Pulmonary Vascular Disease: Pulmonary Vascular Disease |

Undiagnosed and Unsuspected Nephrotic Syndrome in a Young Adult Presenting as Submassive Pulmonary Embolism FREE TO VIEW

Arjun Khanna, DM
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All India Institute of Medical Sciences, Delhi, India

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A506. doi:10.1016/j.chest.2016.02.528
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SESSION TITLE: Pulmonary Vascular Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: We describe here, the case of a previously healthy young male who presented with unprovoked extensive pulmonary embolism. At the time of presentation, he had no clinical features suggestive of nephrotic syndrome (NS). However, during the work up of pulmonary embolism (PE) the patient developed anasarca which prompted the work up for various etiologies, including NS.

CASE PRESENTATION: A 36 year old male, previously healthy, presented with 3 months history of shortness of breath. CT scan of the chest revealed bilateral pulmonary embolism with left sided pulmonary infarct. Doppler scan of both lower limbs was not suggestive of any deep venous thrombosis. The patient was incompletely investigated for thrombophila in the form of protein C level (172%), protein S level (130%) and factor V leiden mutation analysis, which were all normal. The patient presented to our centre with worsening dyspnea and, for further evaluation. An emergency 2D Echo revealed adequate systolic function of the left and right ventricles and an echogenic mass, suggestive of embolus was visualized in the main pulmonary artery. During the course of the hospital stay, the patient started developing anasarca. A repeat CT pulmonary angiogram (CTPA) was done which revealed extensive bilateral pulmonary emboli with extension of the thrombus in secondary and tertiary branches of the right lobe, right renal vein and the inferior vena cava. Repeat blood biochemistry revealed, low total serum protein levels (3.80g/dl), decreased total serum albumin (1.10g/dl). 24 hour urinary protein examination showed urinary protein loss of 15 gm/day establishing the diagnosis of nephrotic syndrome. Right sided kidney biopsy and was suggestive of membranous glomerulopathy. He was started on tablet dabigatran, tacrolimus, prednisolone and atorvastatin

DISCUSSION: Patients with nephrotic syndrome are at an increased risk for thrombotic events such as deep venous thrombosis, renal vein thrombosis, and pulmonary embolism1. Enhanced platelet aggregation, and renal losses of anticoagulant proteins such as antithrombin III (ATIII) are thought to underlie the excessive thrombotic risk in patients with nephrotic syndrome. Patients with NS also have an increased risk of renal vein thrombosis and, in some patients with NS and PE, the origin of pulmonary embolus is from the renal veins as was the case in our patient 2.

CONCLUSIONS: Our case is unique, as the patient had PE as the primary presenting feature and did not have any evidence of NS prior to this episode.

Reference #1: J. S. Cameron and J. Hicks, “The origins and development of the concept of a ‘nephrotic syndrome’,” American Journal of Nephrology, vol. 22, no. 2-3, pp. 240-247, 2002.

Reference #2: S. Lionaki, V. K. Derebail, S. L. Hogan et al., “Venous thromboembolism in patients with membranous nephropathy,” Clinical Journal of the American Society of Nephrology, vol. 7, no. 1, pp. 43-51, 2012.

DISCLOSURE: The following authors have nothing to disclose: Arjun Khanna

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