Contribution from the Field
Radiologic vignette: Hughes-Stovin syndrome
Article first published online: 4 FEB 2005
Copyright © 2005 by the American College of Rheumatology
Arthritis Care & Research
Volume 53, Issue 1, pages 114–116, 15 February 2005
How to Cite
Fischer, A., Korman, D. S. and West, S. G. (2005), Radiologic vignette: Hughes-Stovin syndrome. Arthritis & Rheumatism, 53: 114–116. doi: 10.1002/art.20907
- Issue published online: 4 FEB 2005
- Article first published online: 4 FEB 2005
- Manuscript Accepted: 19 MAY 2004
- Manuscript Received: 24 APR 2004
A 49-year-old African American man with a medical history notable for long-standing systemic hypertension and deep vein thrombus of the lower extremities 1 year earlier presented with recurrent hemoptysis. There was no history of antecedent fever, weight loss, fatigue, headache, chest pain, dyspnea, leg edema, arthralgias, rash, mucosal ulcerations, or ocular disease. He was not receiving antiplatelet or anticoagulant therapy. Physical examination revealed stable vital signs and coarse breath sounds over bilateral lung fields. Coagulation study findings were normal, there was no leukocytosis, and the erythrocyte sedimentation rate was normal.
Posteroanterior and lateral radiographs of the chest showed an ill-defined 4.5-cm mass in the basilar portion of the left lower lobe and a 3.2-cm mass lateral to the upper portion of the right hilum. Contrast-enhanced computed tomography of the thorax was notable for multiple nodular densities throughout both lung fields, the largest of which was located in the left upper lobe (see arrow in Figure 1). Gadolinium-enhanced magnetic resonance imaging with magnetic resonance angiography of the chest revealed a saccular aneurysm in the left upper lobe (see thin arrow in Figure 2). A fusiform aneurysm was identified in the left lower lobe (see thick arrow in Figure 2). A tiny saccular aneurysm was seen in the right lower lobe. In addition, multiple venous occlusions were noted in the superior vena cava and the left brachiocephalic vein.
A diagnosis of Hughes-Stovin syndrome was made.
Initial treatment consisted of intravenous methylprednisolone (1 gm/day for 3 days) and oral cyclophosphamide (1.5 mg/kg/day). The patient's hemoptysis resolved and he was discharged on a tapering dose of prednisone and daily oral cyclophosphamide. Seven months later, the patient had another episode of hemoptysis. Pulmonary angiography was performed for consideration of embolization therapy. Angiography failed to reveal any of the previously identified pulmonary aneurysms. Instead, several segmental defects were present peripherally in both lungs, consistent with healing of multiple aneurysms. Two years later, gadolinium-enhanced magnetic resonance angiography revealed complete resolution of the multiple pulmonary aneurysms (see Figure 3). In addition, no new areas of aneurysmal dilatation were noted.
Aneurysms of the pulmonary arteries are rare (1). Underlying risk factors for pulmonary artery aneurysms (PAAs) include infection, structural cardiac abnormalities, structural vascular abnormalities, and pulmonary hypertension (Table 1). In many cases, PAAs appear to have resulted from the interplay among several of these factors (1). There are 2 idiopathic syndromes associated with PAAs: Behçet's disease and Hughes-Stovin syndrome (1–3).
|Causes of PAA without arteriovenous communication|
|Tuberculosis (Rasmussen's aneurysms)|
|Other (bacterial and fungal)|
|Structural cardiac abnormalities|
|Congenital heart disease|
|Acquired cardiac abnormalities|
|Structural vascular abnormalities|
|Causes of PAA with arteriovenous communication|
|Associated with hereditary hemorrhagic telangiectasia|
Behçet's disease is a multisystem inflammatory disease of unknown etiology (2, 4, 5). Patients with Behçet's disease often present with vascular, cutaneous, pulmonary, neurologic, rheumatologic, gastrointestinal, and genitourinary manifestations (2, 4, 5). The prevalence of pulmonary involvement in Behçet's disease varies from 1% to 8% (2). Pulmonary vascular involvement can lead to the formation of PAAs, thrombotic vessel occlusion, pulmonary infarction, or pulmonary hemorrhage (2).
Hughes-Stovin syndrome is an exceedingly rare disorder consisting of multiple pulmonary aneurysms and peripheral venous thrombosis (6). Since its first description by Hughes and Stovin in 1959, there have been <30 reports of its occurrence in the English medical literature. All but 2 of the reported cases occurred in men, and it typically affects men at a young age (reported cases ranged in age from 12 to 40 years) (7, 8).
The pathogenesis of Hughes-Stovin syndrome is not known. In their initial report of the autopsy evidence from their 2 patients (6), Hughes and Stovin described histologic evidence of a congenital qualitative defect in the bronchial artery walls that led to degenerative changes in the vessel walls, causing inadequate nutrition of the pulmonary arteries. In the setting of pulmonary embolic disease, inflammation and vessel wall destruction occurred and aneurysms were formed. Subsequent studies have failed to find similar defects in the bronchial arteries, thereby providing little support for their theory. Histologically the aneurysms may resemble mycotic aneurysms, but no infectious agent has been identified in any case to date. It has been suggested that Hughes-Stovin syndrome may be a variant of Behçet's disease (3). Their clinical presentations can overlap significantly; both are more common in young men, the pulmonary manifestations of the 2 can be identical, and the histology of the aneurysms in both entities can be similar (1, 4). However, the concept of pathogenic kinship remains speculative because the etiologies of both disorders remain unknown.
The presenting features of Hughes-Stovin syndrome are those of PAAs and peripheral venous thrombosis. Clinical manifestations of PAAs are nonspecific and include hemoptysis, cough, dyspnea, chest pain, and signs of pulmonary hypertension. Other associated features reported in Hughes-Stovin syndrome include fever and elevated intracranial hypertension.
Due to the nonspecific nature of the clinical manifestations of PAAs, they can be extremely difficult to diagnose. PAAs appear as hilar enlargements or round, lobulated opacities on chest radiographs (2). Although conventional angiography is considered the gold standard for diagnosis of PAAs, other noninvasive modalities may aid in the diagnosis. Helical computed tomography reveals excellent vascular images with only a small amount of contrast material. Magnetic resonance angiography is also useful but has been reported to be less sensitive than helical computed tomography in the detection of small aneurysms (2).
PAA has a poor prognosis and is one of the leading causes of death in patients with Behçet's disease (2). Similarly, Hughes-Stovin syndrome is often fatal as a result of massive hemoptysis due to PAA rupture. Optimal management of PAAs due to Behçet's disease and Hughes-Stovin syndrome is not known. Although there are no controlled trials, recommended therapy is a combination of cyclophosphamide and glucocorticoids (7). The glucocorticoids may be tapered according to the clinical response but the cyclophosphamide regimen is often continued for at least 1 year after complete remission (2). Antibiotics are ineffective and anticoagulant and thrombolytic therapy are contraindicated. Surgical resection has been the traditional treatment of choice for severe cases of massive hemoptysis due to PAAs. However, surgical intervention should be considered cautiously because of the high operative morbidity and mortality. Transcatheter arterial embolization is a less invasive procedure that holds promise as an attractive alternative to surgery (7).
- 8A case of Hughes-Stovin syndrome associated with Budd-Chiari syndrome. Case Rep Clin Pract Rev 2002; 3: 92–4., , , , , , et al.