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| ORIGINAL ARTICLE |
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| Year : 2021 | Volume
: 8
| Issue : 4 | Page : 341-346 |
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Idiopathic large-vessel vasculitis presenting as acute abdomen and mesenteric ischemia
Natarajan Sekar1, Iyappan Ponnuswamy2
1 Department of Vascular and Endovascular Surgery, Kauvery Hospital, Chennai, Tamil Nadu, India
2 Department of Radiodiagnosis, Kauvery Hospital, Chennai, Tamil Nadu, India
| Date of Submission | 19-Dec-2020 |
| Date of Acceptance | 02-Jan-2021 |
| Date of Web Publication | 9-Dec-2021 |
Correspondence Address:
Natarajan Sekar
Department of Vascular and Endovascular Surgery, Kauvery Hospital, Chennai, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijves.ijves_172_20
Objective: The term “large-vessel vasculitis” encompasses the spectrum of primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches. The two major categories of large-vessel (LV) vasculitis are giant cell arteritis and Takayasu arteritis. LV vasculitis of unknown etiology presenting as mesenteric ischemia is presented here. Materials and Methods: This is a single-center retrospective analysis of 17 patients who presented with mesenteric ischemia over a period of 7 years. Their age ranged from 18 to 69 years. There were five females and the rest were male. The presenting symptoms were abdominal pain, nausea, malaise, and low-grade fever. Peripheral pulses were normal. Computed tomography (CT) aortogram showed typical wall edema and inflammatory tissue encasing the superior mesenteric artery, celiac artery, and the adjacent aorta in all the patients. Thrombosis and localized dissection in the superior mesenteric and celiac arteries was seen in some. Erythrocyte sedimentation rate and C-reactive protein were elevated, but other vasculitis markers were normal. Results: All of them were treated with glucocorticoids with immediate relief from the symptoms. Anticoagulation was given for those with thrombus, stenosis, and dissection. None had any intervention. At 1-year follow-up, there was no recurrence, and all were asymptomatic. Conclusion: Idiopathic LV vasculitis can present as mesenteric ischemia and acute abdomen. CT aortogram typically shows inflammatory tissue around the visceral artery and the aorta. The etiology is still uncertain. However, this condition has many similarities with LV giant cell arteritis (GCA-LV). Hence, all investigations should be done to rule out GCA. They respond very well to glucocorticoids. Correct diagnosis can avoid an unnecessary surgery. Long-term follow-up is required in order not to miss any late complications This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Keywords: Celiac artery, giant cell arteritis, large-vessel vasculitis, mesenteric ischemia, superior mesenteric artery
How to cite this article:
Sekar N, Ponnuswamy I. Idiopathic large-vessel vasculitis presenting as acute abdomen and mesenteric ischemia. Indian J Vasc Endovasc Surg 2021;8:341-6 |
| Introduction | |  |
Giant cell arteritis (GCA) is a chronic, idiopathic, granulomatous vasculitis of medium and large arteries. Commonly, it affects the extracranial branches of the carotid artery usually temporal arteries. It is also known to affect large vessels (LVs) like aorta and its major branches (LV-GCA). Majority of the time, they are asymptomatic, and diagnosis is picked up by routine investigation done for patients with classical GCA. Chronic mesenteric ischemia due to LV-GCA is known. Acute abdominal pain in LV-GCA is usually due to aortic dissection or threatened rupture of aortic aneurysm. LV vasculitis of unknown etiology presenting as acute and subacute mesenteric ischemia is presented here.
| Materials and Methods | | |
After prior approval by our Institutional Review Board, this single-center retrospective analysis of 17 patients presenting with LV vasculitis was conducted.
Seventeen patients were referred to the vascular surgical outpatient in the past 7 years (2013–2019) with acute abdominal pain. Their age varied between 18 and 69 years. The youngest patient was a female. There were four patients in the age group of 30–50 years. The rest were over the age of 50 years. Five were females and the rest males. The clinical presentation is shown in [Table 1].
Headache, polymyalgia, rashes, and jaw or limb claudication were not seen in any patient. Three patients gave a history of eating seafood before the onset of symptoms. Initially, all these patients were seen by physicians and medical gastroenterologists and were treated as gastritis and or food poisoning. When there was no relief in symptoms, computed tomography (CT) of the abdomen with contrast was done and then were referred to the department of vascular surgery. CT aortogram showed periarterial inflammatory edema and soft-tissue reaction encasing and forming a cuff around the aorta, celiac artery, or superior mesenteric artery (SMA) or branches of SMA in almost all the patients [Figure 1]a, [Figure 1]b, [Figure 1]c The other findings were thrombosis, dissection, stenosis, saccular aneurysm of SMA and CA [Figure 2]a and [Figure 2]b and features of mesenteric stranding, bowel edema, and ischemic stricture of the small bowel [Figure 3]a and [Figure 3]b. The CT aortogram findings are listed in [Table 2]. One patient had isolated thrombosis of the celiac artery extending also into the branches [Figure 4]a and [Figure 4]b. She had infarction of tail of the pancreas and spleen, elevated liver enzymes, and gastric mucosal ischemia and needed prolonged hospitalization. One patient presented with subacute intestinal obstruction due to ischemic stricture of the terminal ileum. SMA showed inflammatory cuffing near the origin and thrombosis of the distal ileal branch in this patient. This patient had been symptomatic for over 2 months and had even undergone laparoscopic adhesion release with no relief in symptoms. Two patients underwent 18F-Fluorodeoxyglucose positron emission tomography (18FDG-PET) showing increased uptake of FDG, only in the inflamed aorta, and the rest of the arterial tree did not show any increased up-take. | Figure 1: (a) Fifty-nine-year-old male. Computed tomography aortogram showing inflammatory tissue encasing the superior mesenteric artery. Aorta is normal (b) Computed tomography aortogram showing arteritis involving superior mesenteric artery and its branches (small arrows) luminal irregularity and an ulcer causing saccular projection can be seen (c) 67-year-old male. Computed tomography aortogram showing inflammatory tissue around aorta. Superior mesenteric artery shows dissection and inflammation (arrow)
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 | Figure 2: (a) Celiac artery showing inflammatory wall thickening and an ulcer causing a saccular dilatation (b) Computed tomography aortogram showing 10 mm thick inflammatory wall edema and thickening around the superior mesenteric artery seen as a hypoechoic area around the vessel lumen. Duplex scan showed the typical Halo sign (c) Dissection in the superior mesenteric artery. Dissection is seen extending into the branches of superior mesenteric artery causing narrowing of the Jejunal branches (arrow)
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 | Figure 3: (a) Computed tomography aortogram showing inflammatory cuffing around the superior mesenteric artery and dissection of the jejunal branches causing mesenteric stranding and edema of a segment of Jejunum (Arrow). (b) Computed tomography aortogram showing inflammation involving superior mesenteric artery branches causing mesenteric stranding and severe bowel edema (small arrows)
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 | Figure 4: (a) Forty-six-year-old female Diabetic and hypertensive. Computed tomography aortogram showing isolated celiac artery thrombosis extending into hepatic and splenic arteries. Splenic infarct is seen. (b) Lateral view of the same patient showing thrombosed Celiac artery and normal superior mesenteric artery and aorta
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Three patients had diabetes and two others had hypertension. Fifteen of the 17 patients had elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Those with severe symptoms had very high elevation of ESR and CRP. In two patients, antinuclear antibodies were weakly positive. Anti-neutrophil cytoplasmic antibodies (ANCA) were normal in all. All other markers for vasculitis were normal. Hepatitis B antigen was positive in two patients. Temporal artery biopsy was not done in any of the patients since most of them were young and none had any feature suggestive of GCA. Moreover, Doppler study of axillary brachial and temporal arteries did not show any wall abnormality.
Patients were started on oral glucocorticoids (Prednisolone) and clopidogrel. Initial dose of glucocorticoids given was 0.5–1 mgm/kg/day. Eight patients who had severe symptoms were admitted and started on injection methylprednisolone 500–1000 mg per day for 3 days followed by oral prednisolone. Those with evidence of dissection, arterial thrombus, and bowel edema were started on the therapeutic dose of anticoagulation (low molecular weight heparin) which was changed to oral anticoagulant at discharge and continued for 3 months. The patient with celiac artery thrombosis needed total parenteral nutrition since she had gastric mucosal ischemia and would not tolerate oral feeding. In all patients, symptoms dramatically disappeared in 24–48 h after starting steroids. The high-dose steroid was maintained for 4–6 weeks or till the inflammatory markers returned to normal.
The dose was gradually reduced and was stopped by 6 months. One patient showed elevation of inflammatory markers after stopping steroids, and a repeat CT showed inflammatory cuffing around the aorta had not fully resolved. She has been restarted on oral steroids, although she is completely asymptomatic. Clopidogrel was continued for 1 year in all. Follow-up CT showed a complete resolution of the periarterial inflammatory cuffing in all others. Small saccular aneurysm of SMA seen in one patient resolved completely, and the intima looked normal [Figure 5]a, [Figure 5]b, [Figure 5]c. All patients with bowel edema were closely observed so that bowel gangrene is not missed. In one patient, the dissection in the SMA and celiac arteries stabilized, and the distal flow was maintained [Figure 6]a and [Figure 6]b. He was completely asymptomatic. The patient who had severe inflammation around the entire length of celiac artery and SMA developed stenosis of the celiac and occlusion of the proximal SMA [Figure 7]a, [Figure 7]b, [Figure 7]c. Since he was completely asymptomatic, no intervention was done. None of the patients needed any intervention, and they have remained asymptomatic. All patients were followed up for a minimum period of 1 year after stopping steroids, and none had shown any recurrence of symptoms or ischemic bowel stricture or aneurysmal dilatation. | Figure 5: (a) Superior mesenteric artery dissection and inflammation extending along the branches causing luminal irregularity and narrowing. (b) In the same patient a small saccular aneurysm from superior mesenteric artery (arrow) is seen. Lumen irregularity and stenosis in superior mesenteric artery seen. Aorta is atherosclerotic with ostial narrowing of both superior mesenteric artery and CA (c) Same patient after steroid therapy. Superior mesenteric artery lumen appears smooth, wall thickening has disappeared and the saccular aneurysm has healed.
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 | Figure 6: (a) Computed tomography aortogram showing arteritis involving both superior mesenteric artery and CA. Inflammatory wall edema forming a cuff around both the arteries. There is dissection involving both the superior mesenteric artery and CA (b) The same patient after treatment with steroids. Repeat CT aortogram shows dissection in the superior mesenteric artery and CA is stable and the distal arterial flow is maintained. There is mild aneurysmal dilatation at dissection site. There is stenosis of the celiac artery. Inflammatory wall edema around the artery has completely resolved
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 | Figure 7: (a) Computed tomography aortogram in a 50-year-old male showing inflammatory tissue around the superior mesenteric artery and celiac artery. Narrowing and irregularity of the lumen in both arteries is seen (b) Same patient showing thick inflammatory tissue around celiac artery (>5 mms). (c) Same patient repeat computed tomography aortogram post treatment with steroids and anticoagulation-superior mesenteric artery shows juxta ostial total occlusion and irregular stenosis distally. Celiac artery and inferior mesenteric artery show stenosis of ostium. The meandering artery has dilated well. Patient was completely asymptomatic, and no intervention was done
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| Discussion | | |
The term “LV vasculitis” encompasses the spectrum of primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches. The two major categories of LV vasculitis are GCA also known as temporal arteritis and Takayasu arteritis (TA).
The other common type is the poly arteritis nodosa which mostly affects the small and medium sized vessels. ANCA-associated vasculitis can sometimes involve the aorta and is extremely rare. All these patients tested negative for ANCA.
TA is common in Asia but mainly affects the younger people and is much more common in females. The important diagnostic criteria for TA include arch vessels involvement mainly carotid and subclavian arteries and typical arteriographic findings.[1] In this series, majority of the patients were males and older. They had no clinical or angiographic features typical of TA.
GCA is also common in women and affects those older than 50 years.
Classical GCA affects the extracranial branches of the carotid artery usually temporal artery and typically presents with new onset headache, jaw claudication, nodular tender temporal artery, and systemic illness such as fever, polymyalgia, weight loss, and fatigue. LV GCA can affect the extracranial arteries mainly limb arteries and aorta.
Autopsy studies have shown histological evidence of large-vessel involvement in 80% of cases of cranial GCA.[2],[3] Routine imaging studies of patients with GCA have demonstrated extensive radiographic large-vessel involvement (e.g., aorta and its major branches) in up to 83% of patients, although most of them were asymptomatic.[4],[5],[6],[7]
Patients with LV-GCA show clinically significant stenosis of the subclavian and axillary artery in 10%–15% of patients. Aortic aneurysm and dissection are also more common in them.
There is a 17-fold increase in thoracic aneurysm and 2.4 fold increase of abdominal aneurysm as compared with the general population.[8],[9],[10]
GCA disease classification is based on the 1990 American College of Rheumatology criteria,[11] which primarily based on cranial symptomatology.
They are:
- Age at onset > 50 years
- New onset headache
- Temporal artery nodular and tender
- Elevated ESR
- Temporal artery biopsy.
For diagnosis of GCA 3 out of 5 criteria must be positive.
Unfortunately, LV-GCA cannot fit in these diagnostic criteria since many times patient may have only LV-GCA and not have any features of cranial GCA. Patients with isolated LV-GCA may present only with constitutional symptoms, polymyalgia symptoms, elevated inflammatory markers, or fever of unknown origin. Clinical signs of large-vessel disease may be silent, and only routine examination of all the vascular system will pick up the pulse deficit, vascular bruits, pulse discrepancies, and aortic regurgitation murmur.
Therefore, vascular imaging studies, such as magnetic resonance angiography, CT angiography, 18F-FDG-PET, and Color duplex ultrasonography are often required to confirm a diagnosis of LV-GCA.[12]
The classical signs in imaging include circumferential wall thickening with contrast enhancement, wall edema (Halo sign), vascular stenosis, occlusion, and aneurysmal changes.
The halo sign was explicitly defined as hypoechoic area around the vessel lumen causing a homogeneous wall swelling of at least 1.5 mm at Duplex scan.[4] PET scan shows increased uptake of FDG in the arterial wall indicating inflammatory activity.
Nearly all patients with LV-GCA had vasculitis of at least one epiaortic artery (particularly the carotid, subclavian, and axillary arteries), which were evaluated in all studies. Virtually, all patients with lower limb vasculitis were found in another study to have upper limb vasculitis as well.[4],[5]
In this series, all the patients showed increased wall thickening and edema and inflammatory encasement of aorta, SMA, and celiac arteries. However, the onset was acute, and the disease was localized to the visceral arteries and the adjoining aorta in all the patients. No patient had any cranial symptoms, polymyalgia, limb ischemia, or any significant arterial lesion elsewhere in the body. Two patients who were over the age of 60 years had atherosclerotic changes in the aorta with mild aneurysmal dilatation and osteal narrowing of SMA and celiac artery. Literature search revealed few case reports of GCA causing chronic mesenteric ischemia but none causing acute mesenteric ischemic symptoms.[13],[14] All GCA patients who had presented with acute abdominal symptoms had either aortic dissection or aneurysm with rupture.[15] Isolated SMA and celiac artery dissections have also been documented earlier. However, vasculitis was not considered as the cause in any of them.[16]
Hence, it is difficult to label these patients as GCA-LV. It could be an immunological response to dietary allergent or some viral infection. Three patients gave a history of eating sea food immediately before the onset of symptoms, and they also had loose stools along with acute abdominal pain and one of them had isolated celiac artery thrombosis. All others were completely asymptomatic prior to this, and no etiological factor could be thought of. Primary retroperitoneal fibrosis (RPF) is another condition where there is peri-aortitis. However, RPF will appear like an inflammatory mass in the retroperitoneum involving the aortic bifurcation, iliac vessels, inferior vena cava, and it also encases the ureter. None of these patients had this feature. Laboratory parameters, including ESR and CRP, are commonly used to determine ongoing activity in patients with GCA. Other vasculitis markers are done to rule out other causes of vasculitis. Level of inflammatory markers as well as the arterial thickening in the Duplex and CT scan can be used to assess the response to the treatment. Definitive histopathologic confirmation is often difficult to obtain, because temporal artery biopsy may be normal and aortic and other LV biopsies are often not available, unless there is a vascular calamity requiring aortic surgery.
Glucocorticoids remain the treatment of choice to induce remission in patients with GCA and in those with evidence of large-vessel involvement. Initial doses of 0.75–1.0 mg/kg/day followed by gradual tapering are recommended. Methotrexate has also been used with good results. Ustekinumab, an interleukin (IL)-12/IL-23-blocking monoclonal antibodies, Abatacept, a selective T-cell co-stimulation modulator, Tocilizumab, an IL-6 receptor alpha antagonist have all been used demonstrating superior clinical efficacy and a glucocorticoid-sparing effect in patients with GCA.[6]
Patients with GCA showing intense inflammation in the thoracic aorta (as demonstrated by 18FDG uptake) at the diagnosis appear to be more prone to the subsequent development of aortic dilatation.[17] Hence, long-term follow-up is required in these patients. In this series, all patients responded immediately to glucocorticoids and did not need any other immunosuppressants. One patient in this group who had severe inflammation around both SMA and celiac artery went on to develop total occlusion of that segment of the artery after steroid therapy. However, he remained asymptomatic and did not need any intervention. Similarly, those who had localized dissection in the celiac artery and SMA also improved with steroids and anticoagulation. Surgery or endovascular intervention during the acute inflammatory stage carries a higher rate of failure and should be avoided. It has been well documented that asymptomatic isolated visceral artery dissections resolve spontaneously and only 8%–12% of the symptomatic ones require intervention.[18] Hence, long-term follow-up is needed. There was no relapse at 1-year follow-up in any of these patients.
It is difficult to pin point the etiology of this condition. It could be a variant of the large-vessel GCA or could be an idiopathic variety. Longer follow-up is needed to see if they evolve into GCA or any other type of autoimmune disease.
| Conclusion | | |
Isolated LV arteritis involving SMA and celiac arteries is extremely rare. This condition should be considered when other common causes for acute abdominal pain have been ruled out. This condition is different from LV GCA though there are many similarities. Early diagnosis is important since they respond very well to glucocorticoids, and unnecessary surgery or intervention can be avoided.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
[Table 1], [Table 2]
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