment. Patients with a negative ANCA were shown to have a worse renal outcome compared with those that had a positive ANCA, but similar survival rates. CONCLUSIONS: A careful evaluation of DAH must be performed even when the etiology is obvious. The gold standard for a definitive diagnosis is through open lung biopsy and should be performed if the patient can tolerate it. Reference #1: Saladi L, Shaikh D, Saad M, et al. Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis. Medicine (Baltimore). 2018;97(23):e10954. doi:10.1097/MD.0000000000010954 DISCLOSURES: No relevant relationships by Alireza Nathani, source=Web Response No relevant relationships by Sean OConnor, source=Web Response No relevant relationships by Heath White, source=Web Response