PULMONARY ARTERIAL HYPERTENSION AS A MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOUS

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Systemic Lupus Erythematosus (SLE) has long been associated with pulmonary manifestations such as pleural effusion, pulmonary hemorrhage, and pleuritis. Rarely, pulmonary arterial hypertension (PAH) develops as a consequence of SLE, usually presenting on average 3.2 years after diagnosis. We present a case of PAH as the initial manifestation of a patient who would later be diagnosed with SLE. CASE PRESENTATION: A 59 year old female with no medical history presented to the pulmonary clinic with dyspnea for two years. She stated that her dyspnea is present at rest but worsened with exertion. She had no other symptoms aside from generalized fatigue and denied cough, sputum production, chest pain, skin lesions or sores. Her symptoms had progressively worsened and previous treatment with prednisone 40mg po daily for a presumptive diagnosis of sarcoidosis had not led to any benefit. Previous biopsies for pulmonary nodules of undetermined significance did not render a diagnosis due to lack of available tissue. Normal spirometry and lung volumes were noted on pulmonary function testing. 6 minute walk test showed an SpO2 of 94% at the start, and 89% at completion and heart rate increased from 113 to 117 beats per minute. High resolution Chest CT was performed that showed no change in pulmonary nodules from previous. There was evidence of pulmonary artery enlargement. A right and left heart catheterization was performed revealing no coronary artery disease, but mean pulmonary artery pressure was elevated at 52mmHg, pulmonary capillary wedge pressure was 4mmHg and pulmonary vascular resistance was 15mmHg. She had adequate reactivity to adenosine. Rheumatologic evaluation later revealed positive ANA with a titer of 1:640, RF IgM, anti-cardiolipin IgM and anti-phospholipid IgM. Complements were decreased. The patient underwent a surgical wedge resection, and pathology revealed bronchial-associated lymphoid tissue. She was diagnosed with SLE as well as group 1 PAH, and initiated on hydroxychloroquine, ambrisentan and nifedipine with subjective and objective improvement in her clinical course. DISCUSSION: Pre-capillary PAH is the form closely linked with SLE-associated histopathological changes affecting small lung vessels. PAH is diagnosed by right heart catheterization revealing a mean pulmonary artery pressure >25mmHg, and can be defined as pre-capillary if the pulmonary capillary wedge pressure is <15mmHg. Bronchus-associated lymphoid tissues have been found in animal studies to be auto-immunologically active, and contribute to pulmonary vascular remodeling and pulmonary hypertension. CONCLUSIONS: This case highlights the close link between autoimmune conditions, small-vessel pulmonary vascular changes related to lymphoid aggregation and pulmonary hypertension. Targeted treatment for both group 1 PAH and SLE should be considered early in such patients, and initiated without delay. Reference #1: Colvin, Kelly L, et al. “Bronchus-Associated Lymphoid Tissue in Pulmonary Hypertension Produces Pathologic Autoantibodies.” American Journal of Respiratory and Critical Care Medicine, vol. 188, 2013, pp. 1126–1136. Reference #2: Perros, Frederic, et al. “Pulmonary Lymphoid Neogenesis in Idiopathic Pulmonary Arterial Hypertension.” American Journal of Respiratory and Critical Care Medicine, vol. 185, 2012, pp. 311-321. Reference #3: Badlam, Jessica B, et al. “Steps forward in the treatment of pulmonary arterial hypertension: latest developments and clinical opportunities.” Therapeutic Advances in Chronic Disease, 8(2-3), pp. 47-64. DISCLOSURES: No relevant relationships by Alexandra Gastesi, source=Web Response No relevant relationships by Julia Ladna, source=Web Response No relevant relationships by Glenn Singer, source=Admin input No relevant relationships by Kenneth Wojnowski, source=Web Response