C45-19 A Severe Case of Yellow Nail Syndrome With Cardiac Manifestations

Abstract Introduction Yellow Nail Syndrome (YNS) is a rare multisystem disorder characterized by a clinical triad of yellow dystrophic nails, lymphedema, and respiratory manifestations. We present a case of complicated YNS with cardiac involvement—an association previously reported only 13 times in the literature. Case Report A 42-year-old male with a history of YNS with bilateral pleural effusions requiring repeated thoracentesis, now status-post bilateral pleurodesis, recurrent pericardial effusions status-post pericardial window ×2, yellow dystrophic nails, and lymphedema, presented with shortness of breath and fever. On evaluation, pneumonia was diagnosed alongside recurrent bilateral pleural effusions, more pronounced on the right. Due to prior bilateral pleurodesis, thoracentesis was deferred, and the patient recovered with intravenous antibiotics. Currently, he remains stable with chronic pleural effusions, managed in the outpatient setting with daily furosemide for symptomatic relief. Of note, during the hospital course the patient mentioned that he has numerous family members who also suffer from lymphedema and effusions, being worked up in various hospitals. Discussion The diagnosis of Yellow Nail Syndrome requires the presence of at least two of its three cardinal features. Lymphedema, typically involving gravity-dependent limbs, is often the initial manifestation. Pulmonary involvement commonly presents as chronic cough due to recurrent pleural effusions, which are typically bilateral, exudative, and lymphocyte-predominant. Bronchiectasis is also a frequent finding. Nail changes, characteristically yellow and dystrophic, are highly suggestive but may be absent at disease onset. The complete triad is observed less frequently at initial presentation. The true prevalence of YNS currently, remains undetermined given the limited number of reported cases. There is now increasing suggestion of a possible cardiac manifestation of this syndrome, with few cases of recurrent pericardial effusions reported in the literature. Most cases are sporadic; however, associations with titanium implants and environmental exposures have been reported. A more recent study in genes involved with cell polarity demonstrated a possible familial etiology. The underlying pathogenesis is believed to involve lymphatic dysfunction leading to impaired drainage and fluid accumulation. Management remains primarily symptomatic with no standardized treatments. Surgical interventions such as pleurodesis and decortication have demonstrated up to 81% effectiveness in controlling recurrent pleural effusions. The clinical course of YNS is variable, but morbidity can be significant due to recurrent respiratory complications and chronic lymphedema. This severe presentation of YNS contributes to the growing understanding of the syndrome by highlighting its potential cardiac involvement and points toward a genetic etiology in this rarely reported condition. This abstract is funded by: none