Chronic intestinal pseudo-obstruction in systemic lupus erythematosus

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.     

Intestinal pseudo-obstruction and ureterohydronephrosis as the presenting manifestations of relapse in a lupus patient

Intestinal pseudo-obstruction (IPO) is a rare complication of systemic lupus erythematosus (SLE). We present a 32-year old female with SLE for seven years. She was admitted with profound fatigue, frequent vomiting, colicky abdominal pain, diarrhoea and intermittent dysuria for the past 12 months. Imaging studies revealed dilated small and large bowel loops with thickened intestinal wall and multiple fluid levels. Urinary tract involvement was also demonstrated. The patient responded well to immunosuppressive treatment. IPO in the context of SLE has been described only in anecdotal case reports. Half of the cases developed this complication during the course of lupus as in the present case. Concomitant ureterohydronephrosis was present in approximately two-thirds of the cases. Early recognition of the syndrome is necessary for the institution of the appropriate medical treatment and prevention of inappropriate surgical intervention.     

Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Intestinal pseudo-obstruction (IPO) is a rare but dangerous complication of systemic lupus erythematosus (SLE) when the patient has no other manifestations except gastrointestinal symptoms. We performed 1 patient with a 2-month history of recurrent vomiting and abdominal distension. She admitted past surgical histories of cesarean section and appendectomy. A physical examination revealed tenderness in the right lower abdominal on palpation and bowel sounds were weak, 2 to 3 bpm. An x-ray and CT of her abdomen showed intestinal obstruction. The initial diagnosis was adhesive intestinal obstruction. She received surgical treatment because her symptoms had gradually become more frequent and persistent. But she vomited again 2 weeks later after the surgery. Further immunology tests indicated that she had an IPO secondary to SLE. We treated the patient with methylprednisolone pulse for 3 days and followed by prednisone orally. The patient had a good response. Complete remission was achieved on 8 years follow-up.The importance of IPO secondary to SLE lies in an early diagnosis. After the diagnosis is established, immunosuppressive therapy should be the initial and first-line treatment, and surgical intervention is often disappointing and should be carefully avoided. It is necessary to enhance awareness of doctors to IPO secondary to SLE.     

Néphropathie lupique liée à un déficit immunitaire commun variable d’évolution favorable sous immunoglobulines intraveineuses

We report a 24-year-old woman who presented with a nephrotic syndrome as the revealing manifestation of systemic lupus erythematosus (SLE) and an associated hypogammaglobulinemia related to a common variable immunodeficiency (CVID). Outcome of SLE was favourable with intravenous immunoglobulin treatment solely. Relationships between SLE and CVID are discussed.     

系统性红斑狼疮并发的假性肠梗阻

目的 分析系统性红斑狼疮(SLE)罕见的临床并发症——假性肠梗阻(IPO)，以引起临床医师重视。方法 结合文献复习，回顾性分析北京协和医院6年来SLE合并IPO患者10例。结果 10例女性患者平均年龄29．4岁(14～50岁)，SLE诊断均符合1982年美国风湿病协会关于SLE的诊断标准，并无硬皮病和重叠综合征的临床表现，无腹部手术史。所有患者抗核抗体滴，度增高，其中4例为轻度增高，双链DNA阳性5例。6例IPO为SLE内脏病变的首发症状，并同时伴有肾盂积水、输尿管扩张，其中1例死于肠穿孑L。结论 IPO是SLE的一个罕见但严重的并发症，由内脏平滑肌受累运动障碍所致，自身抗体谱有其特点。及早诊断，联合肾上腺皮质激素和环磷酰胺治疗对于改善预后非常重要。     

La pancréatite lupique : une série de six cas

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.     

Rupture spontanée du tendon d’Achille à la phase précoce d’un lupus érythémateux systémique

Spontaneous tendon rupture is rare in the course of systemic lupus erythematosus (SLE). Its incidence rate remains unknown. The pathogenesis of this manifestation is complex and poorly understood. We report a 39-year-old woman who presented with a spontaneous Achille's tendon rupture as the presenting presentation of SLE, before any corticosteroid therapy. All the patients previously published were receiving corticosteroids and reported in some an associated traumatism. Risk factors are prolonged disease duration, chronic therapy with corticosteroids, deforming arthropathy of the hands, and inactive disease.     

Intestinal pseudo-obstruction in systemic lupus erythematosus

In order to document intestinal pseudo-obstruction (IPO) as a recently recognized manifestation of systemic lupus erythematosus (SLE), we report the case or a woman with SLE who presented with IPO and we review 21 other previously reported cases from an English literature search. In 41% of the cases, IPO was the initial manifestation of their underlying lupus. The clinical and laboratory features were not significantly different from those reported in large series of patients with SLE, except for an apparent association with an urinary tract involvement (ureterohydronephrosis and interstitial cystitis). The pathogenic mechanism of this complication is not fully understood, but seems to be heterogeneous. IPO responded readily to high dose steroid therapy in all patients, but in some cases this complication evolved regardless of the underlying disease activity. A high level of awareness of this complication is needed to avoid unnecessary surgical intervention.     

Clinical Analysis of 61 Systemic Lupus Erythematosus Patients With Intestinal Pseudo-Obstruction and/or Ureterohydronephrosis: A Retrospective Observational Study

The objective of this article is to investigate the clinical features of intestinal pseudo-obstruction (IPO) and/or ureterohydronephrosis in systemic lupus erythematosus (SLE).Sixty-one SLE patients with IPO and/or ureterohydronephrosis were analyzed retrospectively. A total of 183 cases were randomly selected as controls from 3840 SLE inpatients without IPO and ureterohydronephrosis during the same period. Patients were assigned to 1 of the 3 groups (SLE with IPO and ureterohydronephrosis, SLE with IPO, and SLE with ureterohydronephrosis). The clinical characteristics, treatments, and prognosis were compared between the 3 groups.There were 57 females and 4 males, with a mean age of 32.0 years. IPO was the initial manifestation of SLE in 49.1% of the cases, whereas ureterohydronephrosis in 32.5%. All patients were initially treated with a high-dose steroid. Thirty-one of these patients (50.8%) also received intravenous methylprednisolone pulse therapy. Two patients died of bowel perforation and lupus encephalopathy, and the other 59 patients (96.7%) achieved remission after treatment. The incidences of fever, glomerulonephritis, nervous system involvement, serositis, erythrocyte sedimentation rate elevation, hypoalbuminemia, hypocomplementemia, and anti-SSA antibody positivity were significantly higher in patients with IPO and/or ureterohydronephrosis than in the control group (without IPO and ureterohydronephrosis). Also, patients with IPO and/or ureterohydronephrosis had higher SLE Disease Activity Index scores than control patients. Compared with SLE patients with IPO, the patients with IPO and ureterohydronephrosis had a significantly higher incidence of gallbladder wall thickening, biliary tract dilatation, and serositis, whereas the patients with ureterohydronephrosis had less mucocutaneous involvement and serositis. Eight of the 47 IPO patients who initially responded well to immunotherapy relapsed; however, all responded well to retreatment with adequate immunotherapy. Of these 8 patients, 4 relapsed following poor compliance and self-discontinuation of steroid or immunosuppressant therapy. The rate of poor compliance with immunotherapy and the number of organ systems involved in patients in the recurrent IPO group were significantly higher than those in the nonrecurrent IPO group.IPO and ureterohydronephrosis are severe complications of SLE. As patients usually respond readily to early optimal steroid treatment, early diagnosis and timely initiation of glucocorticoid are important to relieve symptoms, prevent complications, and improve prognosis.     

Acute bowel intussusception revealing celiac disease: a new case and literature review

Introduction

Acute bowel intussusception is a rare manifestation in adult, which mainly involves the small intestine. Celiac disease is a frequent small bowel disease that is largely undiagnosed in adults. We report a patient in whom spontaneously regressive small bowel intussusception was the presenting manifestation of celiac disease.

Case report

A 40-year-old man was admitted for a right-sided iliac abdominal pain related to a small bowel intussusception. Laparotomy ruled out a digestive tumor. Persistence of diffuse abdominal pain associated with progressive and unexplained weight loss for several months led to the diagnosis of celiac disease, which was confirmed by the presence of specific serum autoantibodies and histological duodenal villous atrophy.

Conclusion

The association between small bowel intussusception and celiac disease does not seem to be fortuitous. Based on this report and the literature review, we suggest that celiac disease can favour small bowel intussusception, even in adulthood. Therefore, diagnosis of celiac disease must be discussed in the presence of unexplained intussusception.     

系统性红斑狼疮并发假性肠梗阻及输尿管肾盂积水的临床及实验室特点

目的通过分析系统性红斑狼疮(SLE)并发假性肠梗阻(IPO)及输尿管肾盂积水患者的临床及实验室特点,以提高临床医生对此并发症的认识。方法回顾性分析本院2000—2005年SLE并发IPO患者10例,收集临床及实验室资料,分析其发病情况、病情活动情况及受累脏器、病程、治疗及预后特点。结果以肠梗阻为首发者3例,同时伴输尿管肾盂积水者7例(7/9),膀胱壁增厚、毛糙5例(5/9),伴有胆囊壁增厚或壁毛糙者6例(6/8)。抗核抗体阳性10例,抗dsDNA抗体阳性6例,抗SSA抗体阳性6例,抗核糖核蛋白(RNP)抗体阳性5例,抗心磷脂抗体IgG阳性4例(4/7)。所有病例均有血清补体C3和C4的降低。肠梗阻症状均在大剂量糖皮质激素治疗1周左右缓解。结论IPO常与输尿管肾盂积水及胆囊壁增厚相伴发,提示由内脏平滑肌受累本身运动障碍所致,及时使用肾上腺糖皮质激素治疗对于控制病情、改善预后非常重要,应避免不必要的手术干预。     

An unusual case of intestinal pseudo-obstruction presenting in an adolescent with juvenile-onset systemic lupus erythematosus: A diagnostic challenge

Introduction

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple systemic manifestations. The disease itself typically causes only 2–30% of SLE-associated gastrointestinal conditions.

Intestinal pseudo-obstruction in inactive systemic lupus erythematosus: An unusual finding

Chronic intestinal pseudo-obstruction (CIP) is an infrequent complication of an active systemic lupus erythematosus (SLE). We illustrate a case of SLE inactive-related CIP. A 51-year old female with inactive SLE (ECLAM score 2) was hospitalized with postprandial fullness, vomiting, abdominal bloating and abdominal pain. She had had no bowel movements for five days. Plain abdominal X-ray revealed multiple fluid levels and dilated small and large bowel loops with air-fluid levels. Intestinal contrast radiology detected dilated loops. CIP was diagnosed. The patient was treated with prokinetics, octreotide, claritromycin, rifaximin, azathioprine and tegaserod without any clinical improvement. Then methylprednisolone (500 mg iv daily) was started. After the first administration, the patient showed peristaltic movements. A bowel movement was reported after the second administration. A plain abdominal X-ray revealed no air-fluid levels. Steroid therapy was slowly reduced with complete resolution of the symptoms. The patient is still in a good clinical condition. SLE-related CIP is generally reported as a complication of an active disease. In our case, CIP was the only clinical demonstration of the SLE.     

Infection aiguë à cytomégalovirus révélatrice d’un lupus érythémateux systémique

Systemic lupus erythematosus (SLE) remains of unknown origin. Herpes viridae infections seem to play a role in the pathogenesis of this disease. We report a 31-year-old man who presented an acute cytomegalovirus (CMV) infection with persistent fever and myopericarditis as the presenting manifestation of SLE. This case report emphasizes a difficult differential diagnosis between SLE and an acute CMV infection and suggests a possible role of this virus in the pathogenesis of SLE.     

Smooth muscle myopathy as an underrecognized manifestation of active systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with protean manifestations. We here present a case of unexplained diarrhoea and abdominal pain in a patient with SLE. Investigations revealed dilatation of stomach, small bowel and colonic wall, biliary and pancreatic ducts, renal collecting systems and ureters as well as thoracic aorta and major pulmonary arteries, as manifestations of a smooth muscle myopathy that was responsive to immunosuppressive therapy with cyclosporin A.     

Abdominal pain as the initial and sole clinical presenting feature of systemic lupus erythematosus.

Classically, a diagnosis of systemic lupus erythematosus (SLE) is dependent on renal, rheumatological, cutaneous and neurological target organ damage with supporting serological markers. A previously healthy 26-year-old Japanese woman whose only manifestation of otherwise occult SLE was severe abdominal pain is reported. A computed tomographic scan of the abdomen revealed thickened loops of small bowel, endoscopic findings were nonspecific and jejunal biopsy revealed a nonspecific enteritis. Laboratory studies revealed lymphopenia, hypocomplementemia, a positive antinuclear antibody, a weakly positive anti-Smith and a strongly positive anti-double stranded DNA. There was a prompt symptomatic recovery with immunosuppressive therapy. The authors' experiences, and a review of the literature suggest that a diagnosis of SLE should be considered in young Asian women who present with significant but clinically enigmatic gastrointestinal illness.     

Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome： Case report and review of the literature

INTRODUCTION Gallbladder disease is no more common in patients with systemic lupus erythematosus (SLE) than in the general population[1]. Scarce reports on acalculous cholecystitis (ACC) in SLE or in antiphospholipid syndrome (APS) have been described in …     

Neurolupus (1 partie). Description et démarche diagnostique et thérapeutique dans les manifestations neurologiques centrales et psychiatriques au cours du lupus érythémateux systémique

Systemic lupus erythematosus (SLE) is an autoimmune disease, which primarily affects skin and joints. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. A classification, published in 1999 by the American College of Rheumatology (ACR) research committee, described 12 CNS syndromes and seven peripheral neurologic syndromes compatible with “neuropsychiatric systemic lupus erythematosus” (NPSLE). Despite this consensus, studies which have been published since 1999 have reported a prevalence of NPSLE varying from 20 to 97 %, which shows the diagnosis difficulty and the heterogeneity of neuropsychiatric manifestations in SLE. In order to understand the limits of this classification, we propose in this first part an exhaustive review of publications describing neuropsychiatric manifestations according to the ACR 1999 classification. We also detail case definitions, prevalence and risk factors, clinical characteristics and diagnosis of each lupus-related psychiatric and CNS manifestation.     

Lupus peritonitis presented as vague abdominal complaints in a SLE patient.

A 40-yr-old Caucasian woman who had been suffering from systemic lupus erythematosus (SLE) since five years developed vague abdominal complaints whilst under treatment with a low dose of steroids. She had been admitted because of vomiting and abdominal tenderness. The ESR and CRP levels were rising and the C4 level had been persistently low in the preceding months. Normal non-invasive procedures did not allow a diagnosis to be made. Therefore exploratory laparotomy was performed and revealed a non-bacterial peritonitis and an oedematous jejunum. She responded well to a high dose of prednisone. Serositis of the peritoneum as well as bowel vasculitis may be a rare manifestation of SLE despite apparent control of other lupus manifestations. In this patient serositis flares were associated with a rise in CRP level.     

L’entérite : une manifestation peu fréquente et corticosensible du lupus érythémateux systémique

Introduction

. Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis.

Case reports

. We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded.

Conclusion

. Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.