蛋白丢失性肠病61例临床分析

目的 通过对蛋白丢失性肠病临床资料的总结分析,提高对本病的认识.方法 对北京协和医院1997至2009年诊断的61例蛋白丢失性肠病进行分析总结.结果 男26例,女35例,年龄16～77(40±15)岁.水肿为首发症状51例;腹水为主要症状41例;合并双侧胸腔积液23例;腹痛16例,腹泻33例;所有患者均有显著的低蛋白血症.37例患者经核素99Tcm标记白蛋白显像证实存在肠道蛋白丢失,24例为临床诊断.原发病主要为系统性红斑狼疮(28例),先天性淋巴管扩张(12例).治疗上以原发病治疗为主.结论 蛋白丢失性肠病临床并非罕见,以严重的低蛋白血症和多浆膜腔积液为特征,核素99Tcm标记白蛋白显像是特异性的诊断方法之一,治疗上以原发病治疗为主,预后与原发病控制与否相关.

Abstract：

Objective To increase the understanding in protein-losing enteropathy (PLE).Methods Sixty-one PLE patients were enrolled in the study and the clinical characteristics, complicated disease, diagnosis and treatment were analyzed. Results The age of the patients was 16-77 (40±15)years, and the gender ratio was 35:26 (female: male). The main clinical manifestations were bilateral lower limb edema in 51 cases, ascites in 41 cases, bilateral pleural effusion in 23 cases, pericardial effusion in 13cases, abdominal pain in 16 cases and diarrhea in 33 cases. The prominent abnormality in laboratory examinations was hypoalbuminemia. The underlying diseases include systemic lupus erythematosus (SLE) in 28 cases, intestinal lymphangiectasia in 12 cases, hepatic cirrhosis in 5 cases, heart diseases in 5 cases,Crohn's disease in 3 cases, membranous nephropathy in 2 cases, Budd-Chiari syndrome in 1 case. Four cases happened after abdominal operation and 1 case after radiation therapy of gastric cardia cancer. Thirtyseven cases were diagnosed by 99Tcm-labelled human serum albumin scintigraphy and 24 cases were diagnosed clinically. Treatment was focused on underlying diseases. The clinical manifestations in 21 cases of SLE improved after SLE was controlled. In 2 cases of intestinal lymphangiectasia and one with Crohn's disease, the clinical manifestations improved after surgery. The other patients had no improvement.Conclusions PLE was not uncommon in clinical practice. Its predominant characteristics were severe hypoalbuminemia, edema and dropsy of serous cavity. PLE can complicate other diseases such as SLE,intestinal lymphangiectasia. Treatment should be focused on primary disease.     

Outcome of surgical resection for protein-losing enteropathy in systemic lupus erythematosus

Protein-losing enteropathy (PLE) is an uncommon manifestation associated with systemic lupus erythematosus (SLE). Here, a case with SLE and concomitant hypoalbuminemia is reported. Technetium-99m albumin scintigraphy demonstrated a localized lesion in the ascending colon, and the diagnosis of SLE-related PLE was established. Due to a poor response to medical treatment, this patient received surgical resection, but relapse still developed later on. Recurrent protein-lose from the remaining of the colon was documented by repeated images. This report discusses the management of SLE-related PLE and the role of nuclear medicine scintigraphy in the investigation of PLE.     

Lupus protein-losing enteropathy (LUPLE): a systematic review

Lupus protein-losing enteropathy (LUPLE) is a well reported but a rare manifestation of systemic lupus erythematosus (SLE). The main objectives of this study are to raise awareness of LUPLE that can be easily missed by internist, rheumatologist, gastroenterologist and nephrologist, and then to be considered in any patient with unexplained edema, ascites, and hypoalbuminemia. A systematic review was performed with 112 patients who met the eligibility criteria and were critically appraised. The LUPLE was ultimately diagnosed by either Tc-^99m albumin scintography (^99mTc-HAS) or fecal alpha-1-antitrypsin clearance test. Clinical features of patients, at the time of LUPLE diagnosis, were as follows: age was 34 ± 14.2 years; the female to male ratio was 5.8:1; the mean time to development of LUPLE after diagnosis of SLE was 4.19 ± 4.7 years. There was a predominance of Asian (64.7%) while 29.5% were white or Hispanic patients. Eighty percent had peripheral edema, 48% had ascites, 38% had pleural effusion, and 21% had pericardial effusion. Forty-six percent had diarrhea, 27% had abdominal pain, 22% had nausea, and 19% had vomiting. Hypoalbuminemia was the most common characteristic laboratory finding (96%). A 24-h urine protein was less than 0.5 gm in (71%). Almost all patients (96%) had positive ANA with predominant speckled patterns (55%) and hypocomplementemia (79%). Colonoscopy showed mucosal thickening in 44% of patients, and the majority of patients (52%) revealed no abnormalities; on the other hand, intestinal histology either revealed mucosal edema, inflammatory cell infiltrate, lymphangiectasia, mucosal atrophy or vasculitis in 80% of patients. All patients were started on steroids. Thirty-four percent responded to steroids alone. Sixty-six percent were started with other immunosuppressive therapies, which include cyclophosphamide (46%), azathioprine (33%), and a combination of cyclophosphamide and azathioprine (7%). A few reported cases responded to either cyclosporine or etanercept. Prognosis was very good with steroids combined with immunosuppressive therapy. This is the first systematic review of LUPLE and should be considered as an etiology of unidentified edema, ascites, and hypoalbuminemia.     

Exudative enteropathy in disseminated lupus erythematosus. Report of 3 cases and review of the literature

Protein-losing enteropathy (PLE) is characterized by loss of essentially protein substances into the gastrointestinal tract. Few reports of PLE supervening in patients who have systemic lupus erythematosus (SLE) have appeared in the literature. We report three new cases. All three were women who had a severe form of SLE involving several organs. PLE was diagnosed on the basis of an increased clearance of alpha 1 antitrypsin. The severeness of the clinical picture in all three patients justified the use of immunosuppressive agents (corticosteroids and pulse cyclophosphamide therapy) which were effective. These cases are compared to the 24 previously reported. The frequency of PLE during an SLE flare-up is probably underestimated. It should be looked for in SLE patients who have edema by means of the simple alpha 1 antitrypsin test. PLE is often found in severe clinical forms of SLE and should be managed using corticosteroids either alone or in association with immunosuppressive drugs.     

Protein-losing enteropathy exacerbated with the appearance of symptoms of systemic lupus erythematosus

A 38-year-old woman visited our hospital with edema on her face and conjunctivae. The underlying disease was not clarified, and she did not visit the hospital afterwards. She suffered from diarrhea, polyarthralgia, Raynaud's phenomenon, malar rash and hair loss in the subsequent two years, and was hospitalized because of hypoproteinemia. Her urine, liver and heart test results did not account for her hypoproteinemia. She was diagnosed as having protein-losing enteropathy (PLE) associated with SLE based on the 99mtechnetium-labeled human serum albumin scintigraphy findings, clinical findings and laboratory results of antinuclear and anti-Sm antibodies. This case report demonstrates a strong association between PLE and SLE because PLE was aggravated along with the appearance of SLE symptoms and PLE subsided with prednisolone treatment along with improvement of SLE.     

Entéropathies exsudatives : à propos de cinq observations et revue de la littérature

Objective

Protein-losing enteropathy (PLE) is a rare entity with multiple etiologies. The diagnosis is confirmed by the elevation of faecal alpha-1-antitrypsin clearance. We report five cases of PLE and review the clinical characteristics and prognosis factors.

Methods

We retrospectively reviewed the medical report of 5 patients with PLE seen at the Department of internal Medicine between Hedi Cheker Hospital between 1996 and 2012.

Results

Five women with a mean age of 44.8 years (25–70 years) were studied. The initially suggestive clinical symtomatology was lower edema was in 3 cases, ascites in a patient while EE was discovered incidentally in another case. There were no gastrointestinal symptoms in all cases. Biologically, hypoproteinemia with hypoalbuminemia was found in all patients, hypogammaglobulinemia in 3 patients and lymphopenia in 3 cases. Hypocalcemia was present in one case, moreover, there was no digestive malabsorption in others cases. Renal function was normal without proteinuria in all cases. PLE was confirmed by the elevation of the clearance of alpha -1-antitrypsin in all patients. The investigations revealed systemic lupus erythematosus (SLE) in one case, a duodenal lymphangiectasia associated with non specific ulcerative ileitis in another. Celiac disease was highly likely in a patient, an iatrogenic origin was implicated in another (magnesium hydroxide). However, no cause was found in the fifth patient. All patients received a high-protein diet with specific treatment in three cases. The outcome was good in four patients with resolution of edema and correction of laboratory abnormalities.

Conclusion

PLE is a rare entity with digestive or nondigestive causes. Dietary measurement is generally indicated associated with the treatments of the more common causes.     

A case of systemic lupus erythematosus presenting with protein-losing enteropathy.

We report an unusual case of systemic lupus erythematosus presented with protein-losing enteropathy. A 24-year-old girl was referred to our hospital with generalized edema, thrombocytopenia, hypoalbuminemia, hypercholesterolemia, hypocomplementemia, antinuclear antibody (ANA) (speckled pattern) and anti- SSA/Ro positivities, and elevated CA125 antigen appeared in the blood examination. On the radiological studies, she had mild pleural effusion and moderate ascites which were transudate. A diagnosis of protein-losing enteropathy was made on the basis of increased 99mTc-labelled human immunoglobulin scintigram showing abnormal radioactivity. Endoscopic gastric, duodenal and jejunal biopsies showed chronic inflammation, but vasculitis and immune complex deposition findings were not present. Renal biopsy revealed no definitive findings of lupus nephritis. By the administration of corticosteroids, hypoalbuminemia began to improve, but steroid doses were decreased due to steroid-induced myopathy. Temporary hemiparesis and facial paralysis developed in the patients' follow up. Her cranial magnetic resonance imaging revealed chronic ischemia, and the patient was considered to have neurological involvement due to systemic lupus erythematosus. protein-losing enteropathy and other symptoms then improved dramatically after monthly intravenous cyclophosphamide (three times) combined with oral low-dose corticosteroids. The combination of azathioprine and low-dose steroids was used as maintenance medication. Although about 30 protein-losing enteropathy -associated systemic lupus erythematosus cases have been reported, the patients having initial symptoms as protein-losing enteropathy are rare in the literature. Protein-losing enteropathy -associated systemic lupus erythematosus cases probably represent a subgroup of systemic lupus erythematosus, the characteristics of which are hypocomplementemia, protein-losing enteropathy, ANA positivity showing speckled pattern and anti-ds DNA negativities. In the patients with systemic lupus erythematosus with edema and hypoalbuminemia without renal protein loss, protein-losing enteropathy-associated systemic lupus erythematosus should be kept in mind.     

蛋白丢失性肠病的临床特点分析

目的概括蛋白丢失性肠病(protein losing enteropathy,PLE)的临床特点及诊治情况。方法以"蛋白丢失性肠病"或"小肠淋巴管扩张"为主题词,在万方、维普、CNKI检索我国2000～2010年公开发表的PLE病例报道。结果纳入的77份病例均除外摄入不足、肝脏合成减少和肾脏丢失,并且证实蛋白从肠道丢失或有影像学和内镜下的诊断依据。其中男30例,女47例,性别比为1∶1.57,成人起病平均年龄(41.06±5.88)岁,儿童起病平均年龄(5.57±2.33)岁。男女起病年龄与病程差异均无统计学意义。本文PLE的病因儿童组以小肠淋巴管扩张症(55.6%)为主,成人组以系统性红斑狼疮(43.9%)为主。PLE的首发症状为浮肿(84.4%),其次分别为腹泻(48.1%)、腹胀(31.2%)、腹痛(28.6%)、消瘦乏力(18.2%)等。结论国内PLE以中年为主,女性多见,起病年龄与病程无性别差异。由于缺乏特异性的临床表现,容易误诊为其他疾病。病程迁延,但预后相对较好。     

Mixed connective tissue disease associated with protein losing enteropathy: successful treatment with intravenous cyclophosphamide therapy.

A patient with mixed connective tissue disease who developed protein losing enteropathy (PLE) is described. The PLE and other symptoms improved dramatically after monthly intravenous administration of 700 mg/day cyclophosphamide three times combined with oral prednisolone, while they were ineffective to the treatment with intravenous methyl-prednisolone 500 mg per day for 3 days. The serum level of CA125 antigen paralleled the severity of symptoms, signs and laboratory data associated with PLE. Thus, pleural effusion, ascites, edema and hypoalbuminemia improved along with the decrease in the level of CA125, suggesting that CA125 might be a marker of the activity of PLE.     

Cutaneous lesions of the digits in systemic lupus erythematosus: 50 cases

The objective of this study was to observe the clinical and pathologic features of digital lesions in a cohort of 50 patients with systemic lupus erythematosus (SLE). Biopsy and pictures of digital lesions were performed in 50 consecutive patients with SLE and digital lesions. A clinical diagnosis of vasculitis was previously suggested in 36% of cases. Pictures were reviewed by three dermatologists and all the tissue sections were analysed by the same pathologist. Files of patients were reviewed retrospectively. Activity of SLE was established according to the lupus activity index (LAI). Digital lesions in SLE were frequently painful (60%) with a finger-pulp inflammation (70%). According to clinical and pathological correlation, five patients had acute cutaneous lupus, five subacute cutaneous lupus, 21 discoid lupus and 15 chilblain lupus. Two patients presented vasculitis: one had an urticarial vasculitis concomitantly to a lupus flare, the other had an erythema elevatum diutinum, independent of SLE evolution. Thrombosis of dermal vessels was present in two patients with SLE-associated antiphospholipid syndrome and in two patients with chilblain lupus. LAI was >1.5 in only seven patients. These results highlight the tendency to clinically overestimate the prevalence of cutaneous vasculitis of the fingers in patients without active SLE. Clinical features of cutaneous lupus of the digits are polymorphous. So, a pathological examination of the lesions is often necessary for diagnosis and proper management.     

MALT lymphoma of the small bowel with protein-losing enteropathy

Mucosa-associated lymphoid tissue (MALT) lymphoma usually arises from chronic inflammation. We herein report a case of small intestinal MALT lymphoma with protein-losing enteropathy (PLE). A 73-year-old woman presented with lower leg edema and severe hypoalbuminemia. She had a medical history of pylorus-preserving pancreaticoduodenectomy with Billroth II reconstruction. Oral and anal route double-balloon enteroscopies revealed irregular nodular mucosal lesions with erosion extending from the jejunum to terminal ileum. Histopathological evaluation of the biopsied mucosa showed proliferation of small-to-medium-sized lambda light chain-restricted B cells. Plasmacytic differentiation and lymphoepithelial lesions were present, leading to the diagnosis of MALT lymphoma. Tc-99m albumin scintigraphy indicated tracer exudation in the small bowel, suggesting the presence of PLE. Combination immunochemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) regimen improved both MALT lymphoma and PLE, whereas rituximab monotherapy was not successful. This case is considered to be common type of MALT lymphoma at an uncommon site and is distinct from immunoproliferative small intestinal disease (IPSID). To our knowledge, this is the first case of non-IPSID-type small intestinal MALT lymphoma complicated by PLE. Gastrointestinal reconstruction may be responsible for underlying chronic inflammation via small intestinal bacterial overgrowth.     

Protein-losing enteropathy and premature ovarian failure in a young woman with systemic lupus erythematosus

Protein-losing enteropathy (PLE) and autoimmune oophoritis are unusual manifestations of systemic lupus erythematosus (SLE). Autoimmune oophoritis may result in menstrual disturbance and spontaneous premature ovarian failure. However, there is no validated examination to confirm the diagnosis and it is easily neglected in patients with ovarian insufficiency. A 31-year-old woman with SLE presented with PLE and autoimmune oophoritis during the long course of flares and remissions in her lupus activity. The synchronism implied the association between the two. Moreover, both conditions simultaneously had a good response to cyclosporine A (CsA) therapy.     

系统性红斑狼疮患者并发心律失常的类型及相关危险因素分析

目的 分析系统性红斑狼疮（SLE）患者并发心律失常的类型及相关危险因素。方法 回顾性分析2003年11月至2013年2月期间经本院确诊为SLE的559例住院患者（男60例,女499例）,将其分为心律失常组和非心律失常组,收集各项检查检验指标,采用多因素分析SLE并发心律失常的独立危险因素。结果 559例SLE患者中有142例（25. 4%）并发心律失常。其中以窦性心动过速所占比例最高（56. 34%）,其次为窦性心动过缓（16.9%）,再其次为I度房室传导阻滞和室性早搏（均为6. 34%）,其余为其他心律失常（0. 7% ~3. 5%）。单因素分析显示,SLE患者合并心律失常的危险因素有合并多系统损害、其他结缔组织病、心包积液、左房扩大、高甘油三酯、高血糖、低高密度脂蛋白、低血浆白蛋白、低钙血症、高C反应蛋白、抗Sm阳性及抗RNP阳性（P〈0. 05）。多因素分析显示,独立危险因素有抗RNP阳性、左房扩大及低血浆白蛋白（P〈0. 05）。结论 SLE患者可并发各种类型心律失常,其中以窦性心动过速最为常见;独立危险因素有抗RNP阳性、左房扩大及低血浆白蛋白。     

Analysis of 15 patients with systemic lupus erythematosus manifesting with negative immunofluorescence anti-nuclear antibodies after treatment

The purpose of this study was to investigate the clinical and laboratorial characteristics of patients with systemic lupus erythematosus (SLE) manifesting with negative immunofluorescence anti-nuclear antibodies (IFANA) after treatment for the better understanding of negative conversion of IFANA. Demographic characteristics, clinical and laboratory data of hospitalized SLE patients between March 2006 and May 2011 were retrospectively reviewed. Fifteen cases with negative IFANA were identified in 960 patients. All of the 15 patients were severe, 11 patients manifested with nephritic range proteinuria and hypoalbuminemia, 8 patients were complicated with severe infection and all of the patients had been treated with glucocorticoid and immunosuppressant. Anti-ENA antibodies were positive in 4 of 15 patients. Eight patients died after average 1-year follow-up. Collectively, negative IFANA is mainly attributed to nephritic-range proteinuria; and large-dose glucocorticoid, immunosuppressant and severe infection are also important factors for negative IFANA. Antinuclear antibody can be detected in some SLE patients with negative IFANA by changing the detection method and titer. Negative conversion of IFANA often indicates unfavorable prognosis for severe patients.     

系统性红斑狼疮的心脏损害

目的：探讨系统性红斑狼疮（SLE）的心脏受累表现，并分析年龄、性别，病程及抗心磷脂抗体（ACL）与SLE心脏损害的相关性。方法：回顾性总结分析1998年9月至2000年8月我院诊断SLE的入院病人272例，结果：272例SLE病人中145例（53．3％）具有心脏损害，10例病人具有心脏病相关症状（6．7％），心脏损害包括心电图（ECG）ST－T异常61例（42．1％），心包积液60例（41．4％），肺动脉高压23例（15．9％），心肌损害22例（15．2％），心律失常20例（13．8％），瓣膜病19例（13．1％），左心耳血栓1例（0．6％）。心脏损害组与无心脏损害组之间年龄，性别差异无差异性（P＞0．05，病程差异有显著性（P＜0．01），ACL阳性组与阴性组瓣膜损害发生率差异有显著性（P＜0．01），结论：SLE可以累及心脏各个部分，其中以ECC ST－T缺血性改变和心包受累最为常见。SLE累及心脏时多属无症状型，SLE心脏损害与病程有关，抗心磷脂抗休与心脏瓣膜损害关系密切。     

系统性红斑狼疮合并Evans综合征临床特点

目的总结系统性红斑狼疮(systemic lupus erythematosus,SLE)合并Evans综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月SLE合并Evans患者的临床表现及实验室特点及治疗和预后。结果 SLE并发Evans综合征患者22例,占同期SLE住院患者3400例的0.65%。其中男3例,女19例,平均35.1岁(16~53岁)。22例患者中以血液系统受累[特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)或自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)]为首发表现的11例(50%),确诊SLE后诊断Evans综合征者6例,二者同时诊断的5例。SLE并发Evans综合征时,患者往往有多系统受累,表现为肾脏受累13例(59.1%),皮肤黏膜受累、关节炎各9例(40.9%),神经系统受累4例(18.2%),胃肠道、肺部受累各2例等。Evans综合征多发生于SLE活动期,患者平均狼疮活动指数评分(11.45±7.6)分(3~30分)。伴发其他结缔组织病5例(22.7%)。经激素联合免疫抑制剂治疗后,20例好转,2例无效者应用利妥昔单抗后好转。结论 SLE合并Evans综合征罕见,发生于SLE多系统受累及活动期。部分患者以ITP或AIHA为SLE首发表现,应及时筛查多种自身抗体,并定期随访密切观察,以期早期诊治。     

An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. We report here an unusual case of seronegative SLE presented as vasculitis with rash, lower gastrointestinal system bleeding and acute renal failure. The patient was a 13-year-old boy, with abdominal distention, pretibial edema, arthritis and petechia on bilateral ankles. He had deteriorated renal functions (creatinine 1.65 mg/dl), hypoalbuminemia (1.6 g/dl) and hypocomplementemia with nephrotic range proteinuria and hematuria. He developed pleural effusion and peritonitis. Serum ANA, anti dsDNA, p ANCA, c ANCA, anticardiolipin IgM and IgG titers were negative. A renal biopsy was performed which revealed diffuse proliferative glomerulonephritis with full-house staining pattern in immunofluorescent microscopic examination suggesting Class IV Lupus Nephritis. He was administered a total of six courses of monthly intravenous pulse methyl prednisolone, dipyridamole, oral cyclophosphamide followed by azothiopirine and oral prednisolone therapy. The renal functions and serum albumin levels turned normal but peritonitis persisted and disappeared after the third pulse steroid therapy. In conclusion, we presented this patient to remind the possibility of SLE in such seronegative patients with unusual findings in order to avoid the delay in the management of this disease with high mortality and morbidity if not treated. Full-house nephropathy is an important clue especially for the diagnosis of ANA negative SLE.     

Features of systemic lupus erythematosus in patients with autoimmune hepatitis

Although systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) are distinct diseases, in clinical practice differentiation of one from other may be difficult. The aim of this study was to asses features of SLE in patients with diagnosis of AIH.Thirty patients [mean age: 52.4 ± 11.8 years; 23 (76.7 %) female] were included in the study. Seven (23.3 %) of the patients full filled 4 or more criteria for classification of SLE. None of the patients had muco-cutaneous lesions characteristic to SLE. Three patients had rheumatoid factor negative arthritis, and 2 patients had pericardial effusion. Four patients had significant thrombocytopenia (<100 × 10³/μL), and one of these patients had pancytopenia. None of the patients had hematuria, but 3 patients had proteinuria which did not affect renal function during the study period. One patient died due to pancytopenia-associated pulmonary infection. Among the treated patients with SLE features, 2/5 (40 %) achieved ALT normalization and 9/12 (75 %) of the remaining patients achieved ALT normalization (Fisher’s exact test; p = 0.28) during the study period. Although the difference is non-significant, treatment response of AIH patients with SLE features seemed to be delayed and incomplete compared to other patients, but with the limited number of patients it is inconvenient to reach a definitive conclusion. Further studies are needed to identify role of features of SLE on treatment response in patients with AIH.     

Clinical characteristics of concurrent and sequentially presented lupus-related protein-losing enteropathy: What are their differences?

Our objective was to compare patients with concurrent and sequentially presented systemic lupus erythematosus (SLE)-related protein-losing enteropathy (PLE). Patients with history of SLE admitted for PLE were selected and their clinical, laboratory, endoscopic and imaging characteristics, treatment and outcome were analyzed. From 2001 to 2010, 21 and 27 patients had concurrent and sequentially presented SLE-related PLE, respectively, and their clinical characteristics were comparable except the following: the concurrent group had more pleural effusion (P < 0.01), cutaneous (P < 0.03), neurological (P = 0.02) manifestations, higher creatine phosphokinase (127.6 IU/L vs. 105.7 IU/L, P < 0.05) and lactate dehydrogenase (504.0 IU/L vs. 422.2 IU/L, P < 0.05); whereas the sequential group had higher anti-double strand DNA titer (179.8 vs. 100.4, P < 0.05), 24-h urine protein excretion (1.1 g/d vs. 0.6 g/d, P < 0.05) and increased proteinuria after onset of PLE (0.21 g/d vs. 1.1 g/d, P < 0.04). The endoscopic, histological and radiological features were comparable between the two groups. More patients from the sequential group required more potent immunosuppressive therapy for induction (55.6% vs. 14.3%, P = 0.002) and maintenance (48.2% vs. 9.5%, P < 0.01).The concurrent group associated with better treatment outcomes, with requiring shorter mean time (4.5 months vs. 7.9 months, P = 0.03) for normalbuminemia and more individuals (90.5% vs. 63%, P < 0.02) achieving normalbuminemia in first year. The complications were infrequent: two drug-related adverse events from each group, one patient each from the concurrent group developed shingle and SLE nephropathy. PLE associated with concurrent and sequentially presented of SLE are comparable in clinical behavior; and the immunosuppressive therapy is generally well-responded and tolerated. However, the concurrent group is associated with better disease activity control.     

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.