The Journal of Rheumatology
Volume 40, no. 1
Polyarteritis Nodosa ANDREAS P. DIAMANTOPOULOS and PETTER PETTERSEN J Rheumatol 2013;40;87-88 http://www.jrheum.org/content/40/1/87 1. Sign up for our monthly e-table of contents http://www.jrheum.org/cgi/alerts/etoc 2. Information on Subscriptions http://jrheum.com/subscribe.html 3. Have us contact your library about access options [email protected] 4. Information on permissions/orders of reprints http://jrheum.com/reprints.html The Journal of Rheumatology is a monthly international serial edited by Earl D. Silverman featuring research articles on clinical subjects from scientists working in rheumatology and related fields.
Downloaded from www.jrheum.org on October 18, 2015 - Published by The Journal of Rheumatology
Images in Rheumatology
Polyarteritis Nodosa
ANDREAS P. DIAMANTOPOULOS, MD, Department of Rheumatology; PETTER PETTERSEN, MD, Department of Radiology, Hospital of Southern Norway HF, Kristiansand, Norway. Address correspondence to Dr. Diamantopoulos, Department of Rheumatology, Hospital of Southern Norway HF, Kristiansand Service Box 416, 4604 Kristiansand, Norway. E-mail: [email protected]. J Rheumatol 2013;40:87–8; doi:10.3899/jrheum.121036
Polyarteritis nodosa (PAN) is a rare form of vasculitis affecting the medium-size arteries1,2. Even with minimal clinical symptoms, presentation can be dramatic when seen with advanced imaging techniques that show aneurysm rupture and hemorrhage. A 59-year-old man was admitted to the emergency department with abdominal pain. A C-reactive protein 160 mg/dl, hemoglobin 5.1 g/dl, and blood pressure 153/84 mm Hg were noted. No arthralgias/myalgias, neuropathies, or fever were observed. He had an episode of knee arthritis 1 month before admission. A computed tomography (CT)
scan of the abdomen revealed intraabdominal fluid and rupture of the liver (Figure 1, arrows). An angiogram of the right hepatic artery revealed multiple aneurysms (the largest showed signs of active bleeding; Figure 2, arrow) and stenoses. A successful embolization was performed, isolating the large aneurysm from the circulation and stopping the bleeding. The patient was diagnosed with PAN1,2. The typical aneurysms and stenoses in conventional angiography are the hallmarks of PAN3. Our patient was initially treated with 1 g of methylprednisolone intravenously (IV) in 3 subsequent
Figure 1. Computed tomography of the abdomen, coronal view. Intraabdominal fluid and rupture of the liver is visible (arrows).
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2013. All rights reserved.
Diamantopoulos and Pettersen: Polyarteritis nodosa
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87
Figure 2. Angiography of the hepatic artery. Microaneurysms and stenoses are visible. The largest aneurysm shows signs of active bleeding (arrow).
days, followed by 80 mg of oral prednisolone daily and monthly IV cyclophosphamide pulses of 700 mg/m3. A rapid improvement of clinical signs and inflammation was noted after the initiation of treatment. Sixteen months later, the patient continued to be in remission with 150 mg/day of azathioprine and 5 mg/day of prednisolone. The patient has not been re-evaluated with conventional angiography; however, a CT angiography of the abdomen performed after 16 months did not reveal any signs of liver or medium-sized vessel pathology. The noteworthy aspect of our case is the dramatic and fulminant presentation of PAN with aneurysm rupture and abdominal hemorrhage, accompanied by minimal clinical symptoms.
88
REFERENCES
1. Cohen RD, Conn DL, Ilstrup DM. Clinical features, prognosis, and response to treatment in polyarteritis. Mayo Clin Proc 1980; 55:146-55. 2. Haugeberg G, Bie R, Bendvold A, Larsen AS, Johnsen V. Primary vasculitis in a Norwegian community hospital: a retrospective study. Clin Rheumatol 1998;17:364-8. 3. Ewald EA, Griffin D, McCune WJ. Correlation of angiographic abnormalities with disease manifestations and disease severity in polyarteritis nodosa. J Rheumatol 1987;14:952-6.
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2013. All rights reserved.
The Journal of Rheumatology 2013; 40:1; doi:10.3899/jrheum.121036
Downloaded from www.jrheum.org on October 18, 2015 - Published by The Journal of Rheumatology
Volume 40, no. 1
Polyarteritis Nodosa ANDREAS P. DIAMANTOPOULOS and PETTER PETTERSEN J Rheumatol 2013;40;87-88 http://www.jrheum.org/content/40/1/87 1. Sign up for our monthly e-table of contents http://www.jrheum.org/cgi/alerts/etoc 2. Information on Subscriptions http://jrheum.com/subscribe.html 3. Have us contact your library about access options [email protected] 4. Information on permissions/orders of reprints http://jrheum.com/reprints.html The Journal of Rheumatology is a monthly international serial edited by Earl D. Silverman featuring research articles on clinical subjects from scientists working in rheumatology and related fields.
Downloaded from www.jrheum.org on October 18, 2015 - Published by The Journal of Rheumatology
Images in Rheumatology
Polyarteritis Nodosa
ANDREAS P. DIAMANTOPOULOS, MD, Department of Rheumatology; PETTER PETTERSEN, MD, Department of Radiology, Hospital of Southern Norway HF, Kristiansand, Norway. Address correspondence to Dr. Diamantopoulos, Department of Rheumatology, Hospital of Southern Norway HF, Kristiansand Service Box 416, 4604 Kristiansand, Norway. E-mail: [email protected]. J Rheumatol 2013;40:87–8; doi:10.3899/jrheum.121036
Polyarteritis nodosa (PAN) is a rare form of vasculitis affecting the medium-size arteries1,2. Even with minimal clinical symptoms, presentation can be dramatic when seen with advanced imaging techniques that show aneurysm rupture and hemorrhage. A 59-year-old man was admitted to the emergency department with abdominal pain. A C-reactive protein 160 mg/dl, hemoglobin 5.1 g/dl, and blood pressure 153/84 mm Hg were noted. No arthralgias/myalgias, neuropathies, or fever were observed. He had an episode of knee arthritis 1 month before admission. A computed tomography (CT)
scan of the abdomen revealed intraabdominal fluid and rupture of the liver (Figure 1, arrows). An angiogram of the right hepatic artery revealed multiple aneurysms (the largest showed signs of active bleeding; Figure 2, arrow) and stenoses. A successful embolization was performed, isolating the large aneurysm from the circulation and stopping the bleeding. The patient was diagnosed with PAN1,2. The typical aneurysms and stenoses in conventional angiography are the hallmarks of PAN3. Our patient was initially treated with 1 g of methylprednisolone intravenously (IV) in 3 subsequent
Figure 1. Computed tomography of the abdomen, coronal view. Intraabdominal fluid and rupture of the liver is visible (arrows).
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2013. All rights reserved.
Diamantopoulos and Pettersen: Polyarteritis nodosa
Downloaded from www.jrheum.org on October 18, 2015 - Published by The Journal of Rheumatology
87
Figure 2. Angiography of the hepatic artery. Microaneurysms and stenoses are visible. The largest aneurysm shows signs of active bleeding (arrow).
days, followed by 80 mg of oral prednisolone daily and monthly IV cyclophosphamide pulses of 700 mg/m3. A rapid improvement of clinical signs and inflammation was noted after the initiation of treatment. Sixteen months later, the patient continued to be in remission with 150 mg/day of azathioprine and 5 mg/day of prednisolone. The patient has not been re-evaluated with conventional angiography; however, a CT angiography of the abdomen performed after 16 months did not reveal any signs of liver or medium-sized vessel pathology. The noteworthy aspect of our case is the dramatic and fulminant presentation of PAN with aneurysm rupture and abdominal hemorrhage, accompanied by minimal clinical symptoms.
88
REFERENCES
1. Cohen RD, Conn DL, Ilstrup DM. Clinical features, prognosis, and response to treatment in polyarteritis. Mayo Clin Proc 1980; 55:146-55. 2. Haugeberg G, Bie R, Bendvold A, Larsen AS, Johnsen V. Primary vasculitis in a Norwegian community hospital: a retrospective study. Clin Rheumatol 1998;17:364-8. 3. Ewald EA, Griffin D, McCune WJ. Correlation of angiographic abnormalities with disease manifestations and disease severity in polyarteritis nodosa. J Rheumatol 1987;14:952-6.
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2013. All rights reserved.
The Journal of Rheumatology 2013; 40:1; doi:10.3899/jrheum.121036
Downloaded from www.jrheum.org on October 18, 2015 - Published by The Journal of Rheumatology
