Ruptured hepatic aneurysm as first presenting symptom of polyarteritis nodosa

Ruptured hepatic aneurysm as first presenting symptom of polyarteritis nodosa Polyarteritis nodosa (PAN) is an inflammatory vasculitis that creates regions of stenosis and aneurysm formation. The authors describe a 66-year-old female with hepatic artery rupture as the first presentation of undiagnosed PAN, presenting with abdominal pain followed by hemorrhagic shock. This aneurysm was suture ligated with a successful outcome. A mesenteric arteriogram demonstrated lesions consistent with PAN including aneurysms of the left gastric branches, right and left hepatic arteries, and beaded appearance of the iliac artery. However, she developed massive pulmonary embolism from which she did not recover after discharge. Postmortem examination confirmed left hepatic artery aneurysm rupture and changes consistent with PAN on gross anatomical examination and histology. This report provides a unique overview of the disease process through imaging, gross anatomic specimen and pathology. Life- threatening hepatic artery aneurysm rupture is an uncommon presentation of PAN which may benefit readers in creating a more robust differential diagnosis. aspartate aminotransferase of 208 U/L (nl 15–37 U/L) and total INTRODUCTION bilirubin of 2.3 mg/dL (nl 0.2–1.0 mg/dL). Performance of an Polyarteritis nodosa (PAN) is a systemic inflammatory vasculitis abdominal ultrasound was attempted but not completed as the that causes intimal proliferation and elastic lamina disruption. patient became hypotensive, tachycardic and diaphoretic. This multifocal disruption of the vessel results in aneurysm After fluid resuscitation and hemodynamic stabilization, IV formation alternating with stenosis creating a characteristic contrast-enhanced CT revealed a large fluid collection consist- ‘rosary sign’ on imaging. Spontaneous aneurysm rupture is rare ent with hemoperitoneum (Fig. 1a). A bright area of contrast and can cause life-threatening hemorrhage. Prompt diagnosis near the left hepatic artery was noted abutting the lesser curva- and appropriate intervention is paramount for survival in PAN ture of the stomach with no active extravasation (Fig. 1b). She aneurysm ruptures. again became vitally unstable with a significant drop in hem- atocrit to 19% (nl 37–47%). Massive transfusion protocol was initiated and the patient was taken to the operating room for CASE REPORT exploratory laparotomy rather than interventional radiology A 66-year old female with hypertension, cervical dysplasia and due to hemodynamic instability and a clinically acute surgical stage II breast cancer presented with sudden right upper quad- abdomen. rant abdominal pain associated with nausea, vomiting and Upon entering the abdomen, 2500 mL of blood was evacu- anorexia. Initial laboratory tests showed a hematocrit of 33.9% ated from the peritoneal space with an additional 400 mL (nl 37–47%), alanine aminotransferase of 518 U/L (nl 13–61 U/L), hematoma abutting the lesser curvature of the stomach. Upon Received: August 30, 2017. Revised: October 11, 2017. Accepted: November 30, 2017 © The Author(s) 2018. Published by Oxford University Press. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Ruptured hepatic aneurysm as first presenting symptom 65 Figure 1: (a) A contrast-enhanced CT of the abdomen shows a large fluid collec- tion in the region of the left upper quadrant, abutting the lesser curvature of the stomach. Questionable active extravasation of contrast medium (a, white arrow) in the region of the left hepatic artery with shoddy celiac axis noted (c). The CT numbers range from 130 to 200 Hounsfield units in the area, consistent with possible contrast material. exploration of the hematoma, irregular vascular anatomy was noted, bleeding from the left hepatic artery was identified, Figure 2: (a and b) Above: Mesenteric angiography showing aberrant anatomy suture ligation with distal and proximal control performed, and as well as numerous visceral aneurysms including the right and left hepatic, a drain placed. and left gastric branches, without active contrast extravasation. (c) A contrasted IR mesenteric angiography was obtained and revealed a tor- angiogram examination of the right external iliac and femoral arteries showing a beaded appearance of the iliac and normal femoral artery. tuous and aneurysmal takeoff of the celiac trunk (Fig. 2a), Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018 66 M. Roberto et al. Figure 3: (a–d) Above: Near saddle pulmonary embolism (a, yellow arrow). Aberrant arterial anatomy with hepatic artery originating from the SMA. Left hepatic artery with suture ligation and thrombosis (b). Left hepatic artery aneurysm (b, white circle). Full thickness inflammatory necrosis with a pseudoaneurysm that had dis- sected (c). Full thickness inflammatory necrosis of the left hepatic artery consistent with polyarteritis nodosa (d). aberrant anatomy with a replaced right hepatic artery, splenic DISCUSSION artery arising from the SMA and multiple mesenteric aneur- PAN is a systemic transmural inflammatory vasculitis that ysms including the intrahepatic right hepatic artery, left gastric affects medium-sized arteries [1, 2]. Inflammation of the vessel branches and left hepatic artery (Fig. 2b) raising suspicion for wall and intimal proliferation creates luminal narrowing which PAN. Contrast examination of the right external iliac and com- can lead to stenosis and insufficiency. The same inflammatory mon femoral arteries demonstrated an abnormal beaded process causes disruption of the elastic lamina leading to appearance of the iliac artery (Fig. 2c). There was no active aneurysm formation and possible spontaneous rupture with extravasation identified. life-threatening bleeding. Multifocal segments of stenosis and Prophylactic subcutaneous heparin was initiated on post- aneurysm formation are characteristically identified as a ‘ros- operative Day 3 since the acute bleed was directly visualized ary sign’ or ‘beads on a string’ as seen in this patient in Fig. 2c. and suture ligated in the operating room with IR angiography Unlike other vasculitides, PAN does not involve small arteries and CT imaging demonstrating no extravasation. She remained or veins, and is not associated with anti-neutrophil cytoplasmic stable on vital signs and serial hematocrits and was discharged antibodies [3]. on Day 10. Plans were made for outpatient autoimmune and Inclusion of three of the following criteria has been found to vasculitis workup as an outpatient following discharge. The have a sensitivity and specificity of 82 and 87%, respectively for patient returned to the emergency department on post- the classification of PAN: unexplained weight loss, livedo reti- operative Day 12 after a syncopal episode associated with chest cularis, testicular pain, myalgias, neuropathy, new-onset dia- pain. The patient lost vitals, underwent advanced cardiovascu- stolic blood pressure elevation, elevated blood urea nitrogen, lar life support, and subsequently expired. Postmortem autopsy hepatitis B infection, ateriographic abnormalities and biopsy of revealed a massive pulmonary embolism and anomalous arteries containing polymorphonuclear cells [4]. Our patient abdominal visceral vasculature (Fig. 3b). On microscopic exam- endorsed a 25 pound weight loss, myalgia of bilateral lower ination, transmural inflammation with associated necrosis and extremities, neuropathy of the left proximal thigh, and was dissected pseudoaneurysm was found in the left hepatic artery found to have arteriographic abnormalities as demonstrated which was sutured and thrombosed (Fig. 3c and d). These find- (Fig. 2a–c). Autopsy yielded transmural inflammatory changes ings were consistent with a diagnosis of PAN. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Ruptured hepatic aneurysm as first presenting symptom 67 of medium arteries and regions of stenosis and aneurysm CONFLICT OF INTEREST STATEMENT formation consistent with PAN (Fig. 3b–d). None declared. Medical management of PAN is stratified by severity of dis- ease with the goal of active disease remission and control of arterial disease and organ damage progression. Patients with FUNDING mild disease manifest constitutional symptoms with no signifi- No sources of funding. cant end-organ damage or life-threatening conditions. Initial monotherapy with oral glucocorticoids is followed by dose tapering once clinical improvement is noted. Moderate to ETHICAL APPROVAL severe disease is defined by serious manifestations such as any Aside from informed patient consent, ethical approval was not degree of renal insufficiency, hypertension secondary to vasculitis, required for this case report. symptomatic arterial stenosis, aneurysms or ischemic disease. This may be treated with high-dose glucocorticoids (intravenous in life-threatening disease) and a second immunosuppressive CONSENT drug, such as cyclophosphamide. Once in remission, cyclo- Written informed consent was obtained directly from the phosphamide may be transitioned to azathioprine or metho- patient for publication of this case report prior to her passing. trexatefor maintenancedue to itstoxicityprofile. Arterial perforation in the setting of PAN is emergent and almost certainly fatal. Travers et al. described 17 patients with a GUARANTOR clinical diagnosis of PAN in which 35% developed mesenteric Shinban Liu is the guarantor for this case report. perforation and hemorrhage. Despite interventional efforts, there was 100% mortality [5]. These results were mirrored in a similar study where 30% of PAN patients developed an acute REFERENCES abdomen with up to 100% mortality [6]. With acutely ruptured 1. Balow JE. Renal vasculitis. Kidney Int 1985;27:954. aneurysms, prompt diagnosis, resuscitation and hemostasis 2. Sato O, Cohn DL. Polyarteritis and microscopic polyangiitis. through transarterial embolization or surgery is paramount for In: Klippel JH, Dieppe PA, eds. Rheumatology. St Louis: Mosby, patient survival. Patients with known PAN should be placed on 2003, 1290–99. a regimen of glucocorticoids in addition to an immunosuppres- 3. Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW. Anti- sant depending on the severity of disease for disease remission neutrophil cytoplasmic antibodies: current diagnostic and and should receive routine evaluation to monitor disease pro- pathophysiological potential. Kidney Int 1994;46:1. gression. Aneurysms identified on imaging may benefit from 4. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, prophylactic stenting to avoid rupture. Aneurysmal rupture McShane DJ, et al. The American College of Rheumatology and acute bleed should be considered in a robust differential 1990 criteria for the classification of polyarteritisnodosa. diagnosis in patients with PAN presenting with hemodynamic Arthritis Rheum 1990;33:1088. instability and abdominal pain to aid in expedited treatment. 5. Travers RL, Allison DJ, Brettie RP, Hughes GR. Polyarteritisnodosa: a clinical and angiographic analysis of 17 cases. Semin Arthritis ACKNOWLEDGEMENTS Rheum 1979;8:184–99. The researchers would like to thank the patient described for 6. Levine SM, Hellmann DB, Stone JH. Gastrointestinal involve- her willingness to participate in this research endeavor prior to ment in polyarteritisnodosa (1986–2000): presentation and her passing. outcomes in 24 patients. Am J Med 2002;112:386. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Oxford Medical Case Reports Oxford University Press

Ruptured hepatic aneurysm as first presenting symptom of polyarteritis nodosa

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Abstract

Polyarteritis nodosa (PAN) is an inflammatory vasculitis that creates regions of stenosis and aneurysm formation. The authors describe a 66-year-old female with hepatic artery rupture as the first presentation of undiagnosed PAN, presenting with abdominal pain followed by hemorrhagic shock. This aneurysm was suture ligated with a successful outcome. A mesenteric arteriogram demonstrated lesions consistent with PAN including aneurysms of the left gastric branches, right and left hepatic arteries, and beaded appearance of the iliac artery. However, she developed massive pulmonary embolism from which she did not recover after discharge. Postmortem examination confirmed left hepatic artery aneurysm rupture and changes consistent with PAN on gross anatomical examination and histology. This report provides a unique overview of the disease process through imaging, gross anatomic specimen and pathology. Life- threatening hepatic artery aneurysm rupture is an uncommon presentation of PAN which may benefit readers in creating a more robust differential diagnosis. aspartate aminotransferase of 208 U/L (nl 15–37 U/L) and total INTRODUCTION bilirubin of 2.3 mg/dL (nl 0.2–1.0 mg/dL). Performance of an Polyarteritis nodosa (PAN) is a systemic inflammatory vasculitis abdominal ultrasound was attempted but not completed as the that causes intimal proliferation and elastic lamina disruption. patient became hypotensive, tachycardic and diaphoretic. This multifocal disruption of the vessel results in aneurysm After fluid resuscitation and hemodynamic stabilization, IV formation alternating with stenosis creating a characteristic contrast-enhanced CT revealed a large fluid collection consist- ‘rosary sign’ on imaging. Spontaneous aneurysm rupture is rare ent with hemoperitoneum (Fig. 1a). A bright area of contrast and can cause life-threatening hemorrhage. Prompt diagnosis near the left hepatic artery was noted abutting the lesser curva- and appropriate intervention is paramount for survival in PAN ture of the stomach with no active extravasation (Fig. 1b). She aneurysm ruptures. again became vitally unstable with a significant drop in hem- atocrit to 19% (nl 37–47%). Massive transfusion protocol was initiated and the patient was taken to the operating room for CASE REPORT exploratory laparotomy rather than interventional radiology A 66-year old female with hypertension, cervical dysplasia and due to hemodynamic instability and a clinically acute surgical stage II breast cancer presented with sudden right upper quad- abdomen. rant abdominal pain associated with nausea, vomiting and Upon entering the abdomen, 2500 mL of blood was evacu- anorexia. Initial laboratory tests showed a hematocrit of 33.9% ated from the peritoneal space with an additional 400 mL (nl 37–47%), alanine aminotransferase of 518 U/L (nl 13–61 U/L), hematoma abutting the lesser curvature of the stomach. Upon Received: August 30, 2017. Revised: October 11, 2017. Accepted: November 30, 2017 © The Author(s) 2018. Published by Oxford University Press. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Ruptured hepatic aneurysm as first presenting symptom 65 Figure 1: (a) A contrast-enhanced CT of the abdomen shows a large fluid collec- tion in the region of the left upper quadrant, abutting the lesser curvature of the stomach. Questionable active extravasation of contrast medium (a, white arrow) in the region of the left hepatic artery with shoddy celiac axis noted (c). The CT numbers range from 130 to 200 Hounsfield units in the area, consistent with possible contrast material. exploration of the hematoma, irregular vascular anatomy was noted, bleeding from the left hepatic artery was identified, Figure 2: (a and b) Above: Mesenteric angiography showing aberrant anatomy suture ligation with distal and proximal control performed, and as well as numerous visceral aneurysms including the right and left hepatic, a drain placed. and left gastric branches, without active contrast extravasation. (c) A contrasted IR mesenteric angiography was obtained and revealed a tor- angiogram examination of the right external iliac and femoral arteries showing a beaded appearance of the iliac and normal femoral artery. tuous and aneurysmal takeoff of the celiac trunk (Fig. 2a), Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018 66 M. Roberto et al. Figure 3: (a–d) Above: Near saddle pulmonary embolism (a, yellow arrow). Aberrant arterial anatomy with hepatic artery originating from the SMA. Left hepatic artery with suture ligation and thrombosis (b). Left hepatic artery aneurysm (b, white circle). Full thickness inflammatory necrosis with a pseudoaneurysm that had dis- sected (c). Full thickness inflammatory necrosis of the left hepatic artery consistent with polyarteritis nodosa (d). aberrant anatomy with a replaced right hepatic artery, splenic DISCUSSION artery arising from the SMA and multiple mesenteric aneur- PAN is a systemic transmural inflammatory vasculitis that ysms including the intrahepatic right hepatic artery, left gastric affects medium-sized arteries [1, 2]. Inflammation of the vessel branches and left hepatic artery (Fig. 2b) raising suspicion for wall and intimal proliferation creates luminal narrowing which PAN. Contrast examination of the right external iliac and com- can lead to stenosis and insufficiency. The same inflammatory mon femoral arteries demonstrated an abnormal beaded process causes disruption of the elastic lamina leading to appearance of the iliac artery (Fig. 2c). There was no active aneurysm formation and possible spontaneous rupture with extravasation identified. life-threatening bleeding. Multifocal segments of stenosis and Prophylactic subcutaneous heparin was initiated on post- aneurysm formation are characteristically identified as a ‘ros- operative Day 3 since the acute bleed was directly visualized ary sign’ or ‘beads on a string’ as seen in this patient in Fig. 2c. and suture ligated in the operating room with IR angiography Unlike other vasculitides, PAN does not involve small arteries and CT imaging demonstrating no extravasation. She remained or veins, and is not associated with anti-neutrophil cytoplasmic stable on vital signs and serial hematocrits and was discharged antibodies [3]. on Day 10. Plans were made for outpatient autoimmune and Inclusion of three of the following criteria has been found to vasculitis workup as an outpatient following discharge. The have a sensitivity and specificity of 82 and 87%, respectively for patient returned to the emergency department on post- the classification of PAN: unexplained weight loss, livedo reti- operative Day 12 after a syncopal episode associated with chest cularis, testicular pain, myalgias, neuropathy, new-onset dia- pain. The patient lost vitals, underwent advanced cardiovascu- stolic blood pressure elevation, elevated blood urea nitrogen, lar life support, and subsequently expired. Postmortem autopsy hepatitis B infection, ateriographic abnormalities and biopsy of revealed a massive pulmonary embolism and anomalous arteries containing polymorphonuclear cells [4]. Our patient abdominal visceral vasculature (Fig. 3b). On microscopic exam- endorsed a 25 pound weight loss, myalgia of bilateral lower ination, transmural inflammation with associated necrosis and extremities, neuropathy of the left proximal thigh, and was dissected pseudoaneurysm was found in the left hepatic artery found to have arteriographic abnormalities as demonstrated which was sutured and thrombosed (Fig. 3c and d). These find- (Fig. 2a–c). Autopsy yielded transmural inflammatory changes ings were consistent with a diagnosis of PAN. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Ruptured hepatic aneurysm as first presenting symptom 67 of medium arteries and regions of stenosis and aneurysm CONFLICT OF INTEREST STATEMENT formation consistent with PAN (Fig. 3b–d). None declared. Medical management of PAN is stratified by severity of dis- ease with the goal of active disease remission and control of arterial disease and organ damage progression. Patients with FUNDING mild disease manifest constitutional symptoms with no signifi- No sources of funding. cant end-organ damage or life-threatening conditions. Initial monotherapy with oral glucocorticoids is followed by dose tapering once clinical improvement is noted. Moderate to ETHICAL APPROVAL severe disease is defined by serious manifestations such as any Aside from informed patient consent, ethical approval was not degree of renal insufficiency, hypertension secondary to vasculitis, required for this case report. symptomatic arterial stenosis, aneurysms or ischemic disease. This may be treated with high-dose glucocorticoids (intravenous in life-threatening disease) and a second immunosuppressive CONSENT drug, such as cyclophosphamide. Once in remission, cyclo- Written informed consent was obtained directly from the phosphamide may be transitioned to azathioprine or metho- patient for publication of this case report prior to her passing. trexatefor maintenancedue to itstoxicityprofile. Arterial perforation in the setting of PAN is emergent and almost certainly fatal. Travers et al. described 17 patients with a GUARANTOR clinical diagnosis of PAN in which 35% developed mesenteric Shinban Liu is the guarantor for this case report. perforation and hemorrhage. Despite interventional efforts, there was 100% mortality [5]. These results were mirrored in a similar study where 30% of PAN patients developed an acute REFERENCES abdomen with up to 100% mortality [6]. With acutely ruptured 1. Balow JE. Renal vasculitis. Kidney Int 1985;27:954. aneurysms, prompt diagnosis, resuscitation and hemostasis 2. Sato O, Cohn DL. Polyarteritis and microscopic polyangiitis. through transarterial embolization or surgery is paramount for In: Klippel JH, Dieppe PA, eds. Rheumatology. St Louis: Mosby, patient survival. Patients with known PAN should be placed on 2003, 1290–99. a regimen of glucocorticoids in addition to an immunosuppres- 3. Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW. Anti- sant depending on the severity of disease for disease remission neutrophil cytoplasmic antibodies: current diagnostic and and should receive routine evaluation to monitor disease pro- pathophysiological potential. Kidney Int 1994;46:1. gression. Aneurysms identified on imaging may benefit from 4. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, prophylactic stenting to avoid rupture. Aneurysmal rupture McShane DJ, et al. The American College of Rheumatology and acute bleed should be considered in a robust differential 1990 criteria for the classification of polyarteritisnodosa. diagnosis in patients with PAN presenting with hemodynamic Arthritis Rheum 1990;33:1088. instability and abdominal pain to aid in expedited treatment. 5. Travers RL, Allison DJ, Brettie RP, Hughes GR. Polyarteritisnodosa: a clinical and angiographic analysis of 17 cases. Semin Arthritis ACKNOWLEDGEMENTS Rheum 1979;8:184–99. The researchers would like to thank the patient described for 6. Levine SM, Hellmann DB, Stone JH. Gastrointestinal involve- her willingness to participate in this research endeavor prior to ment in polyarteritisnodosa (1986–2000): presentation and her passing. outcomes in 24 patients. Am J Med 2002;112:386. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/2/omx100/4898993 by Ed 'DeepDyve' Gillespie user on 16 March 2018

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Oxford Medical Case ReportsOxford University Press

Published: Feb 1, 2018

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