Pulmonary veno-occlusive disease
Pulmonary veno-occlusive disease (PVOD) is an extremely rare form of
pulmonary hypertension, affecting mostly in children and young adults as
a result of a progressive obstruction of small pulmonary veins that
leads to elevation in pulmonary vascular resistance and right
ventricular failure.
A. Causes
Although the cause of Pulmonary veno-occlusive disease (PVOD) is considered unknown, some studies suggested the following
1. Oral contraceptives
According to the study by the University of California San Francisco,
there ia 2 cases of PVOD that developed in 2 young women soon after the
initiation of oral contraceptives (OCs). The first patient is a
14-year-old girl, with no medical history, who started taking an OC 3
weeks before the onset of symptoms. The second patient is an 18-year-old
girl, diagnosed 2 years previously with systemic lupus erythematosus
and lupus anticoagulant, who started taking an OC 4 months before the
onset of symptoms(7).
2. Idiopathic or complicating other conditions
According to the study by the Hôpital Antoine Béclère, Assistance
Publique – Hôpitaux de Paris, Université Paris-Sud, Clamart, France,
PVOD has been described as idiopathic or complicating other conditions,
including connective tissue diseases, HIV infection, bone marrow
transplantation, sarcoidosis and pulmonary Langerhans cell
granulomatosis(8).
3. Infections, Toxic Exposures, Thrombotic Diathesis, Autoimmune Disorders may also be the possible causes of PVOD
a. Infection and Toxic Exposures
No convincing data have linked PVOD to a specific infectious insult,
although an “influenzalike illness” has preceded the development of PVOD
in many cases, and serologic evidence suggestive of recent infection
with one of several agents (including Toxoplasma gondii and measles) has
been documented around the time when PVOD was diagnosed(8a). According
to the study by the Service de Pneumo-Allergologie, there is a report of
case of a 27 year old male intravenous drug abuser with HIV infection
and pulmonary hypertension. Open lung biopsy led to the diagnosis of
pulmonary veno-occlusive disease. This second case of pulmonary
veno-occlusive disease raises the question of a relationship between HIV
infection and lesions involving the pulmonary veins. However, the
pathogenesis of vascular changes remains to be elucidated(9). Other
study report a case of a Caucasian female with a long history of
progressive dyspnoea ultimately diagnosed as focal granulomatous
venulitis leading to a pulmonary veno-occlusive disease-like
pathology(10).
b. Thrombotic Diathesis
According to the study by research team lead by Tsou E, there is a
report of a 23-year-old woman in her 27th week of gestation presented
with clinical findings of progressive pulmonary hypertension. After
cardiac catheterization she went into labor and was delivered by
cesarean section. She died shortly thereafter from right heart failure.
Pulmonary venoocclusive disease was found at autopsy. Hemodynamic
changes during pregnancy, labor, delivery, and the postpartum period may
have contributed to her deterioration and death(11).
c. Autoimmune Disorders
There is a report of a 26-year-old woman with systemic lupus
erythematosus (SLE) developed dyspnea and hypoxemia on exertion. She
died from rapidly progressive respiratory failure. Autopsy revealed
right ventricular hypertrophy and occlusion of the pulmonary veins
compatible with pulmonary venoocclusive disease (PVOD), according to the
study by Keio University School of Medicine(12).
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Sources
(7) http://www.ncbi.nlm.nih.gov/pubmed/22884388
(8) http://www.ncbi.nlm.nih.gov/pubmed/19118230
(8a) http://www.atsjournals.org/doi/full/10.1164/ajrccm.162.5.9912045#h15
(9) http://www.ncbi.nlm.nih.gov/pubmed/8620974
(10) http://www.ncbi.nlm.nih.gov/pubmed/19251802
(11) http://www.ncbi.nlm.nih.gov/pubmed/6738961
(12) http://www.ncbi.nlm.nih.gov/pubmed/8014950
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