D2.57 - Recurrent pleural effusion in patient with hereditary angioedema: a case report

Background:

Hereditary angioedema (HAE) is a rare genetic disease that manifests with episodes of cutaneous or submucosal edema. HAE attacks are usually characterized by skin swellings, abdominal attacks, and life-threatening upper airway obstruction. In rare cases angioedema can be localized in the bladder, muscles, joints and kidneys. There are only a handful of reported cases of pleural effusion in patients with HAE, with possible correlation to a previous episode of angioedema. The following case presents a HAE diagnosed patient with recurrent pleural effusions without any identifiable cause.

Case Report:

A 66-year-old male diagnosed with HAE-C1INH-Type 1 at the age of 21. He reported 8 total attacks in the past 2 years, affecting the genitals, gluteus and extremities, and a single abdominal attack. The patient was referred for a routine chest X-ray, which revealed a right-sided pleural effusion. The patient was completely asymptomatic with no significant laboratory changes. Subsequent CT scan confirmed the X-ray findings, and the patient was referred to cardiology, pulmonology and cardiothoracic surgery for evaluation – a diagnostic video-assisted thoracoscopic surgery (VATS) was scheduled. During the procedure a 1600 mL effusion was evacuated, with following adhesiolysis, and parietal pleural and pulmonary biopsies were performed. Talc pleurodesis was implemented and a surgical drain was placed. Histological analysis revealed pleural chronic fibrotic, inflammatory changes, lung tissue with emphysematous changes, atelectatic and peribronchial chronic inflammatory changes. Chest X-ray was performed 3 weeks post procedure, with fibrotic changes and pleural adhesions located in the area of the previously observed effusion. A control CT scan after 4 months, revealing a reduction in volume pleural effusion (compared to the initial CT scan). Regular cardiology, pulmonology and cardiothoracic surgery control checkups are ongoing.

Discussion:

Since now, a couple of cases have been reported of patients with HAE, having pleural effusion with possible link to a previous HAE attack of the surrounding area. Without evidence for probable cause of the findings in the presented case, there could be a causative relationship between unidentified HAE attacks and the aforementioned findings.