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Recurrent spontaneous pneumothorax in a patient with wilson disease who is on long-term D-penicillamine: Case report

Journal of Lung, Pulmonary & Respiratory Research
Gunathilaka MDN,1 Bandara LMH,1 Handagala S,2 Kularatne WNS1


Introduction: Recurrent spontaneous pneumothoraces (PSP) in association with liver disease is well known to occur with alpha 1 antitrypsin deficiency (AATD). Wilson disease (WD) association with recurrent PSP is not a known entity. Case presentation: A 42-year-old nonsmoker man with a history of recurrent PSP was admitted with a 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 with WD. The patient was on treatment with D-penicillamine. After 3 years of the diagnosis of WD, the patient developed left-sided recurrent PSPs where he ended up with a 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. A thoracic surgical referral was arranged for Video-Assisted Thoracoscopic Surgery (VATS) and redo-pleurectomy. The 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 was arranged and consideration of treatment with alternative copper chelating therapy was emphasized. Conclusion: Though rare, it is important to observe for occurrence of PSP in WD patients. This case report will be eye-opening for the association of lung disease and WD and related treatment. 


D-penicillamine, recurrent pneumothorax, wilson disease, case report