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Can Behcet's Disease Related Pulmonary Arterial Aneurysms be Completely Resolved?

Agha A, Bella AM, Assiri AH, Al-Hakami M - Open Rheumatol J (2011)

Bottom Line: Behçet disease is a systemic disease with diverse clinical symptoms which include, but not limited to, patients having oral and genital ulcers and eye involvement.We here report an 18-year-old male presenting with massive hemoptysis and cardiac arrest, having history of ulcers in the oral cavity and genitalia as well as having recent episode of uveitis.On receiving Immunosuppressive treatment for Behcet disease with prednisone and azathioprine over one year the pulmonary arterial aneurysms were completely resolved and the patient was discharged from the hospital albeit with persistent hypoxic brain injury from cardiac arrest.

View Article: PubMed Central - PubMed

Affiliation: Armed Forces Hospital Southern Region, Khamis Mushyt, Kingdom of Saudi Arabia.

ABSTRACT
Behçet disease is a systemic disease with diverse clinical symptoms which include, but not limited to, patients having oral and genital ulcers and eye involvement. We here report an 18-year-old male presenting with massive hemoptysis and cardiac arrest, having history of ulcers in the oral cavity and genitalia as well as having recent episode of uveitis. A pulmonary CT angiography revealed bilateral arterial aneurysms of pulmonary vessels. On receiving Immunosuppressive treatment for Behcet disease with prednisone and azathioprine over one year the pulmonary arterial aneurysms were completely resolved and the patient was discharged from the hospital albeit with persistent hypoxic brain injury from cardiac arrest.

No MeSH data available.


Related in: MedlinePlus

CT angiography of the chest showing multiple pulmonary arterial aneurysms with left pulmonary infarction.
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Figure 1: CT angiography of the chest showing multiple pulmonary arterial aneurysms with left pulmonary infarction.

Mentions: As the diagnosis remained elusive, the family was again called for obtaining additional points in history. On 14th post admission day, during re-interview with the family about recent oral ulcer that the patient had now developed in the mouth while in intensive care unit, they revealed that the patient has recurrent oral and genital ulcers on almost a monthly basis along with recurrent joint pains off and on. His Pathergy test was done which was inconclusive. His ophthalmological examination was significant for posterior uveitis. Rheumatologist’s opinion was sought and it was mutually agreed by the multidisciplinary team to treat the patient with steroids as a case of Behcet’s disease. A second CT chest with contrast was done with reconstruction imaging, which showed massive pulmonary arteriovenous malformations/aneurysm (see Fig. 1) and left small pulmonary infarct without any evidence of thromboembolism. His steroids were changed to Methylprednisolone 1 gram intravenous pulse therapy for 5 days in addition to initiation of azathioprine 50mg. The family was counseled but did not agree to consent for cyclophosphamide therapy due to potential side effects. The steroids were then tapered down over 6 months and maintained on 10 mg prednisolone with azathiprine 75 mg daily along with osteoporosis prevention medications.


Can Behcet's Disease Related Pulmonary Arterial Aneurysms be Completely Resolved?

Agha A, Bella AM, Assiri AH, Al-Hakami M - Open Rheumatol J (2011)

CT angiography of the chest showing multiple pulmonary arterial aneurysms with left pulmonary infarction.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3245488&req=5

Figure 1: CT angiography of the chest showing multiple pulmonary arterial aneurysms with left pulmonary infarction.
Mentions: As the diagnosis remained elusive, the family was again called for obtaining additional points in history. On 14th post admission day, during re-interview with the family about recent oral ulcer that the patient had now developed in the mouth while in intensive care unit, they revealed that the patient has recurrent oral and genital ulcers on almost a monthly basis along with recurrent joint pains off and on. His Pathergy test was done which was inconclusive. His ophthalmological examination was significant for posterior uveitis. Rheumatologist’s opinion was sought and it was mutually agreed by the multidisciplinary team to treat the patient with steroids as a case of Behcet’s disease. A second CT chest with contrast was done with reconstruction imaging, which showed massive pulmonary arteriovenous malformations/aneurysm (see Fig. 1) and left small pulmonary infarct without any evidence of thromboembolism. His steroids were changed to Methylprednisolone 1 gram intravenous pulse therapy for 5 days in addition to initiation of azathioprine 50mg. The family was counseled but did not agree to consent for cyclophosphamide therapy due to potential side effects. The steroids were then tapered down over 6 months and maintained on 10 mg prednisolone with azathiprine 75 mg daily along with osteoporosis prevention medications.

Bottom Line: Behçet disease is a systemic disease with diverse clinical symptoms which include, but not limited to, patients having oral and genital ulcers and eye involvement.We here report an 18-year-old male presenting with massive hemoptysis and cardiac arrest, having history of ulcers in the oral cavity and genitalia as well as having recent episode of uveitis.On receiving Immunosuppressive treatment for Behcet disease with prednisone and azathioprine over one year the pulmonary arterial aneurysms were completely resolved and the patient was discharged from the hospital albeit with persistent hypoxic brain injury from cardiac arrest.

View Article: PubMed Central - PubMed

Affiliation: Armed Forces Hospital Southern Region, Khamis Mushyt, Kingdom of Saudi Arabia.

ABSTRACT
Behçet disease is a systemic disease with diverse clinical symptoms which include, but not limited to, patients having oral and genital ulcers and eye involvement. We here report an 18-year-old male presenting with massive hemoptysis and cardiac arrest, having history of ulcers in the oral cavity and genitalia as well as having recent episode of uveitis. A pulmonary CT angiography revealed bilateral arterial aneurysms of pulmonary vessels. On receiving Immunosuppressive treatment for Behcet disease with prednisone and azathioprine over one year the pulmonary arterial aneurysms were completely resolved and the patient was discharged from the hospital albeit with persistent hypoxic brain injury from cardiac arrest.

No MeSH data available.


Related in: MedlinePlus