Hugles-Stovin syndrome is a very rare and serious disease that causes multiple pulmonary artery aneurysms and several cases of deep vein thrombosis during life. Since the first description of the disease worldwide, fewer than 40 people have been diagnosed in 2013.
The disease can occur in 3 different stages, the first usually present with thrombophlebitis, the second with pulmonary aneurysms and the third and final stage characterized by rupture of an aneurysm that can cause bloody cough and death.
- The doctor best suited to diagnose and treat this disease is the rheumatologist and although its cause is not yet quite known.
- It is believed to be related to systemic vascularitis.
Symptoms of Hugles-stovin include
Usually, a person with Hugles-stovin syndrome has had symptoms for many years and the syndrome may even be confused with Behset’s disease and some researchers believe that this syndrome is actually an incomplete version of Behset’s disease.
This disease is rarely diagnosed in childhood and can be diagnosed in adolescence or adulthood after having the above symptoms and having undergone tests such as blood tests, chest x-rays, MRI or CT scan of the head and chest, as well as doppler ultrasound to control blood and cardiac circulation. There is no diagnostic criterion and the doctor should suspect this syndrome because of its similarity to Bechaet’s disease, but without all its characteristics.
The age of those diagnosed with this syndrome varies between the ages of 12 and 48.
Treatment of Hugles-Stovin syndrome is not very specific, but your doctor may recommend the use of corticosteroids such as hydrocortisone or prednisone, anticoagulants such as enxaparin, pulsed therapy and immunosuppressants such as infliximab or adalimumab that can reduce the risk and also the consequences of thrombosis aneurysms, improving quality of life and reducing the risk of death.
Hugles-Stovin syndrome can be difficult to treat and leads to high mortality because the cause of the disease is unknown and therefore treatment may not be enough to maintain the health of the affected person, as there are few diagnosed cases in the world. , doctors generally do not know about this disease, which can make it difficult to diagnose and treat.
In addition, blood thinners should be used with great caution because in some cases they may increase the risk of bleeding after rupture of an aneurysm and blood loss can be so severe that it prevents life support.