PULMONARY CAPILLARY HEMANGIOMATOSIS: A RARE CASE OF STABLE DISEASE WITHOUT TREATMENT

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension caused by extensive proliferation of pulmonary capillaries within parenchymal, vascular, and bronchial tissue. Typically, PCH progresses rapidly with a median time to lung transplantation of two years post diagnosis. To our knowledge, we present the longest case of biopsy proven PCH that has been stable without treatment for seven years. CASE PRESENTATION: A healthy 23-year old non-smoking male presented to the emergency department (ED) with a 2-week history of hemoptysis associated with intermittent left-sided pleuritic chest pain, fatigue, and dyspnea. His physical exam, routine lab work, and chest X-ray were unremarkable. He was discharged home and prescribed clarithromycin for suspected bronchitis. He returned to the ED months later with ongoing symptoms and was referred for urgent outpatient consultation to pulmonology. Bronchoscopy was performed with no blood on bronchoalveolar lavage analysis. Further serum rheumatologic and vasculitis panels were negative. A non-contrast computed tomography (CT) scan of his chest revealed subtle bilateral upper lung predominant centrilobular ground-glass opacities. (Figure 1) With worsening hemoptysis, he returned to the ED six months later. A CT Chest angiogram was performed and showed progressive bilateral airspace opacities. (Figure 2) The patient was referred to Thoracic Surgery for lung biopsy. This demonstrated septal proliferation of capillaries consistent with PCH without evidence of vasculitis, venous occlusion or microorganisms. Immunofluorescence staining was also negative. (Figure 3) The patient remains stable with no signs of progression or pulmonary hypertension on echocardiogram. His enhanced CT and pulmonary function tests remain normal seven years from his initial presentation. DISCUSSION: The typical presentation of PCH includes progressive dyspnea, cough with or without hemoptysis, chest pain, and fatigue. (1) Chest X-ray can be normal or progress to demonstrate bilateral opacities in keeping with diffuse alveolar hemorrhage (DAH). CT chest often shows diffuse centrilobular ground-glass opacities. (1) Bronchoscopy may not reveal erythrocytes if the patient is not actively bleeding. Diagnosis of PCH is essential, as vasodilators used in pulmonary hypertension can cause hemodynamic instability in PCH. (2) Interestingly, an autopsy case series found incidental cases of PCH in patients who were asymptomatic during life. (3) This raises the possibility that subclinical cases of PCH may exist which do not require urgent referral for lung transplantation. CONCLUSIONS: This case represents the longest active case of PCH after confirmation with biopsy without treatment. Re-evaluating the expected prognosis for individuals diagnosed with PCH is important, as median survival may be longer than currently reported in the literature. Reference #1: O'keefe MC, and Post MD. Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension. Archives of Pathology & Laboratory Medicine, 2015;139(2):274–277. Reference #2: Almagro P, Julia J, Sanjuame M, Gonalez G, Casalots J, Heredia JL, et al. Pulmonary Capillary Hemangiomatosis Associated with Primary Pulmonary Hypertension. Medicine. 2002;81(6):417–24. Reference #3: Havlik DM, Massey LW, Williams WL, and Crooks LA. Pulmonary Capillary Hemangiomatosis- like foci: an autopsy study of 8 cases. Anatomic Pathology. 2000;113(5):665-662. DISCLOSURES: No relevant relationships by Meredith Chiasson, source=Web Response No relevant relationships by Jasper Johar, source=Web Response No relevant relationships by Daria Manos, source=Web Response No relevant relationships by Matthew Nunn, source=Web Response