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Massive hemoptysis and deep venous thrombosis presenting in a woman with Hughes-Stovin syndrome: a case report.

Al-Jahdali H - J Med Case Rep (2010)

Bottom Line: The majority of cases of Hughes-Stovin syndrome are reported among men, with only two cases occurring in women.A case of Hughes-Stovin syndrome occurring in a woman is presented in this report.She was treated successfully with multimodality treatment that includes surgery, steroids and cytotoxic agents.

View Article: PubMed Central - HTML - PubMed

Affiliation: Medical Department, King Saud University for Health Sciences, King Abdulaziz Medical City, Riyadh, 11426, Saudi Arabia. jahdali@yahoo.com.

ABSTRACT

Introduction: Hughes-Stovin syndrome is a very rare disease with fewer than 30 cases reported in the literature. The disease is thought to be a variant of Behcet's disease and is defined by the presence of pulmonary artery aneurysm in association with peripheral venous thrombosis.

Case presentation: A previously healthy 23-year-old Saudi woman presented with massive hemoptysis a day prior to her admission to our hospital. She had a six-month history of recurrent fever, cough, dyspnea, and recurrent oral ulceration. Contrast-enhanced computed tomography scan of her chest and pulmonary angiogram demonstrated a single right-lower lobe pulmonary artery aneurysm. She underwent thoracotomy and right lower lobe resection. Her postoperative course was complicated by deep vein thrombosis. She also developed headache and papilledema, while a magnetic resonance imaging of her brain suggested vasculitis. Based on these clinical presentations, she was diagnosed and treated with Hughes-Stovin syndrome.

Conclusion: The majority of cases of Hughes-Stovin syndrome are reported among men, with only two cases occurring in women. A case of Hughes-Stovin syndrome occurring in a woman is presented in this report. She was treated successfully with multimodality treatment that includes surgery, steroids and cytotoxic agents.

No MeSH data available.


Related in: MedlinePlus

Cardiac magnetic resonance imaging showed the aneurysm in the right interlobar pulmonary artery (white arrow).
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Figure 3: Cardiac magnetic resonance imaging showed the aneurysm in the right interlobar pulmonary artery (white arrow).

Mentions: Transthoracic echocardiography showed a 1.8 cmx1.6 cm non-mobile right ventricular mass attached to her interventricular septum. Transesophogeal echocardiography showed the same mass having a texture similar to her papillary muscle. A cardiac MRI was subsequently done, which showed a right ventricular mass with the same signal of the cardiac muscle. An aneurysm in her right interlobar pulmonary artery was also seen (Figure 3). Further laboratory workups ruled out other connective tissue diseases except for elevated lupus anticoagulant level (LA1 = 54.9, normal = 30 to 44; LA2 = 37.2, normal = 26 to 32).


Massive hemoptysis and deep venous thrombosis presenting in a woman with Hughes-Stovin syndrome: a case report.

Al-Jahdali H - J Med Case Rep (2010)

Cardiac magnetic resonance imaging showed the aneurysm in the right interlobar pulmonary artery (white arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2864281&req=5

Figure 3: Cardiac magnetic resonance imaging showed the aneurysm in the right interlobar pulmonary artery (white arrow).
Mentions: Transthoracic echocardiography showed a 1.8 cmx1.6 cm non-mobile right ventricular mass attached to her interventricular septum. Transesophogeal echocardiography showed the same mass having a texture similar to her papillary muscle. A cardiac MRI was subsequently done, which showed a right ventricular mass with the same signal of the cardiac muscle. An aneurysm in her right interlobar pulmonary artery was also seen (Figure 3). Further laboratory workups ruled out other connective tissue diseases except for elevated lupus anticoagulant level (LA1 = 54.9, normal = 30 to 44; LA2 = 37.2, normal = 26 to 32).

Bottom Line: The majority of cases of Hughes-Stovin syndrome are reported among men, with only two cases occurring in women.A case of Hughes-Stovin syndrome occurring in a woman is presented in this report.She was treated successfully with multimodality treatment that includes surgery, steroids and cytotoxic agents.

View Article: PubMed Central - HTML - PubMed

Affiliation: Medical Department, King Saud University for Health Sciences, King Abdulaziz Medical City, Riyadh, 11426, Saudi Arabia. jahdali@yahoo.com.

ABSTRACT

Introduction: Hughes-Stovin syndrome is a very rare disease with fewer than 30 cases reported in the literature. The disease is thought to be a variant of Behcet's disease and is defined by the presence of pulmonary artery aneurysm in association with peripheral venous thrombosis.

Case presentation: A previously healthy 23-year-old Saudi woman presented with massive hemoptysis a day prior to her admission to our hospital. She had a six-month history of recurrent fever, cough, dyspnea, and recurrent oral ulceration. Contrast-enhanced computed tomography scan of her chest and pulmonary angiogram demonstrated a single right-lower lobe pulmonary artery aneurysm. She underwent thoracotomy and right lower lobe resection. Her postoperative course was complicated by deep vein thrombosis. She also developed headache and papilledema, while a magnetic resonance imaging of her brain suggested vasculitis. Based on these clinical presentations, she was diagnosed and treated with Hughes-Stovin syndrome.

Conclusion: The majority of cases of Hughes-Stovin syndrome are reported among men, with only two cases occurring in women. A case of Hughes-Stovin syndrome occurring in a woman is presented in this report. She was treated successfully with multimodality treatment that includes surgery, steroids and cytotoxic agents.

No MeSH data available.


Related in: MedlinePlus