Intestinal pseudo-obstruction as initial presentation of thymoma.

A 45-year-old-man presented with severe vomiting, constipation, abdominal distention and bilateral ocular abductor palsy. Evaluation revealed diffuse autonomic dysfunction characterized by intestinal pseudo-obstruction, xerophthalmia, xerostomia, postural hypotension, erectile dysfunction and loss of sinus arrhythmia. Paraneoplastic work-up revealed thymoma. Most symptoms resolved after surgical removal of the thymoma. Six weeks later he developed worsening of external ophthalmoparesis with ptosis, responding to acetylcholinesterase inhibitor, confirming myasthenia gravis.     

Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis.

Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon.     

Intestinal pseudo-obstruction caused by neuromyopathy in a patient with systemic sclerosis

Although gastrointestinal involvement in systemic sclerosis is common, the pathophysiology of small-bowel involvement in systemic sclerosis is not fully understood. We report a case of a 56-year-old woman who has systemic sclerosis with intestinal pseudo-obstruction. The small-bowel manometric findings showed low-amplitude intestinal contraction and absence of the migrating myoelectric complex, and the electrogastrography revealed no increase of the slow-wave power. The manometric and electrogastrographic findings demonstrate that neuromyopathy may play a role in pseudo-obstruction of systemic sclerosis.     

Intestinal pseudo-obstruction in systemic amyloidosis

The occurrence of clinical manifestations of mechanical intestinal obstruction in eight cases of systemic amyloidosis is reported. Seven similar cases have been recorded in the literature and are briefly reviewed. Correct diagnosis is important in these cases if useless surgical treatment or prolonged diagnostic investigations are to be avoided. Gastrointestinal involvement by systemic amyloidosis should be considered in a patient presenting with clinical manifestations of mechanical obstruction but paralytic ileus seen on plain films. In these cases an attempt should be made, by means of a barium enema, to exclude an obstructing lesion of the colon or distal small bowel. Once amyloidosis is considered, it can be confirmed easily and safely by biopsy of the rectal or small-intestinal mucosa.     

Intestinal pseudo-obstruction caused by neuromyopathy in a patient with systemic sclerosis

Abstract

Although gastrointestinal involvement in systemic sclerosis is common, the pathophysiology of small-bowel involvement in systemic sclerosis is not fully understood. We report a case of a 56-year-old woman who has systemic sclerosis with intestinal pseudo-obstruction. The small-bowel manometric findings showed low-amplitude intestinal contraction and absence of the migrating myoelectric complex, and the electrogastrography revealed no increase of the slow-wave power. The manometric and electrogastrographic findings demonstrate that neuromyopathy may play a role in pseudo-obstruction of systemic sclerosis.     

Intestinal pseudo-obstruction complicating multiple sclerosis

Three patients with intestinal pseudo-obstruction secondary to multiple sclerosis are reported. This is a serious complication with significant morbidity and mortality, which is infrequently recognized in clinical practice and rarely reported in the medical literature.     

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。     

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.     

Intestinal pseudo-obstruction syndrome in systemic lupus erythematosus

Intestinal pseudo-obstruction (IpsO) occurs in a small subgroup of patients with systemic lupus erythematosus (SLE), and has been under-recognized. The etiology and mechanisms are unclear. Herein, we report two cases. In case 1, IpsO occurred as the complication in a previously diagnosed SLE patient. In case 2, IpsO with an image-evidenced intussusception and a peripheral eosinophilia, was the initial presentation, leading to the diagnosis of SLE. In both cases, IpsO existed concomitantly with ureterohydronephrosis, and evolved into peritonitis, which we called together IpsO syndrome. However, this surgical abdomen-like syndrome had a satisfactory response to glucocorticosteroids instead of antibiotics and surgery. Physician awareness and early diagnosis could positively affect outcomes of this syndrome, avoiding unnecessary surgical intervention.     

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。     

系统性红斑狼疮患者合并假性肠梗阻

目的:观察分析系统性红斑狼疮(SLE)患者合并假性肠梗阻(IPO)的临床特点及预后。方法:2004年1月至2014年4月南京军区南京总医院肾脏科住院确诊为SLE合并IPO患者30例,回顾性分析其临床表现、实验室检查结果、影像学特点、治疗及预后。结果:30例SLE合并IPO患者中,女性26例,男性4例,发生IPO时平均年龄(26.1±11.2)岁,SLE中位病程为27.5月,狼疮活动性指数(SLE-DAI)评分(11.2±7.4)分。6例有明确诱因(5例不洁饮食、1例上呼吸道感染)。IPO临床症状依次为恶心呕吐(76.6%)、腹痛(66.7%)、腹泻(56.7%)和腹胀(30%)。腹部平片及腹部CT可辅助诊断肠梗阻,阳性率75%,7例患者同时合并肾盂、输尿管积水。96.7%患者存在贫血,9例患者血小板减少,8例患者白细胞减少,4例直接Coombs试验阳性。96.7%的患者补体C3降低,ANA阳性率89.7%,抗ds-NDA抗体阳性率51.7%,抗心磷脂抗体阳性率30.4%,狼疮抗凝物阳性率43.8%,抗C1q抗体阳性率47.4%。29例患者接受激素为主[泼尼松0.6~1 mg/(kg·d)]的免疫抑制剂治疗(其中10例甲泼尼龙冲击治疗,11例丙种免疫球蛋白治疗),病情均好转出院,IPO好转中位数时间12d。24例患者长期随访(1~60月),2例死亡,4例行腹膜透析,5例反复发生IPO,至随访终点,肾脏完全缓解率25%,部分缓解率41.7%。结论:SLE累及消化道导致IPO临床相对罕见,但症状较重,容易误诊,早期识别,并及时给予免疫抑制剂治疗。     

系统性红斑狼疮伴发假性肠梗阻十例分析

目的了解系统性红斑狼疮(SLE)伴发假性肠梗阻(IPO)的临床特征,提高对该病的诊治水平。方法收集武汉同济医院2001年7月至2005年7月收治随访的10例SLE伴发IPO的临床资料,按1:2比例选择20例同期收治性别、年龄相配,无IPO伴发的SLE病例对比分析。并搜寻国内、国外(仅限于英文)相关病例40例一并综述。结果10例IPO中,以IPO为首发表现的病例数、IPO合并泌尿系统损害及治疗后稳定病例数分别为1、5和4例,综述文献三者比例分别为46%,64%和46%。与不伴有IPO的单纯SLE病例相比,伴发IPO者其外周血白细胞数相对较高(8.1±5.2vs4.0±1.8,P<0.05),而SLE疾病活动指数(SLEDAI)评分则较低(6.1±2.2vs9.6±1.8,P<0.01),其他病情观察指标差异均无统计学意义。结论IPO可为SLE的首发表现,但往往也是SLE病程中的一种并发症,它常与泌尿系统的病变相关联。激素和免疫抑制剂能够迅速改善病人的临床症状和预后。     

Sinus node dysfunction as an initial presentation of adult systemic lupus erythematosus

Cardiac involvement in systemic lupus erythematosus (SLE) has been well described. However, sinus node involvement with profound sinus bradycardia as an early manifestation of adult SLE has not been reported. A 27-year-old previously healthy female was admitted due to intermittent fever for 4 days. SLE was diagnosed based on clinical manifestations and laboratory data. Profound sinus bradycardia (heart rate?=?41/min) with weakness was noted during hospitalization. ECG abnormalities completely resolved after a high-dose intravenous steroid infusion. Sinus node involvement with significant bradycardia is one of the possible complications in the early stage of adult SLE. Close cardiovascular monitoring and serial ECGs are suggested for early detection of this serious complication of adult SLE.     

Pancarditis as initial presentation of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a multisystem disorder with cardiac involvement in about 50% of cases, yet clinically significant lesions are less common. SLE with pancarditis at initial presentation has so far not been reported. We present a rare case of SLE with culture negative endocarditis, reversible left ventricular (LV) dysfunction and mild pericardial effusion. Treatment with steroids showed improvement in cardiac contractile function and mitral valve vegetations disappeared over 3 months. In a case of pancarditis with culture negative endocarditis, SLE as a possibility must be explored. After excluding infective endocarditis, a short trial of high dose corticosteroids effects overall improvement in carditis of SLE.     

Intestinal pseudo-obstruction in systemic lupus erythematosus: an analysis of nationwide inpatient sample

Clinical Rheumatology - Intestinal pseudo-obstruction (IPO) is a rare gastrointestinal complication of systemic lupus erythematosus (SLE). We aimed to determine the prevalence and in-hospital...     

Intestinal pseudo-obstruction in systemic lupus erythematosus: an uncommon but important clinical manifestation

OBJECTIVES: To document intestinal pseudo-obstruction (IpsO) as a recognised clinical manifestation of systemic lupus erythematosus (SLE) and a possible new clinical entity with its apparent association with ureterohydronephrosis. METHODOLOGY: We report six lupus patients who presented with IpsO and review 12 other cases from an English literature search. IpsO is defined as the presence of clinical features suggestive of intestinal obstruction but without organic obstruction, namely absence of bowel sounds, presence of multiple fluid levels on plain abdominal X-rays and exclusion of organic obstruction by imaging or surgical procedure. Other clinical characteristics related to the underlying lupus, serological and histological findings, treatment modalities and outcomes of these patients were reviewed. RESULTS: All 18 patients fulfilled the ACR revised classification criteria for SLE. None showed any clinical features of scleroderma or overlap syndrome. The mean age of onset of IpsO was 29.0 (15-47) y. The female to male ratio was 16:2. Nine patients had IpsO as the initial presentation of their underlying lupus. Coexisting lupus involvement of other organ systems included glomerulonephritis (n=7), thrombocytopenia (n=5) and cerebral lupus (n=3). The serology data and autoantibody profile of some of the previously reported patients were incomplete. In our series, anti-Ro antibody was positive in 5/6 while anti-RNP was found in 1/6 patients only. All our patients had active lupus serology at presentation. 17/18 patients required the use of high dose systemic corticosteroid therapy while one patient responded to topical adrenocorticotrophin hormone treatment. Response was good and was observed early after commencement. Azathioprine was used as maintenance therapy in 6/18 patients with good effects. An apparent association with the presence of bilateral ureterohydronephrosis was found in 12/18 patients. These patients presented with dysuria without positive bacterial culture though features of chronic interstitial cystitis were not invariably found in these patients. CONCLUSION: IpsO is an uncommon but important manifestation of SLE. The underlying pathology is not fully understood but it may be related to immune complex deposition. The finding of coexisting ureterohydronephrosis suggests that there may also be a central smooth muscle motility problem of neuropathic or myogenic pathophysiology which may or may not be secondary to vasculitis. Early recognition and treatment of IpsO in SLE is important.     

Intestinal transplantation in children with chronic intestinal pseudo-obstruction

BACKGROUND: Children with chronic intestinal pseudo-obstruction (CIPO) often require total parenteral nutrition (TPN) which puts them at risk of liver failure and recurrent line infections. Intestinal transplantation has become a therapeutic option for TPN dependent children with intestinal failure who are failing management with TPN. AIMS: To investigate the outcome of children with CIPO referred for intestinal transplantation. METHODS: A retrospective review was carried out of records and diagnostic studies from 27 patients with CIPO referred for intestinal transplantation. RESULTS: Five children were not listed for transplantation: two because of parental decision, two because of suspicion of Munchausen syndrome by proxy, and one because he tolerated enteral nutrition. Six are still TPN dependent and awaiting transplantation. Eight children died awaiting transplantation. Eight children underwent transplantation. Three died (two months, seven months, and four years after transplant). Five children are alive with a median follow up of 2.6 years (range two months to six years). All transplanted children were able to tolerate full enteral feedings. The postoperative course was complicated by dumping syndrome, Munchausen syndrome by proxy, narcotic withdrawal, and uncovering of achalasia. Conclusion-Intestinal transplantation may be a life saving procedure in children with CIPO. Early referral and thorough pretransplant evaluation are keys to successful transplantation.