Pulmonary Hypertension Associated with Diffuse Panbronchiolitis That Improved with Erythromycin and Home Oxygen Therapy

Pulmonary hypertension (PH) often complicates chronic lung disease. However, there are few reports of PH associated with diffuse panbronchiolitis, and there is no effective treatment. We herein report a 64-year-old woman diagnosed with PH due to diffuse panbronchiolitis. She received erythromycin, carbocysteine, and home oxygen therapy (1 L O(2)/min). After 4 months of therapy, the respiratory function (diffusing capacity of the lungs for carbon monoxide: 23.3% to 76.1%) and PH (mean pulmonary arterial pressure: 50 to 28 mmHg; pulmonary vascular resistance: 680 to 518 dynes・sec・cm(-5); pre- vs post-therapy, respectively) had improved markedly.