Correct!
3. Bronchiolitis obliterans
The patient presented to the emergency room with hypoxic respiratory failure 5 months after her surgery, and tested positive for influenza A. She eventually recovered, and was started on additional medications for her obstructive airway disease, including azithromycin, rosuvastatin, montelukast, Spiriva, a bronchodilator, and steroid inhalers, for which she was intermittently non-compliant. Attempts to taper her corticosteroid therapy resulted in worsening respiratory symptoms as well as return of the oral and genital ulcers. She has remained in remission from her Castleman disease for over 5 years and, while her respiratory function declined rapidly over the ensuing months following her surgery (just over one year after surgery, FVC = 2.06L (57% predicted), forced expiratory volume in the first second of exhalation (FEV1) = 0.63L (21% predicted), and forced expiratory flow rate25-75% = 0.26 L/sec (8% predicted), her pulmonary function has remained relatively stable since. Chest CT performed just over 4 years after initial diagnosis (Figure 11) showed more well-developed features of bronchiolitis obliterans. The patient is alive more than 5 years after her initial diagnosis and has been evaluated for potential lung transplantation.
Figure 11. Axial unenhanced chest inspiratory CT showed widespread but only mildly thick-walled bronchial dilation without intraluminal fluid (a pattern sometimes referred to as “dry bronchiectasis”) with significant bilateral, multifocal inhomogeneous lung attenuation. These findings are indicative of significant obstructive pulmonary disease, and the comparatively thin-walled nature of the bronchial dilation is typical of severe bronchiolitis obliterans.
Diagnosis: Bronchiolitis obliterans associated with paraneoplastic pemphigus and Castleman disease
References