Acute acalculous cholecystitis in a child with systemic lupus erythematosus
- First Online:
- Cite this article as:
- Basiratnia, M., Vasei, M., Bahador, A. et al. Pediatr Nephrol (2006) 21: 873. doi:10.1007/s00467-006-0021-x
- 117 Views
A 10-year-old boy with systemic lupus erythematosus (SLE) developed abrupt right upper quadrant pain and vomiting during the course of his active disease. Antiphospholipid antibody was negative and the C3 level was low. Abdominal sonography showed cholecystitis with sludge balls in the gallbladder. He was treated by high-dose prednisolone with ceftriaxone and metronidazole IV for 3 days but due to poor response, cholecystectomy was performed and no stone was identified. Histopathologic examination showed vasculitis in the medium-sized arteries of the gallbladder wall. He was doing well at the 9-month follow-up after the operation. This report describes the first pediatric case of SLE with acalculous cholecystitis caused by vasculitis of the gallbladder.
KeywordsSystemic lupus erythematosusVasculitisAcalculous cholecystitis
Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement and a wide range of manifestations. Common gastrointestinal manifestations of SLE in children consist of oral ulcer, abdominal pain, dysphagia, gastroesophageal reflux, serositis, pancreatitis, rising liver enzyme levels, and intestinal bacterial overgrowth. Splenic infarction and intestinal perforation are the other rare complications of SLE .
Among patients with SLE, gallbladder involvement is an uncommon event. Acalculous cholecystitis (ACC) is a very rare complication of SLE and a remarkable diversity of risk factors has been implicated in its development. To our knowledge only ten cases of SLE with ACC have been reported so far [2–10]. All of the reported cases were adult patients. In this report we describe the first pediatric case of ACC in a child suffering from SLE. Awareness of the presence of this rare complication may be helpful for accurate diagnosis of the cause of the abdominal pain in children with SLE.
The patient was a 10-year-old boy who was admitted with fever, malaise, polyarthritis, and mild hypertension. Laboratory data showed a high titer of antinuclear antibody (ANA) and low C3 and C4 levels with positive anti-dsDNA. Antiphospholipid Ab level, which was measured by an enzyme-linked immunosorbent assay (ELISA) method (Genesis Diagnostics, Cambridgeshire, UK), was negative. Activated partial thromboplastin time was within normal limits. Urine analysis showed microscopic hematuria and non-nephrotic range proteinuria. Kidney biopsy revealed lupus nephritis class III with no evidence of vasculitis. Treatment was started with prednisolone and hydroxychloroquine.
Two months later he was referred due to exacerbation of lupus nephritis (rise in serum creatinine and proteinuria and decrease in urine output) and cerebritis (convulsion and hypodense area on magnetic resonance imaging). He was treated with IV methylprednisolone pulse therapy and IV cyclophosphamide. Upon relief of the symptoms he was discharged on prednisolone 1.5 mg/kg per day. Three weeks later he was referred with abdominal pain, nausea, vomiting, and fever. Physical examination revealed right upper quadrant tenderness with positive Murphy’s sign.
The main laboratory data of the patient during admission course
White blood cell
Blood urea nitrogen
24-h urine protein
neg <5 U/ml
neg <5 U/ml
>1.1 positive IU/ml
>60 positive IU/ml
>18 positive IU/l
He received IV cyclophosphamide as well as a steroid and was discharged with prednisolone 1.5 mg/kg per day and monthly cyclophosphamide pulse therapy. At the last follow-up (9 months later) he was apparently well and his blood urea nitrogen (BUN) was 18 mg/dl, Cr 1 mg/dl urine protein was 270 mg/day, and antiphospholipid Ab and lupus anticoagulant were negative (LAC; 36.4 S, normal range: 25–43).
Abdominal pain is a challenging diagnostic and therapeutic problem in SLE patients. Abdominal pain may be produced by the disease itself, concomitant disease, or the side effects of drugs. Gastrointestinal vasculitis with or without infarction is the most common etiology of acute abdominal pain in SLE . Most of the patients do not require surgical treatment, but some rare cases such as perforated ulcer and intestinal infarction need surgical intervention.
Abdominal pain originating from the gallbladder can be seen in various settings in SLE. The SLE patients like in other collagen vascular diseases are prone to biliary sludge and stone formation because of biliary dyskinesia . They are also at increased risk of developing ACC. Vascular insufficiency on the basis of low flow or physical occlusion is a plausible consequence of a great number of clinical situations thought to predispose to ACC . There are two main causes of ischemia of the gallbladder in SLE: vasculitis and thrombosis. Most of the cases of SLE which were complicated by acute acalculous cholecystitis have been associated with vasculitis [2–4, 8]. Because of the high risk of morbidity, surgery is the main treatment of ACC. There have been two case reports in the literature addressing successful treatment of SLE-induced ACC with high doses of corticosteroid [2, 3]. In both reports the cholecystitis was diagnosed by the presence of increased thickness of the gallbladder wall and pericholecystic edema but were not proven by pathologic examination. Shin et al. recommended that if the patient’s general condition is good and there is no other risk factor for ACC or its serious complications, high-dose steroid therapy may be considered as the first line of treatment . Despite being on high-dose prednisolone, our patient suffered from diffuse vasculitis in the small-sized and medium-sized arteries. He underwent surgical treatment because of lack of response to medical therapy. In addition abdominal sonography in our patients showed two non-shadowing echogenic structures in favor of sludge ball, but we could not rule out gallstones for sure. The gallstone shadow is dependent on the transducer frequency and on the size and the type of gallstone, and no definite shadow could be seen with the 3.5-MHz transducer used .
SLE patients can produce antiphospholipid antibody frequently and become susceptible to ACC. Dessailloud et al. showed that antiphospholipid antibody syndrome can produce ACC . They found multiple thrombi in the gallbladder veins and no vasculitis. Nolen et al. reported the first successful experience with heparin and antivitamin K therapy in a case of antiphospholipid antibody syndrome . Antiphospholipid antibody (aPL) was negative in our patient. The presence of aPL is more likely associated with thrombosis or fetal miscarriage and a relation between vasculitis and aPL has not been described.
Mesenteric inflammatory veno-occlusive disease (MIVOD) is another rare cause of ACC in SLE . This type of vasculitis exclusively involves mesenteric veins and/or its branches and sparing arteries. Histologically the main inflammatory cells are lymphocytes with occasional granulomatous inflammation. The etiology of MIVOD is unknown. Our case was not MIVOD because of the typical fibrinoid necrosis of the arteries.
All of the reported lupus cases with ACC were adults; the majority were due to vasculitis and all of them except two were treated surgically. In our patient because of being under treatment for active SLE, cholecystectomy was performed. This is the first case report of vasculitis of the gallbladder in a child with SLE. Because not all of the pediatric SLE patients undergo abdominal sonography or computed tomography, the precise incidence of ACC in pediatric-onset SLE is uncertain, and subclinical ACC or mild vasculitis in the gallbladder may be underestimated. Awareness of this finding would be valuable in the evaluation of cholelithiasis and cholecystitis in SLE patients. The decision on medical or surgical treatment should be based on the patient’s general condition and risk factors.
We would like to thank Dr. Sirus Arshadi for interpretation of the sonography in this case.