Described as a form of pulmonary arterial hypertension (PAH), pulmonary veno-occlusive disease (PVOD) is a pathological process involving small veins in the pulmonary circulation, and is estimated to encompass about 5-10% of cases previously diagnosed as idiopathic PAH [1] [2] [3] [4]. Several risk factors have been established, including male gender and increased exposure to tobacco smoke [3] [5]. The disease is marked by an elevation of pressure inside the pulmonary vessels that could eventually result in right ventricular failure [2] [5]. The clinical presentation of pulmonary veno-occlusive disease is strikingly similar to PAH with severe exertional dyspnea as one of the main symptoms. Some authors observed hemoptysis and occult alveolar hemorrhage in a small but significant number of patients [2] [3] [4]. Symptoms may rapidly progress within a short period of time after administration of standard PAH therapy. Although considered a subtype of PAH, PVOD resistance to standard PAH therapy and more pronounced risk for cardiorespiratory insufficiency are distinctive characteristics of the disease.

• Fatigue

  Clinical presentation is non-specific, including dyspnoea, cough and fatigue. Diagnosis of PVOD is typically based on high clinical suspicion with a definitive diagnosis confirmed by histology. [ncbi.nlm.nih.gov]

  It is believed to be associated with blood cancers, such as leukemia and lymphoma, and bone marrow transplantation and chemotherapy Common signs and symptoms of Pulmonary Veno-Occlusive Disease are shortness of breath, coughing-up blood, fatigue, and [dovemed.com]

  Shortness of breath (dyspnea) and tiredness (fatigue) during exertion are the most common symptoms of this condition. [ghr.nlm.nih.gov]

  […] pediatric population, both sexes are equally affected in the adult population, the ratio of male to female patients is approximately 2:1 Its presentation can be similar to other forms of pulmonary arterial hypertension such as progressive dyspnea, cough, fatigue [radiopaedia.org]

• Pathologist

  It is important that both clinician and pathologist be aware of this entity; otherwise, elastic tissue stains may not be performed and the diagnosis missed. [ncbi.nlm.nih.gov]

  Ideally this should be in a multidisciplinary clinical setting with input from respiratory physicians, rheumatologists, cardiologists, radiologists and pathologists with experience in pulmonary vascular disease, as well as specialised nurses and paramedical [em-consulte.com]

• Recurrent Pulmonary Embolism

  Chest roentgenogram finding suggesting postcapillary pulmonary hypertension is a clue to a diagnosis and differentiates this from two other causes of clinical primary pulmonary hypertension, that is, recurrent pulmonary embolism and plexogenic pulmonary [ncbi.nlm.nih.gov]

  Secondary thromboembolic events are common and may be the only clinical evidence of a tumor at presentation, with clinical features similar to those of recurrent pulmonary embolism. [doi.org]

• Sudden Infant Death Syndrome

  Clinical and gross autopsy findings on a 3-month-old infant were consistent with the sudden infant death syndrome. However, on histologic examination pulmonary veno-occlusive disease was found. [ncbi.nlm.nih.gov]

• Infectious Mononucleosis

  This case report is about a patient with PVOD, whose first symptoms of the disease occurred after infectious mononucleosis. [ncbi.nlm.nih.gov]

• Dyspnea

  The patient was a 21-year-old male who complained of dyspnea with hypoxemia followed by loss of consciousness. The abnormalities in chest CT and echocardiography were compatible with a diagnosis of PVOD. [ncbi.nlm.nih.gov]

• Cough

  Clinical presentation is non-specific, including dyspnoea, cough and fatigue. Diagnosis of PVOD is typically based on high clinical suspicion with a definitive diagnosis confirmed by histology. [ncbi.nlm.nih.gov]

  Fatigue on exertion Fainting Coughing up blood Difficulty breathing while lying flat Exams and Tests The health care provider will examine you and ask about your medical history and symptoms. [leehealth.org]

  It is believed to be associated with blood cancers, such as leukemia and lymphoma, and bone marrow transplantation and chemotherapy Common signs and symptoms of Pulmonary Veno-Occlusive Disease are shortness of breath, coughing-up blood, fatigue, and [dovemed.com]

  Clinical Case Female, 51 years of age, ex-smoker, working as a gardener, seen in another center for progressive dyspnea and dry cough which began 8 months previously. Initial spirometry showed severe obstructive disease. [archbronconeumol.org]

• Exertional Dyspnea

  A 58-year-old woman was referred to our hospital with a chief complaint of exertional dyspnea. [ncbi.nlm.nih.gov]

  The clinical presentation of pulmonary veno-occlusive disease is strikingly similar to PAH with severe exertional dyspnea as one of the main symptoms. [symptoma.com]

  Case report A 53-year-old woman with no history of smoking became aware of the onset of exertional dyspnea in early June, 2011. Her symptoms gradually increased, and were accompanied by a dry cough. [omicsonline.org]

  The clinical manifestations are nonspecific and may include exertional dyspnea (60% of all cases), fatigue, angina, syncope, and cor pulmonale ( 1, 14 ). [doi.org]

• Sputum

  There were statistically significant more haemosiderin-laden macrophages in the sputum of patients suffering from PVOD as compared to the other groups (P CONCLUSION: The content of haemosiderin-laden macrophages in the sputum of patients suffering from [ncbi.nlm.nih.gov]

  Sputum collection and processing Sputum from every patient was obtained by a non-invasive manner without any induction by hypertonic saline solution. [journals.plos.org]

  The clinical utility of sputum examination as a noninvasive tool in the work-up of PVOD warrants further study. [doi.org]

• Painful Cough

  cough, hemoptysis or syncope. 16, 20, 22 Physical exam findings are typically nonspecific and consistent with that of pulmonary hypertension. [nature.com]

  Some patients suffer from chest pain, cough (which is often nonproductive), cyanosis, and, rarely, syncope [ 5, 7 ]. Hemoptysis occurred in 8 out of 20 patients in a case series [ 5 ] but in only 1 out of 24 patients in another series [ 6 ]. [karger.com]

• Failure to Thrive

  In infants, the disease is initially observed as feeding difficulty and/or failure to thrive, often accompanied by mild respiratory distress. Two of the infants died suddenly. [ncbi.nlm.nih.gov]

• Cyanosis

  As the condition worsens, affected individuals can develop a bluish tint to the skin (cyanosis), chest pains, fainting spells, and an accumulation of fluid in the lungs ( pulmonary edema ). [ghr.nlm.nih.gov]

  The exam may reveal: Increased pressure in the neck veins Clubbing of the fingers Bluish coloration of the skin due to lack of oxygen ( cyanosis ) Swelling in the legs Your provider may hear abnormal heart sounds when listening to the chest and lungs [mountsinai.org]

  Common signs and symptoms associated with Pulmonary Venoocclusive Disease include: Shortness of breath on exertion Fatigue Cyanosis (bluish skin discoloration) Hemoptysis (coughing-up blood) Syncope (fainting) in later stages How is Pulmonary Veno-Occlusive [dovemed.com]

• Neglect

  As in PAH, progressive dyspnoea on exertion is the most frequent symptom, although it is often neglected by the patients, leading to frequent delay in establishing the diagnosis. [erj.ersjournals.com]

• Forgetful

  When clinicians encounter patients with interstitial lung disease, they should not forget the possibility of PVOD and should be alert for emerging pulmonary hypertension. [ncbi.nlm.nih.gov]

The diagnosis of pulmonary veno-occlusive disease (PVOD) may be difficult to attain solely on clinical grounds, but the importance of a detailed patient history and a proper physical examination must not be overlooked. Physicians should obtain a meticulous personal history since PVOD has been associated with human immunodeficiency virus (HIV) infection, sarcoidosis, Langerhans cell granulomatosis, and connective tissue diseases [5]. During the physical examination, auscultation of the lungs and the heart can provide valuable clues, such as systolic murmurs (which may point to tricuspid regurgitation) and crackles [2]. Many studies have proposed that the diagnosis of pulmonary veno-occlusive disease is ideally made by obtaining a biopsy of the lungs with subsequent histopathological examination [2] [3] [5] [6], but the numerous risks of this procedure advocate the use of non-invasive studies, most important being arterial blood gases and imaging procedures. Pulse oximetry and pulmonary function tests confirm hypoxemia and a low diffusing capacity for carbon monoxide (CO) respectively, whereas high-resolution computed tomography (HRCT), described as a very useful imaging study for the diagnosis of PVOD, reveals centrilobular ground-glass opacities, thickening of the septa, and enlargement of lymph nodes [1] [2] [7].

• Lymphocytic Infiltrate

  Cases with PCH also all showed a mild interstitial lymphocytic infiltrate but there was no venulitis or infarction. [ncbi.nlm.nih.gov]

  infiltrates, lymphocytic venulitis or leukocytoclastic vasculitis may be associated with interstitial fibrosis. 16, 22 Role of vascular injury and endothelial dysfunction The endothelium plays a dynamic role in maintaining normal microvascular blood [nature.com]

We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect. [ncbi.nlm.nih.gov]

KEYWORDS: High resolution computed tomography; Histopathology; Prognosis; Pulmonary hypertension; Pulmonary veno-occlusive disease [ncbi.nlm.nih.gov]

Its etiology is still poorly understood but is related to several risk factors. The histopathology of PVOD is characterized by intimal fibrosis narrowing or the occlusion of small pulmonary veins or venules. [ncbi.nlm.nih.gov]

Etiology The exact etiology of pulmonary veno-occlusive disease (PVOD) remains largely unknown. However, several indirect observations have led to many hypotheses regarding the etiology. [emedicine.medscape.com]

Rare Diseases/epidemiology, Rare Diseases/therapy, Respiratory Function Tests/methods, Risk Factors [pesquisa.bvsalud.org]

This review summarises the epidemiologic, clinico-pathologic and imaging characteristics of PVOD in the setting of CTD and discusses potential management approaches. Copyright  2010. Published by Elsevier Masson SAS. [ncbi.nlm.nih.gov]

Moreover, epidemiological information on CTEPH is scarce [ 10 ]. A multinational registry on CTEPH reported recently provides information on clinical characteristics of the disease, but not on its epidemiology [ 11 ]. [erj.ersjournals.com]

(See Epidemiology, Treatment, and Medication .) In the past, PVOD has been described by various terms, such as pulmonary venous sclerosis, obstructive disease of the pulmonary veins, or the venous form of primary pulmonary hypertension. [emedicine.medscape.com]

Endothelial damage may represent the pathophysiologic foundation of these clinical syndromes. Recent advances in the treatment of hepatic veno-occlusive disease may have application to its pulmonary counterpart. [ncbi.nlm.nih.gov]

This new edition incorporates the latest clinical, pathophysiological and pathological research on pulmonary circulatory disorders. [books.google.de]

The pathophysiology of veno-occlusive disease culminates in occlusion of the pulmonary blood vessels. This could be due to edematous tissue (sclerotic fibrous tissue). [en.wikipedia.org]

Amifostine prevents MMC-induced PVOD in rats and should be tested as a preventive therapy for MMC-induced PVOD in humans. [ncbi.nlm.nih.gov]

These biopsies are dangerous for PH patients, but may be necessary in order to prevent endangering the patient with potentially harmful therapies. [phassociation.org]

Seite 223 - American Thoracic Society/Centers for Disease Control and Prevention/Infectious Diseases Society of America: treatment of tuberculosis. Am J Respir Crit Care Med 2003;167:603-662. ‎ [books.google.com]

1. Montani D, Lau EM, Dorfmüller P, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2016;47(5):1518-1534.
2. Montani D, Price LC, Dorfmuller P, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2009;33(1):189-200.
3. Montani D, Achouh L, Dorfmüller P, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore). 2008;87(4):220-233.
4. Rabiller A, Jais X, Hamid A, et al. Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J 2006;27:108–113.
5. Montani D, O'Callaghan DS, Savale L, et al. Pulmonary veno-occlusive disease: recent progress and current challenges. Respir Med. 2010;104(Suppl 1):S23-32.
6. Porres-Aguilar M, Al-Bayati I, Porres-Muñoz M, et al. Pulmonary veno-occlusive disease as a cause of pulmonary arterial hypertension. Proceedings Proc (Bayl Univ Med Cent). 2015;28(3):350-352.
7. Resten A, Maitre S, Humbert M, et al. Pulmonary hypertension: CT of the chest in pulmonary veno-occlusive disease. Am J Roentgenol. 2004;183:65–70.