Segmental endobronchial valve therapy for a vasculitis-induced emphysema

A 53-year old female patient with history of hypocomplementaemic urticarial vasculitis syndrome (HUVS) and polyarteritis nodosa presented with progressive dyspnoea on exertion due to emphysema. Lung function revealed a severe obstructive ventilator disorder with a forced expiratory volume in 1 second of 22% of predicted, and a significant hyperinflation with a residual volume of 321% of predicted. Multi-detector computed tomography (MDCT) scan and quantitative CT analysis (StratX software) confirmed a lower lobe predominant emphysema. Considering the young age, the very severely impaired lung function, the relatively low nicotine abuse, the exclusion of alpha-1 antitrypsin deficiency, together with the known diagnosis of HUVS, the emphysema was more likely due to the vasculitis than to a typical chronic obstructive lung disease. MDCT scan showed that particularly the segment 8 of the right lower lobe was severely emphysematous destroyed and hyperinflated. Invasive Chartis® measurement revealed no significant collateral ventilation of the isolated segment 8 of the right lower lobe, so that an endobronchial valve placement was performed. Three months following intervention, the MDCT scan revealed a complete collapse of the segment 8 on the right, which was associated with a significant clinical benefit and a mild reduction of the hyperinflation in the lung function test.