Learning objectives
Hughes-Stovin syndrome (HSS) is a rare autoimmune disorder of unknown etiology,
characterized by peripheral deep venous thrombosis (DVT) and pulmonary and/or bronchial aneurysms.
Symptoms include recurrent DVT,
cough,
dyspnea,
fever,
chest pain and haemoptysis.
Less than 50 cases has been published in the literature,most of the patients being young males aged20 to 40.
The pathogenesis is not clear,but assumed to be a consequence of angiodysplasia and vasculitis similar to those in Behcet disease.
The managementincludesmedical (immunosuppressives) and surgical (resection,
embolization) options.
However,
massive pulmonary bleeding...
Background
A 70-year old male patient was admittedbecause of haemoptysis that started ten months before.
His medical history includes sympatectomyat the age of 42 due to clinical signs of arteritis and several episodes of DVT since.
Clinical examination revealed an ulcer on the left foot.
He had no oral or genital ulcers.
Bronchoscopy showed dilated submucosal blood vessels of the intermediate and distal part of the left main bronchus.
Immunological tests revealed antiphospholipid syndrome and thrombophilia.
Clinical and radiological work-up confirmed the diagnosis of HSS.
After...
Imaging findings OR Procedure details
CT aortography was performed and tortuous and dilatated both bronchial arteries were shown with the aneurism of the right bronchial artery measuring 14x10 mm. CT pulmonary angiography also demonstrated the chronic pulmonary embolism of the main left pulmonary artery and bilateral segmental branches for lower lobes.
The aneurismatic dilatation of the lingular branch of the pulmonary artery measuring 7 mm was noted.
Conclusion
HSStypically affects young male patients presenting with the recurrent DVT,
signs of vasculitis and chest problems with haemoptysis. As in our case,
early in the course of the disease chest symptoms may be absent,
but the recurrent DVT in combination with vasculitis alone should prompt the immunological work up.
Inclusion of angiography of the aorta and pulmonary arteries in the work up could lead to the diagnosis of vascular aneurisms thus starting prompt and adequate treatment early.
References
Chalozonitis AN,
Lachanis SB,
Panagiotis M et al.
Hughes-Stovin Syndrome: a case report and review of the literature.
Cases Journal 2009;2:98.
Erkan D,
Yazici Y,
Sanders A et al.
Is Hughes sydrome Behcet's disease?
Clin Exp Rheumatol 2004;22 (Suppl 34):64-8.
Kindermann M,
Wilkens H,
Hartmann W et al.
Hughes-Stovin Syndrome.
Circulation.2003;108:156.