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• W4387248957 abstract "SESSION TITLE: Unusual Manifestations of Unusual Diseases SESSION TYPE: Case Reports PRESENTED ON: 10/09/2023 01:00 pm - 02:00 pm INTRODUCTION: Diffuse cystic lung disease (DCLDs) comprise a diverse group of pulmonary disorders. Birt-Hogg-Dubé syndrome (BHD), a rare and complex genetic disorder characterized by development of benign skin lesions, pulmonary cysts, and renal neoplasia is one of the most commonly encountered DCLDs in practice. While typically asymptomatic, complications associated with BHD include recurrent pneumothoraces. To our knowledge, there are limited reports of pulmonary arteriovenous malformations (PAVMs) associated with BHD. CASE PRESENTATION: A 48-year-old female with a history of prior tobacco use disorder was admitted to the hospital with acute hypoxic respiratory failure in the setting of a recent viral illness. On presentation, she was dyspneic and hypoxemic, which improved with supplemental oxygen. She underwent CTA of the chest to exclude pulmonary embolism, which was notable for bilateral, large, and thin-walled irregular lung cysts of variable size. These cysts were more basilar in distribution, mostly para-mediastinal, abutting the pericardium, and around the major fissures. These cysts were associated with PAVMs bilaterally. The rest of the pulmonary parenchyma appeared normal without ground glass changes (Figure 1,2). Besides her 10-pack-year smoking history, she denied any history of pulmonary, autoimmune/rheumatologic, or malignant disease. She denied any significant exposure to dust or chemicals, and/or familial history of lung cysts, renal cancer, pneumothorax, or sudden death. Further diagnostic work-up was significant for positive ANA titer of >1:640 with positive Anti-SSA titer >8.0 and RF >1024. CT of the abdomen/pelvis was negative for renal masses/lesions. Differential diagnosis included BHD and Lymphocytic Interstitial Pneumonitis associated with Sjogren's syndrome (SS). Our patient did not have symptoms/signs to suggest connective tissue disease or SS. Ultrasound of the salivary glands did not show any evidence of SS. The clinical presentation, radiologic features, and diagnostic work-up favored BHD as the unifying diagnosis. There was a growing concern for untreated PAVMs as the culprit of her respiratory instability and potentially life-threatening complications. CT of the head did not show any AVMs, and a transthoracic echocardiogram was unremarkable (though a bubble shunt study was not performed). She underwent a pulmonary angiogram with embolization of the largest PAVMs. She was eventually discharged home with close follow-up in the pulmonary clinic. A follow-up CTA of the chest 1-month later showed a significant decrease in the size of the AVMs and stable appearance of additional smaller AVMs and pulmonary cysts (Figure 3). DISCUSSION: PAVMs can be congenital or acquired. Most genetic cases are associated with Hereditary Hemorrhagic Telangiectasia. Acquired PAVMs are less common and typically observed in liver cirrhosis or after congenital heart disease surgery (1-3). There are limited reports on the association between PAVMs and DCLD. We present a rare case of multiple PAVMs associated with DCLD in a patient with a probable unifying diagnosis of BHD. CONCLUSIONS: Further studies are needed to identify a clear association, pathophysiology, and clinical course of PAVMs in patients with DCLD, particularly BHD. Life-threatening complications such as massive hemoptysis, hemothorax, stroke or brain abscesses have been reported in 2% of patients with PAVMs, potentially leading to considerable morbidity and mortality if left untreated (1-3). REFERENCE #1: Daccord C, Good J, Morren M, Bonny O, Hohl D, Lazor R. Birt-Hogg-Dubé syndrome. Eur Respir Rev 2020 Sep 17;29(157):200042. doi: 10.1183/16000617.0042-2020. Print 2020 Sep 30. REFERENCE #2: Matsutani N, Dejima H, Takahashi Y, Uehara H, Iinuma H, Tanaka F, et al. Birt-Hogg-Dube syndrome accompanied by pulmonary arteriovenous malformation. J Thorac Dis 2016 Oct;8(10):E1187-E1189. REFERENCE #3: Shin YM, Kim Y, Yang J, Yang B, Choi IA, Lee KM. Recurrent pulmonary arteriovenous malformation in a patient with Sjögren syndrome: A case report. Medicine (Baltimore) 2022 Oct 14;101(41):e30954. DISCLOSURES: No relevant relationships by Fernando Camacho No relevant relationships by Bishal Gyawali No relevant relationships by Joseph Modrak" @default.
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• W4387248957 date "2023-10-01" @default.
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• W4387248957 title "MULTIPLE PULMONARY ARTERIOVENOUS MALFORMATIONS IN A PATIENT WITH DIFFUSE CYSTIC LUNG DISEASE: A CASE REPORT" @default.
• W4387248957 doi "https://doi.org/10.1016/j.chest.2023.07.2168" @default.
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