Ischemic duodenal injury due to systemic lupus erythematosus: A case report

Abstract

BACKGROUND

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple organ systems. Lupus enteritis is a rare but potentially life-threatening gastrointestinal manifestation characterized by immune complex-mediated inflammation that may cause edema, ischemia, or intestinal perforation. Because abdominal symptoms often overlap with those of infection or drug toxicity, an accurate diagnosis requires a high index of suspicion supported by characteristic radiologic findings.

CASE SUMMARY

A 47-year-old female with a known history of SLE and membranous glomerulonephritis presented with acute epigastric pain. Contrast-enhanced computed tomography revealed venous-type ischemia involving the second portion of the duodenum and proximal jejunum. Despite intensive medical management, progressive ischemic changes necessitated surgical resection and duodenojejunostomy. Postoperatively, the patient developed pancreatitis and biliary obstruction at the ampulla of Vater, requiring coordinated multidisciplinary care, including rheumatologic evaluation for disease flares, radiology-guided percutaneous transhepatic biliary drainage, internal stent placement, and pathological confirmation of small-vessel vasculitis. Both complications resolved after intervention. Duodenal involvement in lupus enteritis is exceptionally rare, and cases requiring surgical intervention are even rarer. Consequently, only a few cases have been reported.

CONCLUSION

Prompt recognition and multidisciplinary management are essential for favorable outcomes in patients with severe lupus enteritis and duodenal involvement.

Key Words: Lupus enteritis; Systemic lupus erythematosus; Duodenal ischemia; Biliary complications; Duodenojejunostomy; Case report

Core Tip: Duodenal involvement in lupus enteritis is exceptionally rare and may progress to ischemia requiring surgery. Early recognition is critical, as delayed diagnosis can be life-threatening. Because it is often difficult to predict whether lupus enteritis will respond to steroids or instead progress to ischemia, a multidisciplinary approach and timely decision-making are essential. In this case, early duodenal resection and duodenojejunostomy prevented further deterioration, and postoperative biliary obstruction was successfully managed through intervention. This case highlights that in systemic lupus erythematosus patients with acute abdominal symptoms, lupus enteritis should be considered and coordinated management is crucial.

INTRODUCTION

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that can affect virtually any organ system, including the nervous system, skin, joints, heart, kidneys, and gastrointestinal tract. Gastrointestinal symptoms occur in up to 50% of patients with SLE, but are most often related to medications or infection rather than lupus enteritis itself[1]. Lupus enteritis is a rare but potentially life-threatening complication, characterized by immune complex vasculitis and complement activation within mesenteric vessels leading to submucosal edema, hemorrhage, or ischemic necrosis[2,3].

Failure to recognize or treat this condition promptly can result in bowel infarction or perforation. We present a rare case of duodenal lupus enteritis complicated by ischemic necrosis that required surgical resection, followed by postoperative biliary obstruction. Detailed reports of lupus enteritis involving the duodenum, particularly those requiring surgical intervention, remain scarce, and postoperative biliary complications in this setting have seldom been reported. This case highlights the need for timely surgical and immunological interventions.

CASE PRESENTATION

Chief complaints

The patient began to experience epigastric pain the previous day.

History of present illness

A 47-year-old woman presented to the emergency department with sudden worsening epigastric pain that began the previous day and was squeezing in nature. She did not experience nausea, vomiting, or diarrhea.

History of past illness

Her past medical history included SLE and membranous glomerulonephritis diagnosed five years earlier at another hospital; at that time, she had presented with photosensitive cutaneous eruptions. She had not received immunosuppressive therapy since diagnosis and had been managing intermittent skin symptoms only with antihistamines and moisturizers.

Personal and family history

She reported no prior abdominal surgery and no family history of autoimmune or gastrointestinal disease.

Physical examination

On physical examination, marked epigastric tenderness was observed without rebound tenderness or rigidity. No malar or discoid rash was present, but urticarial facial lesions, subconjunctival hemorrhage, palpable purpura on both hands, and petechiae on both lower legs were observed, suggesting active vasculitic involvement (Figure 1).

Figure 1  Facial urticarial lesions and purpura on both lower legs at presentation.

Laboratory examinations

Initial laboratory values were: White blood cell was 7600/μL (normal 4000-10000/μL), C-reactive protein (CRP) was 1.41 mg/dL (normal < 0.5 mg/dL), erythrocyte sedimentation rate (ESR) was 51 mm/hour (normal < 20 mm/hour), fibrin degradation product (FDP) was 17.96 μg/mL (normal 0-2.0 μg/mL), and D-dimer was 5.35 μg/mL (normal 0-0.48 μg/mL). Complement levels were low, with C3 at 52 mg/dL (normal 90-180 mg/dL) and C4 at 7.08 mg/dL (normal 10-40 mg/dL). Anti-dsDNA IgG was negative (0.7 IU/mL; normal < 10 IU/mL). Urinalysis demonstrated marked proteinuria (+4, approximately 1000 mg/dL) and microscopic hematuria [10-19 red blood cells per high-power field (RBC/HPF)]. Taken together, these findings yielded a Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2000 score of 18 points (vasculitis +8, proteinuria +4, hematuria +4, low complement +2), indicating high disease activity. Elevated ESR and SLEDAI with relatively normal CRP suggested active lupus inflammation rather than infection, while markedly increased FDP and D-dimer implied ongoing microthrombus formation due to vasculitis[4,5]. Given these findings and the computed tomography (CT) features of non-occlusive ischemia, the rheumatology team considered lupus enteritis as the primary diagnosis.

Imaging examinations

Abdominal contrast-enhanced CT demonstrated circumferential wall thickening and mucosal hyperenhancement involving approximately 50 cm of small bowel from the second portion of the duodenum to the proximal jejunum, with surrounding mesenteric fluid and engorged mesenteric veins (Figure 2A and B).

Figure 2 The patient's imaging results. A: Preoperative contrast-enhanced computed tomography (CT; transverse view) shows duodenal wall thickening and mesenteric congestion; B: Coronal CT view demonstrates venous engorgement and submucosal edema extending from the duodenum to the jejunum; C: Contrast-enhanced abdominal CT at 8 months postoperatively shows an intact anastomosis and no abnormal biliary dilation.

No occlusive lesion was detected in the superior mesenteric artery or vein, suggesting venous ischemia rather than arterial obstruction. These findings are consistent with previously described radiologic features of lupus enteritis, where venous congestion and submucosal edema predominate[6-8].

FINAL DIAGNOSIS

Considering the patient’s acute deterioration and radiological evidence of bowel ischemia, the surgical team carefully balanced conservative steroid therapy with exploratory laparotomy. Corticosteroids or nonsteroidal anti-inflammatory drugs may obscure peritoneal signs and delay the detection of necrosis; conversely, delayed surgery increases the risk of transmural infarction and sepsis. Assessment of bowel viability in lupus enteritis is challenging because reperfusion may transiently restore color despite persistent microvascular occlusion[9]. Considering the rapid progression of pain and high SLEDAI scores, exploratory laparotomy was performed to evaluate the irreversible ischemic injury.

TREATMENT

Exploration revealed segmental edema and induration from the second portion of the duodenum to the proximal jejunum. The bowel was pink, but thickened and firm, consistent with venous infarction rather than arterial occlusion. Mesenteric fat was edematous with lymphatic-like fluid extending retroperitoneally along the Toldt fascia. After the Kocher maneuver and right colon mobilization, distal transection was performed at the proximal jejunum using a linear stapler (TLC 55-mm stapler, Covidien^®) at the point where the edema subsided. The mesentery was divided using an energy device. The resected jejunal limb was passed posterior to the superior mesenteric vessels and careful dissection was performed near the pancreatic uncinate process to preserve the ampulla of Vater. Proximal transection of the second portion of the duodenum was performed diagonally using a stapler (TLC 75-mm stapler, Covidien^®), sparing a small edematous segment to avoid ampullary injury. A side-to-side duodenojejunostomy was performed with vertical incisions and hand-sewn closures (full-thickness continuous sutures with PDS 4-0, Ethicon^®; and seromuscular continuous Lambert sutures with Pronova 5-0, Ethicon^®; Figure 3).

Figure 3 Surgical illustration. A: Schematic illustration of the intraoperative findings, showing an edematous duodenum with prominent venous congestion; B: Illustration of the duodenojejunostomy after segmental resection.

Grossly, the resected specimen showed transmural edema and localized mucosal hemorrhage without perforation (Figure 4). Histopathological examination later confirmed ischemic necrosis with small-vessel vasculitis and fibrinoid necrosis, which are typical of lupus enteritis[8,10].

Figure 4  Gross specimen shows transmural edema and hemorrhagic-appearing mucosa without perforation.

OUTCOME AND FOLLOW-UP

High-dose intravenous hydrocortisone (100 mg every 12 hours) was initiated on postoperative day (POD) 2, and continued for 6 days until POD 7. The regimen was transitioned to oral methylprednisolone on POD 8 (10 mg twice daily). No additional immunosuppressive agents, such as cyclophosphamide, mycophenolate, or rituximab were administered during hospitalization. The rheumatology, dermatology, neurology, and cardiology teams jointly evaluated other SLE-related complications. The patient’s vital signs remained stable, and the laboratory findings and subsequent interventions are shown in Figure 5.

Figure 5 Postoperative laboratory trends show transient hyperbilirubinemia and subsequent normalization after stent placement. POD: Postoperative day; MRCP: Magnetic resonance cholangiopancreatography; PTGBD: Percutaneous transhepatic gallbladder drainage; PTBD: Percutaneous transhepatic biliary drainage; Bil: Bilirubin; ALT: Alanine aminotransferase; ALP: Alkaline phosphatase.

On POD 1, the serum amylase and lipase levels rose, indicating mild pancreatitis. On POD 2, total and direct bilirubin levels also rose, suggesting possible ampullary edema or obstruction caused by postoperative inflammation. On POD 3, pancreatic enzyme levels began to normalize; however, bilirubin and liver function test results continued to worsen. Magnetic resonance cholangiopancreatography on POD 5 revealed dilation of both the common bile duct and pancreatic duct, while the ampulla of Vater could not be clearly identified because of the stapling line near the duodenojejunal anastomosis.

Initial biliary decompression was accomplished via percutaneous transhepatic gallbladder drainage (PTGBD) because the peripheral ducts were too narrow for direct percutaneous transhepatic biliary drainage (PTBD) access. On POD 12, the PTGBD catheter was converted to a PTBD by advancing the tube through the cystic duct. Gastroduodenoscopy on POD 14 revealed persistent mucosal edema obscuring the ampulla and precluding endoscopic retrograde cholangiopancreatography-guided stenting. Between PODs 14 and 18, PTBD clamping provoked recurrent epigastric pain, vasculitic skin rash, and subconjunctival hemorrhage, which are findings consistent with an SLE flare triggered by cholestasis. During this period, the dose of oral methylprednisolone was temporarily increased to 20 mg twice daily. On POD 19, a metallic stent was successfully deployed through the PTBD tract across the ampulla and duodenojejunal anastomosis to restore bile flow. Laboratory indices normalized thereafter, and the patient was discharged on POD 30 with both the PTBD catheter and the internal stent remaining in place.

During outpatient follow-up, the internal biliary stent was removed endoscopically approximately 6 months postoperatively. Immediate cholangiography performed through the existing PTBD tract confirmed smooth bile drainage without residual obstruction. The PTBD catheter was removed shortly thereafter without complications. Under rheumatologic supervision, the patient was followed every 2 months. Glucocorticoids were tapered to 2 mg daily and hydroxychloroquine (200 mg daily) was initiated as maintenance therapy.

At the 1-year follow-up, laboratory evaluation demonstrated normalized inflammatory markers (ESR = 15 mm/hour; normal < 20 mm/hour), negative anti-dsDNA IgG, and improved complement levels (C3 = 65.4 mg/dL, normal 90-180 mg/dL; C4 = 12.1 mg/dL, normal 10-40 mg/dL). Urinalysis showed only trace proteinuria (10 mg/dL) without hematuria (< 5 RBC/HPF). These findings correspond to an SLEDAI-2000 score of 0-2, which is consistent with a quiescent disease. Contrast-enhanced abdominal CT obtained at 8 months postoperatively (Figure 2C) confirmed complete resolution of biliary dilation without evidence of recurrent inflammation or ischemic complications.

DISCUSSION

Lupus enteritis results from immune complex-mediated vasculitis of the mesenteric vessels, leading to bowel wall edema, venous congestion, and occasionally, ischemic necrosis[2,3]. Duodenal involvement is extremely rare, likely because the proximal intestine has rich collateral circulation from the pancreaticoduodenal arcade[10,11]. Despite its rarity, the clinical, radiologic, and laboratory findings in this case were highly suggestive of lupus enteritis, which was later confirmed by histopathology. The initial differential diagnosis—including thrombotic microangiopathy, antiphospholipid syndrome, and infectious enteritis—was considered unlikely. There was no evidence of hemolysis, thrombocytopenia, or schistocytes to support thrombotic microangiopathy; no antiphospholipid antibodies or large-vessel thrombosis to suggest antiphospholipid syndrome; and no fever, leukocytosis, or imaging features consistent with infectious enteritis[12].

Radiologically, lupus enteritis typically shows the “target sign” (concentric mural enhancement due to submucosal edema) and the “comb sign” (engorged mesenteric vessels)[7,10]. However, in severely ischemic forms, mucosal enhancement declines or disappears, reflecting compromised perfusion and impending necrosis[8,13]. The absence of superior mesenteric artery or vein occlusion on CT, together with mesenteric fat stranding and venous congestion, indicates a nonocclusive venous ischemic pattern that is more consistent with vasculitic injury than thromboembolism.

Rheumatological parameters such as elevated SLEDAI, high anti-dsDNA titers, and hypocomplementemia are often associated with lupus enteritis activity[4]. The patient’s high SLEDAI score (18) and elevated FDP/D-dimer levels indicated systemic immune activation and microvascular thrombosis. Although high-dose corticosteroids are generally effective within 24-48 hours, refractory or ischemic cases may require cytotoxic agents (e.g., cyclophosphamide) or biologic agents such as rituximab[14,15]. A recent systematic review of the gastrointestinal manifestations of SLE reported that high-dose intravenous corticosteroids and oral prednisolone remain the mainstay of treatment, with additional immunosuppressive agents reserved for severe, refractory, or relapsing cases[16]. On POD 14, cholestasis-induced inflammation triggered a cutaneous flare with an anti-nuclear antibodies titer (1:160) indicating immune activation. Escalation of additional immunosuppressive agents was not pursued because the clinical course did not reflect refractory lupus activity; instead, prompt biliary decompression was the key therapeutic intervention, and the flare resolved after stent placement. Oral methylprednisolone was temporarily increased to manage the transient inflammatory responses. Hydroxychloroquine was subsequently introduced during follow-up as a guideline-recommended maintenance therapy for all patients with SLE, supporting long-term disease control and facilitating corticosteroid tapering[15,17].

Surgical management is generally reserved for irreversible complications such as perforation or bowel infarction, as delayed intervention increases mortality in lupus enteritis[18]. In this patient, ischemia progressed despite high-dose corticosteroids and further delay would have increased the extent of necrosis and more complex reconstruction; therefore, early surgery was performed. Recent comparative data suggest that E-style (end-to-end) duodenojejunostomy may decrease delayed gastric emptying and intraperitoneal infection compared to S-style (side-to-side) configurations[19]. However, in our case, the ischemic segment extended along the second posterior portion of the duodenum near the ampulla of Vater. To avoid pancreaticoduodenectomy, our priority was to preserve the ampullary region, even if a small portion of the marginally perfused duodenum remained. Diagonal proximal transection was performed to maximally remove the devitalized segment, as illustrated in the operative schema (Figure 3B). Areas of edematous and poorly perfused tissue were left as short stapled stumps, and a hand-sewn side-to-side duodenojejunostomy was chosen because it allowed tension-free anastomosis of well-vascularized tissue and represented the most anatomically feasible configuration.

From a pathological perspective, histopathological examination in this case revealed small-vessel vasculitis with fibrinoid necrosis and luminal thrombosis, confirming ischemic injury secondary to lupus activity rather than an atherosclerotic or embolic etiology[2,20]. The presence of transmural necrosis with fibrinoid vascular injury and immune complex-mediated small-vessel inflammation is characteristic of lupus-related vasculitis, consistent with the previously described mechanisms of immune complex deposition and complement-driven vascular necrosis[21]. Immunohistochemistry and complement staining (C3 and C4) demonstrate immune complex deposition in the vessel walls, correlating with active SLE flares[22].

The postoperative pancreatitis and biliary obstruction in this patient can be explained by several plausible mechanisms. First, duodenal resection adjacent to the ampulla of Vater may induce reactive edema, resulting in transient mechanical obstruction of pancreaticobiliary outflow. Second, ischemic injury from mesenteric vasculitis may impair papillary function even if the ampulla is anatomically preserved. Finally, lupus-related microvascular inflammation may involve the pancreaticobiliary ducts and contribute to functional obstruction. Prompt radiological evaluation and timely drainage are essential because cholestasis can precipitate SLE exacerbation through inflammatory cytokine release[23].

This case highlights the need for a multidisciplinary approach. Radiologists play a key role in distinguishing between ischemic and inflammatory patterns; rheumatologists direct the intensity of immunosuppressive therapy; pathologists confirm vasculitic injury; and surgeons intervene when irreversible ischemia develops. Early recognition of duodenal involvement, appropriate immunotherapy, and vigilant postoperative monitoring of biliary complications are critical for achieving favorable outcomes. The patient reported marked relief of abdominal symptoms and expressed satisfaction with her recovery and the overall treatment process.

CONCLUSION

Severe lupus enteritis with duodenal ischemic necrosis is rare. The combination of radiologic venous ischemia, histologic vasculitis, and SLE flares supported an autoimmune etiology. Although corticosteroids remain the cornerstone of therapy, clinicians should be vigilant for ischemic transformation requiring surgical intervention. Close multidisciplinary collaboration and meticulous postoperative management of biliary complications are essential for long-term recovery.

ACKNOWLEDGEMENTS

The authors thank the Departments of Rheumatology, Departments of Radiology, and Departments of Pathology at the Soonchunhyang University Cheonan Hospital for their valuable collaboration in the diagnosis and management of this case.