Hughes–Stovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and . Learning points for clinicians. Pulmonary artery aneurysms (PAA) have a diverse differential diagnosis. Vasculitic features, without evidence of. Hughes-Stovin syndrome (HSS) is a rare autoimmune disorder, characterized by deep venous thrombosis (DVT) and pulmonary and/or bronchial aneurysms.

Author: Vudotilar Vuzshura
Country: Senegal
Language: English (Spanish)
Genre: Business
Published (Last): 18 December 2016
Pages: 175
PDF File Size: 1.20 Mb
ePub File Size: 7.56 Mb
ISBN: 840-1-35021-360-7
Downloads: 97257
Price: Free* [*Free Regsitration Required]
Uploader: Gacage

The pulmonary arteries showed widespread lesions of Regardless of the modality used, a complete visualization of prominent bronchial arteries and pathologic enhancement of pulmonary stvoin should alert physicians to the suspicion of pulmonary vasculitis. Laboratory findings The laboratory findings in HSS patients are non-specific. Background Hughes-Stovin syndrome is a rare condition first described in [ 1 ] which is characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis.

Helicobacter pylori Almost equal proportion of patients with Behcet’s disease and controls had H. BD primarily affects young adults, especially males [ 404151 ]. For BD, the following infectious agents have been implicated in the pathogenesis but never conclusively proven: Specialised Social Services Eurordis directory.

Authors have performed embolization with several agents including steel coils, ethibloc and an epoxy, isobutyl cyanoacrylate [ 8 ]. Am J Emerg Med. After 10 days on heparin therapy, dry cough begun, followed by an episode of moderate haemoptysis — ml. However, the trend obviously needs to be confirmed in other patients before deriving any conclusions. Future research should focus on the immunogenetic relationship between HSS and BD, optimization of immunosuppressive strategies through prospective controlled trials and evaluation of the long-term outcomes of embolization procedures.


Open in a separate window.

N Engl J Med. It is hugehs that the aneurysms seen in BD arise either due to the process of obliterative endarteritis of the vasa vasorum or they are pseudo-aneurysms characterized by edematous vessel walls. Bronchial artery hypertrophy secondary to ischemia that, in turn, has been attributed to the pulmonary artery occlusion [ 21 ].

Hughes-Stovin Syndrome: a case report and review of the literature

Macrophages containing hemosiderin may also be observed. All authors have read and approved the final manuscript. Haemoptysis resolved with intravenous methylprednisolone treatment after about 25 days. In general there is a thrombus formation predisposition affecting the peripheral veins.

Almost equal proportion of patients with Behcet’s disease hughess controls had H. Immunosuppressive therapy Most commonly, immunosuppressive therapy involving a combination regimen of glucocorticoids and cyclophosphamide has been employed as a first line medical management in the treatment of HSS, although its effectiveness remains to be fully established [ 840 ].

This article has been cited by other articles in PMC. Contrast-Enhanced MRA and Contrast-Enhanced MDCTA may provide an alternative [ 12 – 14 ] while 3D volume rendering analysis can ideally visualize the syndrme of an increased number of morphologically abnormal bronchial arteries even before aneurysm formation [ 9 ]. However, it should be noted that selective pulmonary angiography can be hazardous as it carries the risk of aneurysm rupture [ 17 ].

Orphanet: Hughes Stovin syndrome

Comparison with Conventional Angiography. Pulmonary arterial thrombo-embolism and pulmonary arterial mycotic aneurysms in cattle with vena caval thrombosis: Angiodysplasia of bronchial arteries is another debatable hypothesis to account for the vascular changes [ 162837 ]. This page was last edited on 9 Julyat Signs and symptoms of Hughes-Stovin Syndrome.

Diagnosis of HSS is made on the basis of the clinical picture association of venous thrombosis and PAAs in a young patientpatient history and imaging studies chest radiographs, conventional angiography or helical computed huthes for detection and evaluation of the PAAs. From Wikipedia, the free encyclopedia. Other search option s Alphabetical list. The typical presenting features of HSS are related to the presence of the pulmonary aneurysms and peripheral venous thrombosis. Multiple mycotic pulmonary artery aneurysms.


Pulmonary vasculitis in behcet disease: Streptococcus sanguis, Streptococcus mitis and Streptococcus salivarius. Multiple pulmonary aneurysms secondary to Hughes-Stovin syndrome: Durieux et al [ 37 ] used surgical intervention for three cases of HSS who had isolated pulmonary aneurysms and none of them showed any signs of recurrence on subsequent follow ups.

Hughes-Stovin Syndrome: a case report and review of the literature

Overall, patients with HSS have a poor prognosis and aneurysmal rupture is the leading cause of death. Email alerts New issue alert.

There was no previous trauma or features of connective tissue disorders. Unclear role, distribution and pathogenetic relationship of ASCA antibodies in patients with Behcet’s disease.

A case of Hughes-Stovin syndrome associated with hyperhomocysteinemia. Being an extremely rare disease, there is no formally described diagnostic criteria or pathognomonic laboratory investigation for this syndrome. For cases of massive syndfome due to large pulmonary aneurysms or those with lesions confined to one segment or one lung, lobectomy or pneumectomy can be carried out.

Written by