Hugles-stovin syndrome is a very rare and serious disease that causes multiple aneurysms in the pulmonary artery and several cases of deep vein thrombosis during life. Since the first description of this disease worldwide, less than 40 people have been diagnosed by the year 2013.
The disease can present itself in 3 different stages, where the first usually presents with thrombophlebitis, the second stage with pulmonary aneurysms, and the third and last stage is characterized by the rupture of an aneurism that can cause bloody cough and death.
The most suitable doctor to diagnose and treat this disease is the rheumatologist and although its cause is not yet fully known, it is believed that it may be related to a systemic vasculitis.
Symptoms
Hugles-stovin's symptoms include:
- Coughing up blood, difficulty breathing, feeling short of breath; Headache; High, persistent fever; Loss of approximately 10% of weight without apparent cause; Papilledema, which is an enlargement of the optic papilla that represents an increase in pressure within the brain; Swelling and severe pain in the calf; Double vision and Convulsions.
Usually an individual with Hugles-stovin syndrome has symptoms for many years and the syndrome can even be confused with Behçet's disease and some researchers believe that this syndrome is actually an incomplete version of Behçet's disease.
This disease is rarely diagnosed in childhood and can be diagnosed in adolescence or adulthood after presenting the aforementioned symptoms and undergoing tests such as blood tests, chest radiography, MRI or CT scan of the head and chest, in addition to doppler ultrasound to check blood and heart circulation. There is no diagnostic criterion and the doctor should suspect this syndrome due to its similarity to Bechçet's disease, but without all its characteristics.
The age of people diagnosed with this syndrome varies between 12 and 48 years.
Treatment
The treatment for Hugles-Stovin syndrome is not very specific, but the doctor may recommend the use of corticosteroids such as hydrocortisone or prednisone, anticoagulants such as enoxaparin, pulse therapy and immunosuppressants such as Infliximab or Adalimumab that can decrease the risk and also the consequences of aneurysms and thrombosis, thus improving the quality of life and decreasing the risk of death.
Complications
Hugles-Stovin syndrome can be difficult to treat and has a high mortality because the cause of the disease is not known and therefore treatments may not be sufficient to maintain the health of the affected person. As there are few cases diagnosed worldwide, doctors are usually unfamiliar with this disease, which can make diagnosis and treatment more difficult.
In addition, anticoagulants should be used with great care because in some cases they can increase the risk of bleeding after an aneurysm ruptures and blood leakage can be so great that it prevents the maintenance of life.
