Recurrent spontaneous pneumothorax in a patient Wilson disease who is on long term D-penicillamine. Case report

Abstract

Abstract Background Recurrent spontaneous pneumothoraces (PSP) in association with liver disease is well known to occur with alpha 1 antitrypsin deficiency (AATD). Wilson disease (WD) association to recurrent PSP is not a known entity. Case presentation A 42-year-old nonsmoker man with a history of recurrent PSP admitted with large right pneumothorax. Following intercostal chest drain (ICD) patient’s breathing improved and the lung expanded. Despite that, he continued to have a small residual pneumothorax (< 2cm). Twelve years ago, he was diagnosed to have WD. Patient was on treatment with D-penicillamine. After 3 years to the diagnosis of WD, patient had developed left sided recurrent PSPs where he ended up with left-pleurectomy in 2015. For the current event, initially expectant management was done for residual pneumothorax. All the possible causes for PSP were excluded. On follow up, he continued to be symptomatic. Thoracic surgical referral was arranged for Video-Assisted Thoracoscopic Surgery (VATS) and redo-pleurectomy. Safety of future treatment with D-penicillamine is not concluded as there is no convincing evidence to prove it as a culprit agent for PSPs. Multidisciplinary discussion arranged and consideration of treatment with alternative copper chelating therapy was emphasized. Conclusions Though rare, it is important to observe for occurrence of PSP in WD patients. This case report will be an eye opening for association of lung disease and WD and related treatment.