Pulmonary Veno Occlusive Disease: Possible Causes and Treatments

November 12, 2012

Pulmonary Veno Occlusive Disease: Possible Causes and Treatments

Pulmonary veno occlusive disease is a rather unknown rare condition that can strike males and females, beginning at two months old. The causes for this disease is still not known.

According to the World Health Organization, there are a number of processes that will cause pulmonary hypertension. But, pulmonary veno occlusive disease accounts for a minute number of pulmonary hypertension cases. Now, Dr. Julius Hora documented the first known case for pulmonary veno occlusive disease in a seemingly healthy 48-year-old baker who died after suffering a year-long course that developed into dyspnea, cyanosis and edema. But, since this time, this condition is still not well-known including its history, causes and possible treatments.

An In-Depth Look At Pulmonary Veno Occlusive Disease

Pulmonary veno occlusive disease is still relatively unknown with many causes being misdiagnosed as being primary pulmonary hypertension. In the early stages of POVD, the occlusion may actually be loose, edematous tissue that later turns into sclerotic, dense fibrous tissue. This congealing is seen within the lobular septal veins and venules and, in some cases, the larger veins. Plus, dilation of the lymphatics also takes place. Now, plexiform arterial lesions generally seen in idiopathic PAH or pulmonary hypertension patients are not present but some thickening of the arterial medial is present. Over time, recanalization of the veins can also happen.

The Possible Reasons Of Pulmonary Veno Occlusive Disease

Since there is no real information behind what actually causes, all there is a possibility of what causes pulmonary veno occlusive disease, which come from case reports and small case studies. There has been no official study on what causes the condition. So, what are some of the possible reasons someone develops pulmonary veno occlusive disease?

Infection

Although no one infection has stood out in causing PVOD, there have been several infections where POVD has been found such as HIV, measles and Toxoplasma gondii.

Genetics

There have been several cases that suggest that genetics is a reason for PVOD development especially in siblings. After all, the siblings seem to get the condition one year apart from each other. Still, it’s not iron clad as the possibility to environmental exposures also exists for the development.

Toxic Exposures

Exposure to chemicals, such as chemotherapy regimens, has been raised to explain some PVOD cases. And, it’s difficult to identify the culprit chemicals with continued exposure over several years. It’s believed that bleomycin, carmustine and mitomycin are possible causes for PVOD. It’s also thought that changes in drug metabolism that allows for toxic metabolites could be a possible cause for people suffering with PVOD.

Two more possible causes of pulmonary veno occlusive disease include:

  • Radiation/thrombotic diathesis

  • Autoimmune disorders

Although like every other possible cause, it’s all just really speculation.

The Treatment For Pulmonary Veno Occlusive Disease

It’s important to bear in mind that PVOD is actually rare so therapeutic trials have yet to be done. And, according to the majority of doctors, the current state of treatments isn’t very effective. The only real effective treatment for the disease is a lung transplant. Still, some treatments doctors might propose their patients do are:

  • Immunosuppressive medications

  • Vasodilators

  • Anticoagulants

  • Long-term oxygen therapy

  • Experimental therapies

Possible Symptoms Of Pulmonary Veno Occlusive Disease

Keep in mind that pulmonary veno occlusive disease can strike either gender and as young as eight weeks of age. Doctors will look at a patient’s symptoms, which might include:

  • Cough

  • Exertional dyspnea

  • Respiratory tract infection-like illnesses

  • Tiredness

  • Dizziness (later stages)

  • Stomach pressure (later stages)

  • Chest pain (later stages)

It’s important to be seen by your doctor if you notice any of the above symptoms to ensure that it is not pulmonary veno occlusive disease.

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