Alveolar Hemorrhage Secondary to Sarcoidosis-Associated Pulmonary Venoocclusive Disease (PVOD).

SESSION TYPE: ILD Cases IIPRESENTED ON: Tuesday, October 23, 2012 at 11:15 AM - 12:30 PMINTRODUCTION: Hemoptysis is uncommon in patients with sarcoidosis. Recent literature suggests an association between PVOD like changes and sarcoidosis in patients with recurrent hemoptysis.CASE PRESENTATION: A 50-year-old female presented with a 4-year history of recurrent hemoptysis and dyspnea on exertion. Her medical history included iron-deficiency anemia of unknown etiology, an indeterminate interstitial lung disease, and recurrent painless rash in the lower extremities. Skin biopsies suggested contact dermatitis versus a hypersensitivity reaction. The social history was notable for 0.5 pack/day smoking, and exposure to a horse ranch including hay and feeds. Her cardiopulmonary exam was unremarkable. Pulmonary function testing showed normal spirometry and lung volumes, but an isolated reduction in the diffusion capacity of 10.9 mL/min/mmHg (47% predicted). Room air oximetry was normal at rest but 90% on exertion. Echocardiography and cardiac MRI did not suggest pulmonary arterial hypertension. Prior chest CTs revealed mildly prominent mediastinal and hilar adenopathy, mild reticular interstitial infiltrates without honeycombing, centrilobular and paraseptal emphysema, slight interlobular septal thickening, and mild non-specific ground glass opacities without any specific lobe predominance. Transbronchial biopsies showed noncaseating granulomas suspicious for either sarcoidosis or hypersensitivity pneumonitis. The bronchoalveolar lavage showed no lymphocytic alveolitis (1%), and was otherwise negative. Her angiotensin converting enzyme level was elevated at 62 U/L, but the rest of her blood work was unremarkable. After smoking cessation and limiting the exposure to hay and feeds, her CT scan showed improvement in the areas of ground glass opacities and adenopathy. Several months later she presented with recurrent hemoptysis and dyspnea. Repeat chest CT showed significant progression of diffuse ground glass opacities with marked intra-lobular septal thickening, worse in the upper lobes. Video-assisted thoracoscopic lung biopsy revealed typical well formed granulomas of sarcoidosis with vascular involvement, PVOD like changes, and alveolar hemorrhage. The patient was started on systemic corticosteroids with a good clinical response and significant improvement of her CT scan abnormalities.DISCUSSION: PVOD is a very rare, but potentially treatable complication of sarcoidosis. Hemoptysis in these patients is likely due to alveolar hemorrhage from pulmonary venous hypertension resulting from PVOD. Granulomatous infiltration of the pulmonary veins is the likely mechanism for PVOD like features in sarcoidosis.CONCLUSIONS: Although rare, this condition should be considered in the evaluation of patients with hemoptysis and alveolar hemorrhage as well as in patients with a diagnosis of sarcoidosis with atypical radiographic features.1) Jones RM et al Sarcoidosis-related PVOD presenting with recurrent haemoptysis. European Respiratory Journal. 34:517-520,2009.DISCLOSURE: The following authors have nothing to disclose: Jose Soto Soto, Andras Khoor, Augustine LeeNo Product/Research Disclosure InformationMayo Clinic, Jacksonville, FL.