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Aortic aneurysm rupture as a rare complication of granulomatosis with polyangiitis: a case report.

Ohta N, Waki T, Fukase S, Suzuki Y, Kurakami K, Aoyagi M, Kakehata S - J Med Case Rep (2013)

Bottom Line: As far as we know this is the first reported case of successful treatment in a patient with granulomatosis with polyangiitis complicated with aortic aneurysm rupture.The patient was operated on and a J-graft was inserted.He recovered uneventfully.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Otolaryngology, Yamagata University Faculty of Medicine, 2-2-2 Iida-nishi, Yamagata, 990-23, Japan. noohta@med.id.yamagata-u.ac.jp.

ABSTRACT

Introduction: Granulomatosis with polyangiitis is characterized by systemic inflammation of medium and small blood vessels. Aortic involvement in granulomatosis with polyangiitis is extremely rare. As far as we know this is the first reported case of successful treatment in a patient with granulomatosis with polyangiitis complicated with aortic aneurysm rupture.

Case presentation: We describe a case of granulomatosis with polyangiitis in a 38-year-old Japanese man who developed an aortic aneurysm rupture 22 years after disease onset. The patient was operated on and a J-graft was inserted. He recovered uneventfully.

Conclusion: Recommendations in regard to, and consideration of, aortic involvement should be kept in mind in the long-term careful follow up of granulomatosis with polyangiitis.

No MeSH data available.


Related in: MedlinePlus

Histpathological findings of aortic aneurysm from patient with GPA. (A) Histologic section showing considerable arteriosclerosis in the intima and media, with cholesterol clefts. There is intimal thickening with reduplication of the internal elastic lamina. The media is thickened, and the medial smooth muscle cells are replaced by pink hyaline material (hematoxylin and eosin, original magnification ×100). (B) Arteritis with giant-cell formation and lymphocyte and plasma cell infiltration around the vasa vasorum in the media is evident. The aortic media shows patchy infiltration by lymphocytes (plasma cells), with early disruption of the media (hematoxylin and eosin, original magnification ×270).
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Figure 2: Histpathological findings of aortic aneurysm from patient with GPA. (A) Histologic section showing considerable arteriosclerosis in the intima and media, with cholesterol clefts. There is intimal thickening with reduplication of the internal elastic lamina. The media is thickened, and the medial smooth muscle cells are replaced by pink hyaline material (hematoxylin and eosin, original magnification ×100). (B) Arteritis with giant-cell formation and lymphocyte and plasma cell infiltration around the vasa vasorum in the media is evident. The aortic media shows patchy infiltration by lymphocytes (plasma cells), with early disruption of the media (hematoxylin and eosin, original magnification ×270).

Mentions: A 38-year-old Japanese man was admitted to our hospital with the chief complaint of back pain and loss of consciousness. He had been diagnosed with GPA on the basis of the presence of inflammatory nasopharyngeal lesions and glomerulonephritis 22 years ago. At the time, histopathologic examination of a biopsy specimen from the nasal cavity had shown necrotizing giant-cell granulomatous inflammation and a necrotizing granulomatous vasculitis. A serum sample was positive by indirect immunofluorescence for anti-neutrophil cytoplasmic antibodies (×128) (i.e., anti-neutrophil cytoplasmic antibodies were detected in 128 times the diluted serum sample). With the exception of three recurrences, the GPA had remained in remission with treatment with prednisolone (15mg/day). At this latest admission, the patient had chest pain on physical examination. Laboratory tests showed the following: leukocyte count, 15.15 × 109/L; hemoglobin, 10.8mmol/L; C-reactive protein, 1.36mg/dL; and stable serum creatinine, 1.31mg/dL. A chest X-ray showed lateral shift of the left lung (Figure 1A). A thoracic computed tomography (CT) scan showed enlargement of the wall of the thoracic aorta, which was surrounded by a hypo-dense rim, and rupture of a dissected aortic aneurysm (Figure 1B and 1C). The patient underwent emergency surgery. Rupture of a 70mm length of the distal arch of the aorta was confirmed during surgery, and a J-graft was inserted. Culture for syphilis was negative. The aortic aneurysm was embedded in an area of tissue inflammation. Histopathologic examination showed a necrotizing inflammation of the aortic wall and a necrotizing granulomatous vasculitis (Figure 2). The diagnosis of aortic rupture as a complication of GPA was made on pathological findings. Postoperatively, the patient recovered uneventfully and was discharged on the 21st postoperative day. Treatment was continued and consisted of prednisolone (15mg/day). After 6 months, a positron emission tomography scan showed no new signs of vascular inflammation or other aneurysm.


Aortic aneurysm rupture as a rare complication of granulomatosis with polyangiitis: a case report.

Ohta N, Waki T, Fukase S, Suzuki Y, Kurakami K, Aoyagi M, Kakehata S - J Med Case Rep (2013)

Histpathological findings of aortic aneurysm from patient with GPA. (A) Histologic section showing considerable arteriosclerosis in the intima and media, with cholesterol clefts. There is intimal thickening with reduplication of the internal elastic lamina. The media is thickened, and the medial smooth muscle cells are replaced by pink hyaline material (hematoxylin and eosin, original magnification ×100). (B) Arteritis with giant-cell formation and lymphocyte and plasma cell infiltration around the vasa vasorum in the media is evident. The aortic media shows patchy infiltration by lymphocytes (plasma cells), with early disruption of the media (hematoxylin and eosin, original magnification ×270).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3750224&req=5

Figure 2: Histpathological findings of aortic aneurysm from patient with GPA. (A) Histologic section showing considerable arteriosclerosis in the intima and media, with cholesterol clefts. There is intimal thickening with reduplication of the internal elastic lamina. The media is thickened, and the medial smooth muscle cells are replaced by pink hyaline material (hematoxylin and eosin, original magnification ×100). (B) Arteritis with giant-cell formation and lymphocyte and plasma cell infiltration around the vasa vasorum in the media is evident. The aortic media shows patchy infiltration by lymphocytes (plasma cells), with early disruption of the media (hematoxylin and eosin, original magnification ×270).
Mentions: A 38-year-old Japanese man was admitted to our hospital with the chief complaint of back pain and loss of consciousness. He had been diagnosed with GPA on the basis of the presence of inflammatory nasopharyngeal lesions and glomerulonephritis 22 years ago. At the time, histopathologic examination of a biopsy specimen from the nasal cavity had shown necrotizing giant-cell granulomatous inflammation and a necrotizing granulomatous vasculitis. A serum sample was positive by indirect immunofluorescence for anti-neutrophil cytoplasmic antibodies (×128) (i.e., anti-neutrophil cytoplasmic antibodies were detected in 128 times the diluted serum sample). With the exception of three recurrences, the GPA had remained in remission with treatment with prednisolone (15mg/day). At this latest admission, the patient had chest pain on physical examination. Laboratory tests showed the following: leukocyte count, 15.15 × 109/L; hemoglobin, 10.8mmol/L; C-reactive protein, 1.36mg/dL; and stable serum creatinine, 1.31mg/dL. A chest X-ray showed lateral shift of the left lung (Figure 1A). A thoracic computed tomography (CT) scan showed enlargement of the wall of the thoracic aorta, which was surrounded by a hypo-dense rim, and rupture of a dissected aortic aneurysm (Figure 1B and 1C). The patient underwent emergency surgery. Rupture of a 70mm length of the distal arch of the aorta was confirmed during surgery, and a J-graft was inserted. Culture for syphilis was negative. The aortic aneurysm was embedded in an area of tissue inflammation. Histopathologic examination showed a necrotizing inflammation of the aortic wall and a necrotizing granulomatous vasculitis (Figure 2). The diagnosis of aortic rupture as a complication of GPA was made on pathological findings. Postoperatively, the patient recovered uneventfully and was discharged on the 21st postoperative day. Treatment was continued and consisted of prednisolone (15mg/day). After 6 months, a positron emission tomography scan showed no new signs of vascular inflammation or other aneurysm.

Bottom Line: As far as we know this is the first reported case of successful treatment in a patient with granulomatosis with polyangiitis complicated with aortic aneurysm rupture.The patient was operated on and a J-graft was inserted.He recovered uneventfully.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Otolaryngology, Yamagata University Faculty of Medicine, 2-2-2 Iida-nishi, Yamagata, 990-23, Japan. noohta@med.id.yamagata-u.ac.jp.

ABSTRACT

Introduction: Granulomatosis with polyangiitis is characterized by systemic inflammation of medium and small blood vessels. Aortic involvement in granulomatosis with polyangiitis is extremely rare. As far as we know this is the first reported case of successful treatment in a patient with granulomatosis with polyangiitis complicated with aortic aneurysm rupture.

Case presentation: We describe a case of granulomatosis with polyangiitis in a 38-year-old Japanese man who developed an aortic aneurysm rupture 22 years after disease onset. The patient was operated on and a J-graft was inserted. He recovered uneventfully.

Conclusion: Recommendations in regard to, and consideration of, aortic involvement should be kept in mind in the long-term careful follow up of granulomatosis with polyangiitis.

No MeSH data available.


Related in: MedlinePlus