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51 years of age&#44; ex-smoker&#44; working as a gardener&#44; seen in another center for progressive dyspnea and dry cough which began 8 months previously&#46; Initial spirometry showed severe obstructive disease&#46; Bilateral pseudonodular opacities were seen on simple chest X-ray&#46; Computed tomography &#40;CT&#41; of the chest revealed patchy&#44; bilateral ground-glass opacities tending to converge in the upper lobes&#44; with areas of consolidation in the right middle and lower lobes&#44; and nodular images of up to 1<span class="elsevierStyleHsp" style=""></span>cm in diameter &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; In view of these findings&#44; fiberoptic bronchoscopy was performed&#44; giving negative cytology and microbiology results&#46; Transbronchial biopsy did not provide any significant findings&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Wide-spectrum antibiotics&#44; antifungals and high-dose systemic corticosteroids were subsequently started&#44; but respiratory function continued to decline&#46; Transthoracic ultrasound was then requested&#44; showing severe pulmonary hypertension &#40;pulmonary artery systolic pressure 95<span class="elsevierStyleHsp" style=""></span>mmHg&#41;&#44; dilation and slightly reduced right ventricular systolic function and mild-to-moderate tricuspid insufficiency&#46; Thromboembolic disease was ruled out on a repeat chest CT scan&#44; although in this examination&#44; progression of pulmonary interstitial involvement was observed&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient&#39;s condition continued to worsen&#44; so she was transferred to our center and admitted to the intensive care unit&#44; where progression of the lung lesions was confirmed by radiology&#46; A lung biopsy was performed by thoracotomy&#44; but the patient could not be extubated after the procedure and died a few days later&#46; Autopsy showed signs of severe pulmonary hypertension&#44; with peripheral areas of ischemic infarction and foci of interstitial fibrosis&#8211;all compatible with PVOD &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">PVOD is an uncommon and underdiagnosed manifestation that is frequently misclassified among the group of idiopathic PAHs &#40;IPAHs&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Early diagnosis is important&#44; due to its poor prognosis and poor response to pulmonary vasodilators&#44; which can cause severe pulmonary edema&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">In contrast to IPAH&#44; which is associated with mainly precapillary involvement&#44; the physiopathological effect of PVOD is to produce lesions in the post-capillary pulmonary vessels&#46; There is media hypertrophy and intimal fibrosis&#44; causing occlusion of the vascular lumen&#46; Muscle cell proliferation&#44; calcium accumulation in the vascular wall&#44; thrombotic occlusion of the microvessels and occult pulmonary hemorrhage due to post-capillary blockage are also often observed&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Diagnosis is complicated by a clinical presentation similar to that of IPAH&#46; The most common symptom is progressively increasing dyspnea&#46; There may be mild hemoptysis and chronic cough&#46; When pulmonary hypertension is severe&#44; cyanosis&#44; chest pain or syncope with exercise may be observed&#46; Infrequent presentations include diffuse alveolar hemorrhage and sudden death&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Open lung biopsy is the reference test for diagnosis&#46; However&#44; it must be taken into account that while histological proof is needed for diagnosis&#44; the risks presented to patients by this procedure limit its extensive use&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The diffusing capacity of carbon monoxide is usually reduced&#44; and in most cases ventilation is restricted&#46; Hypoxemia is more severe than in IPAH&#44; since it is negatively affected by pulmonary edema&#44; alveolar hemorrhage and extensive vascular obstruction due to fibrosis&#46; The most frequent findings on CT are centrilobular ground-glass opacities&#44; septal lines and mediastinal lymphadenopathies&#46; Pleural effusion may occur in the final stages of the disease&#46; Hemosiderin-laden macrophages may be found in bronchoalveolar lavage&#46; Transbronchial biopsy does not usually lead to a diagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">PVOD has a poorer prognosis than other forms of PAH&#46; The only curative treatment option is lung transplant&#46; Mean survival is 24 months from diagnosis and mortality usually is a result of severe right ventricular dysfunction and massive pulmonary edema&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Risk factors for this disease include BMPR2 gene mutations&#46; PVOD has also been associated with various connective tissue diseases&#46; Association with toxin exposure has also been documented&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;4</span></a> Although there have been cases of hepatic veno-occlusive disease due to inhalation of pyrrolizidine&#44; a naturally occurring alkaloid present in various trees and bushes&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> until now&#44; no case of PVOD associated with this toxin has been reported&#46; Our patient was a gardener and may have been exposed to this type of agent&#44; although it cannot be definitively established that this was the cause of her disease&#46;</p></span></span>"
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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pulmonary veno-occlusive disease &#40;PVOD&#41; is a subgroup of pulmonary arterial hypertension with a poor prognosis&#46; The diagnosis is usually delayed and treatment options other than lung transplantation are unfortunately limited&#46; We report the case of a 51-year-old female gardener diagnosed with PVOD by open lung biopsy before her death&#46; Although there are many reported cases of hepatic veno-occlusive disease due to toxic agents present in nature&#44; such as pyrrolizidine alkaloid exposure&#44; to date this has not been linked to PVOD&#46;</p>"
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        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La enfermedad pulmonar venooclusiva es una forma infrecuente de hipertensi&#243;n arterial pulmonar y su pron&#243;stico es sombr&#237;o&#46; El diagn&#243;stico suele ser tard&#237;o y el &#250;nico tratamiento curativo en la actualidad es el trasplante pulmonar&#46; Presentamos el caso de una mujer jardinera de 51<span class="elsevierStyleHsp" style=""></span>a&#241;os cuyo diagn&#243;stico se obtuvo por biopsia pulmonar abierta realizada previamente a su fallecimiento&#46; Si bien existen casos reportados de enfermedad venooclusiva hep&#225;tica secundaria a agentes t&#243;xicos presentes en la naturaleza&#44; como el alcaloide pirrolizidina&#44; hasta el momento no se han relacionado con la enfermedad a nivel pulmonar&#46;</p>"
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