Protein-losing enteropathy in systemic lupus erythematosus: analysis of the clinical features of fifteen patients.

OBJECTIVE: Protein-losing enteropathy (PLE) is an unusual manifestation of systemic lupus erythematosus (SLE), so its clinical manifestations and management are not well understood. In this study, we try to characterize the basic clinical features and the management of PLE by retrospectively analyzing the clinical data of 15 PLE patients and hope this study can improve the awareness of PLE in lupus patients with severe hypoalbuminemia that could not be explained by other causes. METHODS: The clinical data of 15 SLE patients with PLE hospitalized during November 2001 and April 2006 in Peking Union Medical College Hospital were retrospectively reviewed. The PLE was diagnosed by Tc-99m albumin scintigraphy (99mTc-HAS). The clinical characteristics, laboratory tests, response to treatment, and the outcome were studied. RESULTS: The mean age of PLE onset was 40.1 +/- 15.4 years (19-71 years). Twelve were female and 3 were male. 53.3% (8 of 15) patients had PLE as the initial presentation of SLE. All patients had different degree of peripheral pitting edema. Eleven had ascites, 9 had pleural effusion, and 7 had pericardial effusion. Only 6 patients presented with abdominal pain and diarrhea. Positive antinuclear antibodies (HEP-2) with a speckled pattern were found in all patients, but the antidsDNA antibody was negative in most cases. All patients had marked hypoalbuminemia, 80% had hypocomplementemia, 66.7% had hyperlipoproteinemia, and 40% had hypocalcemia. The liver function tests and the prothrombin time were in normal ranges. The 24-hours urine protein was less than 0.5 g in 60% (9 of 15) and more than 1.0 g in 20% (3 of 15) patients who were renal biopsied but only found to have very mild pathologic changes. Gastrointestinal endoscopy examination discovered generalized edema in the intestinal wall whereas the biopsy showed chronic inflammation only. Most cases had good response to corticosteroid and immunosuppressive therapies. The serum albumin level improved evidently in all patients after treatment and normal scintigraphic finding was found in 9 patients. CONCLUSION: PLE can be the initial presentation of SLE or can develop a very long time after the diagnosis of SLE. The prominent clinical presentations are caused by hypoalbuminemia. 99mTc-HAS is useful not only for the diagnosis of PLE but is also helpful for monitoring the efficacy of treatment. When a SLE patient presents with evident hypoalbuminemia without evidence of other causes, PLE should be considered. Early diagnosis and treatment may improve the prognosis.     

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.     

Protein-losing enteropathy associated with systemic lupus erythematosus: response to cyclophosphamide

Protein-losing enteropathy is a rare manifestation of systemic lupus erythematosus (SLE) leading to hypoalbuminemia and anasarca. We report the case of a woman with SLE who presented chronic hypoalbuminemia diagnosed as protein-losing enteropathy associated with SLE. She was refractory to prednisone and azathioprine administration but showed good response to cyclophosphamide. The diagnosis and management of hypoalbuminemia in lupus-associated enteropathy are discussed.     

Changes in the survival of patients with systemic lupus erythematosus in childhood: 30 years experience in Chile

The objective of this study was to analyse the survival rate and cause of death in children with systemic lupus erythematosus (SLE) during the past 30 years in Chile. A retrospective analysis was performed between 1969 and 2000 on patients attending pediatric rheumatology centres in Santiago, Chile. Survival and causes of death in 31 children followed from 1969 to 1980 fulfilling the 1982 American College of Rheumatology criteria for SLE and treated with oral steroids were compared with 50 other patients who were treated with oral steroids and an aggressive treatment of IV bolus of cyclophosphamide (38 patients) and azathioprine (12 patients). Global survival at five and 10 years follow-up for the patients studied from 1969 to 1980 was 68 and 40%, respectively. During the second study period these values were significantly improved and global survival reached 95% at five years and 90% at 10 years follow-up (P < 0.05). Survival at 10 years follow-up for patients with lupus nephropathy increased from 28% (study period 1964-1980) to 86% (study period 1984-2000). Twelve children died (38%) during the 1964-1980 study period. The causes of death were six due to kidney failure, three due to infectious conditions and another three of unknown causes. During the 1980-2000 study period mortality reached 6% (three cases), two cases died of a lupus flare-up and one case due to infection. In the last three decades, we have seen an important increase in the survival of children with SLE, especially in those patients with renal involvement. Management with immunosuppressive drugs, such as IV cyclophosphamide or azathioprine has changed the prognosis in these children. These results demonstrate that our children with SLE increased their life expectancy but are now faced with new types of morbidity because of the sequelae related to the disease itself.     

Treatment of acquired factor VIII inhibitor using intravenous immunoglobulin in two patients with systemic lupus erythematosus

Acquired inhibitors of factor VIII have rarely been reported in the presence of systemic lupus erythematosus (SLE). Treatment is usually corticosteroids, with or without immunosuppressive agents such as cyclophosphamide or azathioprine. We report here case histories of 2 patients, one with documented SLE, the other with a forme fruste of SLE. Both patients had inadequate responses to corticosteroids and immunosuppressive agents. Both responded to intravenous immunoglobulin, with a decrease in their titers of factor VIII inhibitor and significantly decreased frequency of bleeding episodes despite persistent low-level inhibitor titers. Better control of their SLE symptoms and findings was also observed. Previously reported treatments of acquired factor VIII inhibitors in SLE are discussed.     

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.     

Characteristics and long-term outcome of 15 episodes of systemic lupus erythematosus-associated hemophagocytic syndrome

ABSTRACT: Reactive hemophagocytic syndrome (HS) occurs mainly in the setting of serious infections and lymphomas. HS can occur in the course of 2 active systemic diseases, without simultaneous infection: adult Still disease and systemic lupus erythematosus (SLE). Observations of specific lupus-associated HS are rare, and the long-term outcome of these patients with active SLE is unknown.We retrospectively studied 15 episodes of SLE-associated HS in 12 patients (10 women, 2 men) and noted the long-term outcome. HS occurred at a mean age of 25 years. All patients were febrile with >or=2 cytopenias, and bone marrow aspiration indicated hemophagocytosis. HS revealed SLE in 9 patients and recurred in 3. The main features of SLE-associated HS were a low frequency of hepatosplenomegaly, a high frequency of heart involvement (5 pericarditis, 4 myocarditis requiring transfer to intensive care unit), and a low C-reactive protein level (mean, 15 mg/L). Cutaneous-mucous symptoms of SLE, arthritis, and nephritis were present respectively in 8 (53%), 6 (40%), and 4 (27%) episodes, but symptoms of SLE were absent in 4 episodes at admission. All patients had anti-nuclear antibodies when the HS occurred. Anti-double-stranded DNA antibodies were present in 12 episodes. Treatment was steroids in 14 cases but cyclophosphamide was the only treatment able to control HS in 2 cases. All the cases of SLE-associated HS were controlled by the immunosuppressive regimen. Intravenous immunoglobulins seemed poorly effective. No infectious agent was found.Clinical presentations of the 23 patients with SLE-associated HS described in the literature were reviewed and were similar to those of the current series. The mean follow-up was 88 months (range, 7-240 mo). One patient died at 15 months (sepsis). Among the 5 patients with a follow-up >8 years, 4 always had active disease. During the follow-up of SLE, immunosuppressive drugs were added in 8 patients (cyclophosphamide in 7, azathioprine in 3, mycophenolate mofetil in 2) with significant adverse drug reactions. In the long-term, SLE-associated HS seems to define a severe SLE form with frequent flares, possible HS recurrences, and the need for prolonged immunosuppression.     

Pattern of neuropsychiatric manifestations and outcome in juvenile systemic lupus erythematosus

The aim of this study was to study the neuropsychiatric (NP) manifestations, diagnostic evaluation, treatment and outcome in juvenile systemic lupus erythematosus (SLE). We reviewed the charts of all children with SLE and evidence of NP manifestations as defined by the presence of at least one of the following: headache, cerebrovascular accident (CVA), chorea, seizure, papilledema, and psychiatric or spinal cord manifestations. Out of 90 children with SLE, 20 (16 female) had NP manifestations. The mean age at onset was 8.8 years. The mean period between onset of SLE and NP manifestations was 10.2 months. NP manifestations were the presenting feature in 3 patients. Eleven patients had headache, 10 had psychiatric manifestations, 10 had seizure and 6 had CVA. Coma was seen in 5 patients, chorea in 4, transverse myelitis in 2 and papilledema in 2. Anticardolipin antibodies were high in 12 patients. Five patients had an abnormal CSF study. Nine patients had EEG abnormalities and 13 showed MRI abnormalities. All patients received oral prednisone and 17 were treated with IVMP and immunosuppressive therapy (cyclophosphamide or azathioprine); 85% of our patients recovered completely, but 15% had persistent NP sequelae; 10% died from severe infection. In conclusion, NP involvement in juvenile SLE is common. However, early diagnosis and early treatment with adjunctive intravenous pulse cyclophosphamide may improve the outcome.Abbreviations CVA Cerebrovascular accident - IVMP Intravenous methylprednisolone - NP Neuropsychiatric - SLE Systemic lupus erythematosus     

系统性红斑狼疮合并血栓性血小板减少性紫癜14例临床分析

 目的 探讨系统性红斑狼疮（SLE）合并血栓性血小板减少性紫癜 (TTP) 的临床特点、诊断及治疗。方法 回顾性分析14例SLE合并TTP者的临床表现、实验室检查结果、诊治和转归。结果 14例SLE合并TTP者中,男4例,女10例,中位年龄23（17～69）岁。5例以SLE首发,9例两者同时发病。所有患者均有血小板减少、微血管病性溶血性贫血,发热、神经系统异常、肾功能损害的发生比例分别为12/14、11/14、11/14,其中8例患者具有上述五联征。糖皮质激素（单用或联合静脉注射人免疫球蛋白和环磷酰胺）治疗6例,糖皮质激素联合血浆置换8例,有效率分别为2/6、6/8。8例患者存活,6例死亡。对6例存活者进行了随访,均无复发。结论 SLE与TTP临床表现相似,多次重复血涂片检查对早期明确诊断SLE合并TTP十分重要。SLE合并TTP者肾功能损害程度比单纯TTP或SLE者更为严重,早期诊断并及时应用糖皮质激素联合血浆置换有助于改善患者的预后。     

Systemic lupus erythematosus and antineutrophil cytoplasmic antibodies-associated vasculitis overlap in an elderly woman: A case-based literature review

BackgroundThe overlap of systemic lupus erythematosus (SLE) and anti-neutrophil cytoplasmic antibody (ANCA) associated-vasculitis (AAV) is a rare entity. Aim of the work. To report a rare case of overlap SLE and AAV complicated by small bowel perforations and nephritis.Case presentationAn 81-years-old Chinese woman presented with a two-weeks history of progressive bilateral lower limb weakness and dysuria. An incidental uterine mass was found, and a total hysterectomy was performed with extensive small bowel adhesion and multiple enteric perforations discovered intra-operatively. SLE was diagnosed based on the presence of cutaneous vasculitis, positive antinuclear antibody, anti-double stranded deoxyribonucleic acid, consumed complements, thrombocytopenia, nephritis, and pleural effusion. Positive perinuclear-ANCA and histological findings of the resected small bowel led to evidence of co-existing AAV. Hence, these findings have led to a diagnosis of overlap SLE and microscopic polyangiitis (MPA). The patient received daily hydroxychloroquine (200 mg), azathioprine (50 mg) followed by intravenous (IV) hydrocortisone (200 mg/8 h) and cyclophosphamide (750 mg/m²). The patient’s condition deteriorated with respiratory failure and hypotension and was eventually intubated and ventilated. IV immunoglobulin (4 mg/kg/day) was given for 3 days with resolution of the vasculitic lesions. The renal function rapidly declined with hemodynamic and clinical deterioration and the patient died.ConclusionThis case demonstrates the diagnostic conundrum and complexity in the management of a late presentation of an overlap syndrome with rare life-threatening complications. To our knowledge, this is the first case diagnosed and managed in Malaysia and the oldest patient diagnosed with overlap SLE/AAV in the literature.     

Life-threatening onset of systemic lupus erythematosus coincides with Kikuchi disease in a Croatian patient

BackgroundKikuchi disease (KD) or histiocytic necrotizing lymphadenitis is a benign self-limited extremely-rare disorder of unkown etiology. It is characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. The recognition of KD is crucial as it can be mistaken for or be associated with systemic lupus erythematosus (SLE).Case reportWe present a 29-year-old Croatian female admitted to the Hematology and Oncology Department, General Hospital Dr. Josip Benčević, Croatia in a life threatening condition. She was feverish (40 °C) with chills and weight loss. On clinical examination the patient had bilaterally enlarged cervical, axillary and inguinal lymph nodes with sizes up to 3 cm. Our differential diagnosis was SLE, lymphoma, sarcoidosis, Still’s disease, hemophagocytic syndrome and KD. An extensive workup was done to confirm one of these diagnoses. Methylprednisolone 100 mg iv (1.5 mg/kg) was initiated for 5 days and as the patient’s condition was severe, steroids were maintained. Lymph node biopsy histopathology was compatible with KD. Antinuclear antibody and anti-double-stranded-DNA were positive. The patient fulfilled the classification criteria for SLE. A diagnosis of SLE associated with KD was held. On CT scan there was bilateral pleural effusion and ascites. Brain MRI was compatable with lupus cerebritis. On steroids plus hydroxychloroquine the patient remarkably improved and remained in remission after 3 months.ConclusionPrompt diagnosis and treatment with steroids may save the life of SLE patients with KD and leads to a favorable outcome. Raising the awareness towards this possibly serious association is important.     

Événements infectieux au cours des vascularites nécrosantes systémiques : étude rétrospective de 82 cas

Purpose

The aim of this study was to assess the infections occurring in a series of 82 patients followed for a systemic necrotizing vasculitis and to determine potential risk factors.

Methods

We studied retrospectively the medical files of 23 Churg and Strauss syndrome, 18 periarteritis nodosa, 14 microscopic polyangiitis, and 27 granulomatosis with polyangiitis, over a 15-year period. Infection delay corresponded to the period from treatment to first infection or between two infections.

Results

A total of 61 patients developed 147 infections. Causal agent was identified in 70 cases, 42 were bacterial, 20 viral and 8 fungal. Bronchopneumonia was the most frequent infection (43 %). Sixty-two percent of infections occurred within 2 years after vasculitis diagnosis. Seven infections were major, requiring intensive care, with one infection-death related. Pneumocystis prophylaxis concerned 75 % of patients on cyclophosphamide. Significant factors reducing infection delay were initial hypergammaglobulinemia, hypoalbuminemia, lymphopenia, as well as cyclophosphamide and methotrexate treatment. Large quantities of corticosteroids, cyclophosphamide or azathioprine increased infection delay. This result underlines the early occurrence of infectious complications during vasculitis course.

Conclusion

Infectious events occurring in systemic necrotizing vasculitis are frequent and occurs early in disease course, and could be prevented with simple prophylactic measures. Vasculitis relapse and infection share similarities and this require permanent clinical vigilance.     

Pharmacogenetics of cyclophosphamide and CYP2C19 polymorphism in Thai systemic lupus erythematosus

To assess whether the CYP2C19 polymorphism modified the effect of cyclophosphamide on ovarian toxicity in Thai patients with SLE. We performed a case–control study of female patients with SLE who were treated with cyclophosphamide at Ramathibodi Hospital, Bangkok, Thailand. Cases were patient who had ovarian toxicity (sustained amenorrhoea >12 months or lack of menstruation for >4 months). CYP2C19 polymorphism was genotyped using PCR–RFLP method. Logistic regression was applied to assess CYP2C19 polymorphism as an effect modifier of cyclophosphamide. Seventy-one patients with SLE were enrolled, of which 36 (59.7%) had ovarian toxicity. CYP2C19*2 allele frequencies were 27.8 and 21.4% in the ovarian and non-ovarian toxicity groups. Patients with CYP2C19*1/*1 genotype and higher cumulative dose of cyclophosphamide (>23.75 g) had the highest odds of ovarian toxicity, i.e. 11.0 (95% CI: 1.2–99.1) times higher than patients with the CYP2C19*1/*2 or *2/*2 genotypes who received less cyclophosphamide (<23.75 g). After adjusting for age at start of treatment, this risk increased to 13.6 (95% CI: 1.1–162.2). Our results suggest that a cumulative cyclophosphamide dose of 23.75 g or higher carries a twofold higher risk of ovarian toxicity and the CYP2C19*1/*1 genotype increases the risk of toxicity a further fivefold.     

Therapeutic Plasma Exchange and Cyclosporine in the Treatment of Systemic Lupus Erythematosus

Despite treatment with intensive immunosuppressive drug regimens, often the prognosis of patients suffering from systemic lupus erythematosus (SLE) is poor. Side effects such as infections and malignant tumors often occur. In the present trial, 21 patients (4 male and 17 female, aged 37.9 ± 12.8 years) suffering from SLE for 9.4 ± 2.6 years, were treated for 2.3 ± 1.8 years with drug regimens of corticosteroids, azathioprine and/or cyclophosphamide. Then, over a period of up to 8 years, in addition to conventional therapies, especially in active stages of the disease with extremely high concentrations of anti-DNA-, antinuclear antibodies and circulating immunocomplexes, therapeutic plasma exchange (TPE) sessions were carried out depending on symptomatology. In addition patients received 2.5 ± 0.6 mg cyclosporine/kg body weight/day. Compared to previous treatment modalities, clinical symtoms improved more quickly and more effectively (p = 0.046). After 5 to 48 (17.5 ± 13.8) months, cyclosporine was established as a monotherapy for 8 of 21 patients. In the other cases, corticosteroids, azathioprine and cyclophosphamide were reduced by 40 to 100%. No severe side effects were seen. In acute stages of SLE and in forms with persistently high antibody levels, the addition of TPE sessions and cyclosporine as the basic immunosuppressive drug is usually very effective with regard to improving clinical symptomatology.     

Impact of cyclophosphamide on gonadotropins in menopausal systemic lupus erythematosus patients: Relation to disease activity and damage

BackgroundSystemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease that affects females at reproductive age, where lupus patients experience menopause at younger age. It is debated whether early menopause in lupus patients results from gonadotoxic effect of cyclophosphamide treatment or an autoimmune mediated ovarian injury.Aim of the workTo identify menopausal symptoms and characteristics in an Egyptian cohort of SLE females and its relation to disease activity, disease damage, lupus nephritis and treatment.Patients and methods120 consecutive SLE female patients above the age of 35 were studied. Disease activity was assessed by SLE Disease Activity Index (SLEDAI), and accumulated damage by Systemic Lupus International Collaborative Clinics-Damage Index (SLICC-DI). Laboratory assessment was done including follicle stimulating hormone (FSH) and luteinizing hormone (LH) levels.ResultsThe mean age of the patients was 45.1 ± 8.2 years (35–63 years) and the age at menopause was 45.2 ± 7.3 years (26–54 years). Their mean disease duration was 5.1 ± 5.7 years (1 month to 21 years). The mean SLEDAI was 4.7 ± 3.5 and SLICC-DI was 0.63 ± 0.82. 24 (20%) of patients had premature menopause, 29.2% had natural menopause and 50.8% were menstruating. There was a significant negative correlation between LH and SLEDAI. There was a significant correlation of FSH and LH with the cumulative cyclophosphamide dose.ConclusionSLE patients have early mean age of menopause at 45 years. High LH is associated with lower disease activity. High cumulative cyclophosphamide dose is associated with high FSH and LH. cyclophosphamide is potentially associated with premature menopause.     

Use of cyclophosphamide in the treatment of thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus: report of two cases

 Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). There are no randomized, prospective studies of its treatment. The association of plasma infusions and (or) plasmapheresis with steroids improves survival when compared with steroid treatment alone, but these patients still have a higher mortality than those with "classic" TTP. The role of immunosuppressive drugs in the management of this disorder remains uncertain. We report two cases of TTP in SLE which presumably benefited from the addition of cyclophosphamide to the treatment with plasmapheresis and steroids. Received: January 14, 1999 / Accepted: February 23, 1999     

Serum interleukin-26 is a promising biomarker in systemic lupus erythematosus patients: Particular relation to disease activity and nephritis

Aim of the workTo investigate the clinical utility of serum interleukin-26 (IL-26) in patients with systemic lupus erythematosus (SLE).Patients and methodsThe study was carried out on 42 SLE patients and 42 matched controls. SLE disease activity index 2000 (SLEDAI-2K) and Systemic Lupus International Collaborating Clinics Damage Index (SLICC-DI) were assessed. Serum IL-26 was measured.ResultsThe mean age of patients was 28.8±11.4 years with 78.6% females. 76.2% of patients were active; 7.1% very-high grade, 45.2% high, 16.7% moderate and 31% mild. 42.9% of patients had nephritis. The mean SLEDAI-2K was 10.4±6.4 and SLICC-DI 1.19±1.15. IL26 level was significantly higher in patients (64±76.4 pg/ml) compared to control (8.7±2.6 pg/ml), in active cases (79.2±81.9 pg/ml) compared to those inactive (15.3±4.8 pg/ml) and in those with nephritis (n = 18) (113.4±92.2 pg/ml) compared to those without (n = 24) (23.1±7.6 pg/ml).IL-26 level was significantly higher among cases receiving both steroids and mycophenolate mofetil than those receiving steroids with azathioprine (p = 0.005). There was a significant negative correlation between IL26 and serum albumin, hemoglobin and complement 3 (C3) levels (p < 0.001, p < 0.001 and p = 0.006 respectively). There was also a significant correlation between IL26 and both SLEDAI-2K (r = 0.95, p < 0.001) and SLICC-DI (r = 0.68, p < 0.001). At cut off value 16.3 pg/ml, IL26 differentiated patients and control; sensitivity 90.5%, specificity 100% (F. 2) and at 20 pg/ml detects active from non-active; sensitivity and specificity 100%.ConclusionIL-26 is a promising biomarker of SLE with high sensitivity and specificity. There is a relation of IL-26 with disease activity, damage and nephritis.     

Infection in systemic lupus erythematosus patients

BackgroundInfection is a leading cause of morbidity, mortality and hospital admission in systemic lupus erythematosus (SLE) patients.Aim of the workTo study infection in SLE patients regarding site of infection, pathogenic organism, hospitalization and/or intensive care unit (ICU) admission.Patients and methodsThis study included 79 patients. SLE disease activity index (SLEDAI-2K) and damage index were evaluated. Detailed information about the site of infection and pathogens were reported.Results71 females and 8 male patients (F:M 8.9:1), with a mean age of 29 ± 9.6 years (17–55 years) and disease duration of 5.9 ± 5.7 years, 55 (69.6%) patients had infection at time of study while 24 (30.4%) did not. The SLEDAI-2 k and damage index were significantly higher in SLE patients with infection (14.2 ± 11.8 and 3.7 ± 3.7) compared to those without infection (5.9 ± 5.03 and 1.8 ± 1.3) (p = 0.03 and p = 0.045 respectively). Those with infection had a shorter disease duration (4.9 ± 5.2 vs 8.3 ± 6.2; p = 0.005), received more cyclophosphamide (56.4% vs 16.7%; p = 0.001), higher erythrocyte sedimentation rate (ESR) (75.5 ± 27.1 vs 35.8 ± 24.7 mm/1^sthr) (p < 0.0001) and consumed complement (C3) (71.1 ± 28.4 vs 97.2 ± 28.2; p < 0.0001). 17/55 (30.9%) had more than one site of infection and 46/55 (83.6%) required hospital admission. 17 (30.9%) of hospitalized patients were transferred to the ICU. The main pathogenic organisms were bacterial (40%), fungal (27.3%), viral (10.9%) and unconfirmed in 21.8%. Chest was the commonest site (40%) followed by the skin (34.4%), oropharynx (25.5%) and urinary tract (20%).ConclusionInfection is an important cause of hospital and ICU admission in SLE patients. Early disease, disease activity and damage, cyclophosphamide, ESR and consumed C3 were associated with infection in SLE.     

Novel therapies in lupus nephritis

Renal disease continues to cause major morbidity and some mortality for around 30-40% of patients with systemic lupus erythematosus (SLE). Although the combinations of prednisolone and azathioprine or prednisolone and cyclophosphamide have been beneficial to many patients with SLE, they are not always effective and have significant side effects. It is very encouraging that new immunosuppressive drugs such as mycophenolate mofetil and more targeted therapies e.g., anti-CD20 are coming rapidly to larger scale clinical trials. The treatment of lupus nephritis is set to change quite rapidly in the next decade. In this review we highlight the likely major therapeutic advances.     

Retractile mesenteritis presenting as protein-losing gastroenteropathy.

Retractile mesenteritis is a rare, idiopathic condition characterized by nonspecific inflammation of the mesenteric adipose tissue. The majority of patients present with abdominal pain and/or a palpable mass. In the present report, a 68-year-old man with peripheral edema and mild hypoalbuminemia is presented. Protein-losing gastro-enteropathy was confirmed with an abnormal stool alpha1-antitrypsin clearance test and retractile mesenteritis was diagnosed at laparoscopy. This rare condition may respond to therapy with corticosteroids, azathioprine, cyclophosphamide, colchicine, progesterone, tamoxifen or thalidomide. Gastroenterologists should consider the diagnosis of protein-losing enteropathy in patients who present with unexplained peripheral edema or hypoalbuminemia. The test of choice to confirm this diagnosis is the stool alpha1-antitrypsin clearance test.