Ruptured gastric artery aneurysms in polyarteritis nodosa successfully treated with embolization

Dear editor,

Polyarteritis nodosa (PAN) is a rare form of vasculitis involving small- and medium-sized vessels in multiple organs. Severe gastrointestinal (GI) hemorrhage in PAN due to multiple ruptures of aneurysms of arteries is uncommon and life-threatening. We describe a case of a 61-year-old male patient with PAN who had successful selective embolization of gastric artery aneurysms after presenting with acute abdominal pain. Meanwhile, he was treated with intravenous methylprednisolone and cyclophosphamide pulse therapy. His laboratory and clinical signs were improved, and he obtained clinical remission. We believe that it is necessary for emergency physicians to recognize the manifestation of PAN. Moreover, polyvinylalcohol (PVA) combined with coil embolization is promptly effective and safe in the treatment of ruptured gastric artery aneurysms caused by PAN.

CASE

A 61-year-old male patient presented for recurrent cough with sputum production, headache and fatigue. He was diagnosed with pneumonia before his admission to our hospital and treated with meropenem at 1.5 g/d for 10 d along with thorax puncture and drainage. However, he had no response to the treatment and developed chest pain, mild abdominal pain and myalgia along with a weight loss of 5 kg. Therefore, he visited the emergency department in our hospital. His baseline blood pressure was 122/79 mmHg (1 mmHg=0.133 kPa). Physical examination revealed conjunctival hyperemia, dullness on percussion on both sides of the chest, mild tenderness under the xiphoid process and around the umbilicus, and tenderness of the gastrocnemius. His family history was unremarkable.

In routine clinical investigations, his hematologic test showed white blood cell (WBC) 17.9×10⁹/L, with 95.7% neutrophils and 113 g/L hemoglobin. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were elevated to 237.2 mg/L and 60 mm/h, respectively. Urea nitrogen and creatinine were increased to 21.20 mmol/L and 159 μmol/L, respectively. Perinuclear antineutrophil cytoplasmic antibody (ANCA) was positive, while anti-nuclear antibodies, T-SPOT and all tumor markers were negative. Computed tomography (CT) showed massive bilateral pleural effusion and moderate pericardial effusions and renal and splenic infarction. An angiography was suggested based on his recurrent headaches and chest pains. Magnetic resonance angiography of the head and coronary angiography were normal. Follow-up coeliac angiography and electromyography were suggested, but the patient refused. Based on his clinical manifestations and laboratory tests, systemic vasculitis especially PAN was considered. Intravenous 80 mg methylprednisolone was applied daily. Thereafter, CRP and ESR rapidly decreased to normal levels, and his symptoms improved at that time.

However, 7 d later, he suffered acute abdominal pain with obvious abdominal tenderness. His hemoglobin and blood pressure rapidly decreased to 75 g/L and 98/63 mmHg, respectively. Rehydration and blood transfusion therapy was applied. Contrast-enhanced CT showed massive intraperitoneal bleeding (Figure 1). Percutaneous intraperitoneal angiography revealed ruptured gastric artery aneurysms (Figure 2). Then, emergent arterial embolization using PVA particles combined with coil embolization was performed by interventional radiologists (Figure 3). Fortunately, he had no more bleeding after interventional therapy. Given his history of weight loss greater than 4 kg, myalgia, elevated blood urea nitrogen and creatinine and arteriographic abnormality, the patient fulfilled the American College of Rheumatology (ACR) classification criteria for PAN.[1]

Figure 1.

Contrast-enhanced computed tomography (CT) revealed massive intraperitoneal bleeding (presented as red arrows).

Figure 2.

Digital subtraction abdominal aortogram revealed multiple aneurysms of the gastric artery (presented as red arrows).

Figure 3.

Digital subtraction abdominal aortogram. A: Pre-embolization view revealed the aneurysms (presented as the red arrow) located along the mid-distal portion of the gastric artery. B: Postembolization view revealed the aneurysms had been disappeared (presented as the red arrow), and the gastric artery remained patent.

Intravenous methylprednisolone was continued (80 mg/d for 10 d, then 60 mg/d for 5 d, and gradually reduced to oral methylprednisolone 10 mg/d within 6 months). Meanwhile, pulse cyclophosphamide 0.8 g once per month was applied. At the 6-month follow-up, the patient’s condition had improved significantly, and bilateral pleural and pericardial effusions had disappeared, without recurrent headache or chest and abdominal pain. His serum biochemical abnormalities, CRP and ESR were reduced to normal levels.

DISCUSSION

PAN is a systemic vasculitis affecting small- or medium-sized arteries, with the kidney, peripheral nervous system and skin as the most commonly involved organs.[2] Here, we present a case of PAN with massive GI bleeding due to ruptured gastric artery aneurysms.

PAN is diagnosed according to clinical features, angiography, and histopathology. The ACR proposed the presence of three or more classification criteria being necessary for PAN. Our patient fulfilled 4 of the 10 ACR criteria, with multiple system involvement and gastric artery aneurysms. He suffered acute abdominal pain with a rapid drop in hemoglobin and blood pressure. Ruptured aneurysms were considered and then demonstrated by percutaneous intraperitoneal angiography. Indeed, the prognosis of patients with PAN is related to the severity of visceral involvement. Furthermore, the frequency of aneurysms in PAN is related to clinical severity.[3] As described in a previous study, more than 50% of patients have GI involvement,[4] with common manifestations such as abdominal pain, nausea and diarrhea. There is an incidence of 6% of GI bleeding among PAN patients, which poses immediate threats to life. A ruptured artery aneurysm is a common cause of GI bleeding in PAN patients. A previous literature reported that the mortality rate of ruptured gastric artery aneurysms is 70%.[5] The classic treatment recommended for artery aneurysms is ligation or resection.[6,7] During the past years, therapeutic options have changed for treating ruptured artery aneurysms. Endovascular treatment, particularly coil embolization, has replaced surgical intervention because of its minimally invasive nature. The complications of embolization include thrombosis, organ infarction and pseudoaneurysm formation.[8] Despite the possibility of complications, coil embolization was associated with a shorter hospital stay than surgical treatment and a significant reduction in morbidity and mortality rates of 25% and 67%, respectively.[9,10] This patient suffered from ruptured gastric artery aneurysms, resulting in hemorrhagic shock. Fortunately, PVA combined with coil embolization of the ruptured aneurysm was performed successfully with no complications.

In terms of the severity of PAN with vital organ involvement, previous studies recommend combined treatment with steroids and cyclophosphamide as the classic therapy in PAN;[6] this was effective in our patient. We treated the present patient with prednisone 2 mg/(kg·d), which gradually tapered after clinical remission, as well as cyclophosphamide for 6 months. The patient obtained clinical remission with no recurrence of headache, chest or abdominal pain, and his acute phase reactants remained normal.

CONCLUSIONS

Treating a patient with PAN is a great challenge, as it is a potentially fatal autoimmune disease. This successful case of emergent arterial embolization using PVA combined with coil embolization implies that embolization can be an efficient treatment to treat ruptured artery aneurysm-induced massive bleeding, especially in those with multiple artery aneurysms.