Spontaneous Retroperitoneal Bleeding in a Patient with Systemic Lupus Erythematosus

Background and Objectives: Systemic lupus erythematosus (SLE) is a disease with multiple organ involvement, and spontaneous hemorrhage, especially perirenal hemorrhage, is rare. Case Presentation: We report the case of a 19-year-old teenager with SLE who experienced left flank pain and hypovolemic shock. Abdominal computed tomography revealed a large left retroperitoneal hematoma. Recurrent hypovolemic shock occurred despite the transcatheter arterial embolization of the left renal artery. Repetitive abdominal computed tomography results showed active hemorrhage. Result: An exploratory laparotomy was used to confirm descending colonic mesenteric artery bleeding, which was resolved. The patient needed temporary regular kidney replacement therapy for active lupus nephritis, which terminated one month after discharge. Conclusions: When patients with SLE experience acute abdominal pain, flank pain, or back pain combined with hypovolemia, there is a higher risk of bleeding due to spontaneous hemorrhage, which should be included in the differential diagnosis. Therefore, early diagnosis and adequate emergency intervention are necessary.

Due to the persistently unstable hemodynamic status, we performed abdominal computed tomography angiography and still observed an active hemorrhage in the hematoma with high-density contrast retention (Figure 3a,b).Therefore, the patient underwent an exploratory laparotomy with retroperitoneal exploration.Consequently, a marked ileus, intraperitoneal adhesion, and active oozing of the descending colon mesentery artery were observed, suggesting an inferior mesenteric artery origin.Enterolysis and hematoma evacuation were at approximately 3.9 L. Blood and clot tests were performed.The pathological report showed mixed acute and chronic inflammation with fibrosis without morphological evidence of malignancy.
After surgical intervention, the patient's renal function improved; however, he developed oliguria and shortness of breath 2 weeks later.A 3-day course of low-dose pulse steroid therapy with 160 mg of intravenous methylprednisolone daily was administered, and the patient subsequently received regular hemodialysis because of no recovery of renal function.Consequently, the patient received an indwelling Hickman catheter for long-term renal replacement therapy and was discharged in stable condition.During the outpatient department follow-up, his blood urea, nitrogen, and creatinine levels improved with appropriate urine output one month later.The Hickman catheter was removed, and long-term hemodialysis was no longer required.Unfortunately, this patient had another episode of acute kidney injury four months after discharge.At that time, cardiac arrest combined with respiratory failure occurred.Consequently, he received a tracheotomy because he needed to be trained to wean off the ventilator, and he was transferred to the respiratory care center of another hospital.range: <10.0 IU/mL), and weakly positive anti-cardiolipin IgM: 20.5 U/mL (normal range: <10 U/mL).Other autoimmune antibodies of anti-cardiolipin IgG, β2-glycoprotein IgM, β2-glycoprotein IgG, anti-smith, and anti-ribonucleoprotein were negative.Laboratory data showed decreased C3 (64.4 mg/dL, normal range: 87-200 mg/dL) and C4 (10.2 mg/dL, normal range: 19-52 mg/dL) complement levels, increased lupus anti-coagulant levels of 1.23 (normal range: ≤1.2), and increased IgM (>500 mg/dL, normal range: 45-281 mg/dL) levels.Due to the persistently unstable hemodynamic status, we performed abdominal computed tomography angiography and still observed an active hemorrhage in the hematoma with high-density contrast retention (Figure 3a,b).Therefore, the patient underwent an exploratory laparotomy with retroperitoneal exploration.Consequently, a marked ileus, intraperitoneal adhesion, and active oozing of the descending colon mesentery artery were observed, suggesting an inferior mesenteric artery origin.Enterolysis and hematoma evacuation were at approximately 3.9 L. Blood and clot tests were performed.The pathological report showed mixed acute and chronic inflammation with fibrosis without morphological evidence of malignancy.
After surgical intervention, the patient's renal function improved; however, he developed oliguria and shortness of breath 2 weeks later.A 3-day course of low-dose pulse

Discussion
Anemia is observed in almost all patients with bleeding.Nonspecific signs, such as fatigue, dizziness, or tachycardia, may be present, and severe cases may present as hypovolemic shock.Notably, other symptoms are diverse and strongly associated with the bleeding location.Previous case reports have reported several spontaneous bleeding sites, including the gastrointestinal tract, muscle, corpus luteum, brain, spleen, soft tissue,

Discussion
Anemia is observed in almost all patients with bleeding.Nonspecific signs, such as fatigue, dizziness, or tachycardia, may be present, and severe cases may present as hypovolemic shock.Notably, other symptoms are diverse and strongly associated with the bleeding location.Previous case reports have reported several spontaneous bleeding sites, including the gastrointestinal tract, muscle, corpus luteum, brain, spleen, soft tissue, and adrenal gland in patients with SLE [10,11,16,17,19,[22][23][24].One cohort study demonstrated an increased risk of subarachnoid hemorrhage in patients with SLE [25].
Patients with abdominal pain or fullness may have bleeding sites in the abdominal cavity.However, palpable masses or ecchymoses may indicate the corresponding bleeding region.Patients with spontaneous non-traumatic renal hemorrhage (so-called Wünderlich syndrome) may present with the classic "Lenk's triad" [15], including acute flank pain, flank mass, and hypovolemia.Regarding imaging studies, sonography and computed tomography are indispensable and can accurately detect bleeding events.Consequently, angiography can further determine the bleeding location.
In this present case, the patient presented with symptoms of Lenk's triad, except for a palpable flank mass.He was diagnosed with retroperitoneal bleeding based on abdominal computed tomography results.From the transcatheter arterial embolization examination, the initial bleeding site was considered to be angiodysplasia of the left renal artery.Unfortunately, the active retroperitoneal bleeding was not controlled, and another bleeding site in the branch of the inferior mesenteric artery was confirmed using exploratory laparotomy.We obtained no specimens from the kidney or descending colon mesentery artery during the entire procedure, and no further histological changes were observed based on laboratory outcomes.Furthermore, because of no remarkable prolonged aPTT, we unfavored a coagulopathy disorder to be the cause of bleeding.Thus, we did not have relevant tests such as Von Willebrand factor deficiency or other coagulation disorders due to a lack of coagulation factors (factor XIII, factor IX, Coagulation factor inhibitors, factor eight inhibitor bypass activity, and recombinant activated factor VII).This is the limitation of our case.
We speculate that the patient had active lupus nephritis due to the poorly controlled nephrotic syndrome.SLE-related vasculitis results in subsequent spontaneous renal artery bleeding and favored inferior mesenteric artery bleeding.
Based on a previous analysis of the risk factors for Wünderlich syndrome, the only risk factor in our patient was angiodysplasia of the left renal artery, which was not recovered in the past.Unfortunately, hemorrhage occurred, and the location of bleeding was unusual compared with that described in previous literature reviews.

Figure 1 .
Figure 1.This is a figure showing the CT of the abdomen at the emergency department.Axial view (a) and coronal view (b).There was a large retroperitoneal hematoma in the left retroperitoneal space (yellow asterisk) and suspected active bleeding in the left kidney lower portion (red thick arrow).Mild ascites (green thin arrow) and decreased volume with a small caliber of the aorta and inferior vena cava (blue arrowhead) were noted.

Figure 1 .
Figure 1.This is a figure showing the CT of the abdomen at the emergency department.Axial view (a) and coronal view (b).There was a large retroperitoneal hematoma in the left retroperitoneal space (yellow asterisk) and suspected active bleeding in the left kidney lower portion (red thick arrow).Mild ascites (green thin arrow) and decreased volume with a small caliber of the aorta and inferior vena cava (blue arrowhead) were noted.

Figure 1 .
Figure 1.This is a figure showing the CT of the abdomen at the emergency department.Axial view (a) and coronal view (b).There was a large retroperitoneal hematoma in the left retroperitoneal space (yellow asterisk) and suspected active bleeding in the left kidney lower portion (red thick arrow).Mild ascites (green thin arrow) and decreased volume with a small caliber of the aorta and inferior vena cava (blue arrowhead) were noted.

Figure 2 .
Figure 2.This is a figure showing the transcatheter arterial embolization: The left renal angiography showed minimal contrast extravasation in the left kidney lower portion (red arrow).Angiodysplasia of the left renal artery arteries was found (a).Superselective canulation of the tumor-feeding artery arising from the inferior lobar artery of the left renal artery with Terumo microcatheter (Progreat, 2.7 Fr). Two metallic coils (VoetX 3 mm × 3.3 mm, 4 mm × 4 mm) were placed until the sluggish flow of the left renal artery (yellow arrow) (b).

Figure 2 .
Figure 2.This is a figure showing the transcatheter arterial embolization: The left renal angiography showed minimal contrast extravasation in the left kidney lower portion (red arrow).Angiodysplasia of the left renal artery arteries was found (a).Superselective canulation of the tumor-feeding artery arising from the inferior lobar artery of the left renal artery with Terumo microcatheter (Progreat, 2.7 Fr). Two metallic coils (VoetX 3 mm × 3.3 mm, 4 mm × 4 mm) were placed until the sluggish flow of the left renal artery (yellow arrow) (b).

Figure 3 .
Figure 3.This is a figure showing the CT of the abdomen on day 5 of admission.Axial view (a) and coronal view (b).There was an active hemorrhage in the left retroperitoneal space hematoma (yellow asterisk) with high-density contrast retention (red arrow).The ascites increased compared with the day of admission (green thin arrow).

Figure 3 .
Figure 3.This is a figure showing the CT of the abdomen on day 5 of admission.Axial view (a) and coronal view (b).There was an active hemorrhage in the left retroperitoneal space hematoma (yellow asterisk) with high-density contrast retention (red arrow).The ascites increased compared with the day of admission (green thin arrow).

Table 1 .
Summary of previously published cases of SLE-associated spontaneous perirenal hemorrhage.