Ischemic colitis associated with intestinal vasculitis: Histological proof in systemic lupus erythematosus

I schemic colitis is an uncommon complication in patients with systemic lupus erythematosus (SLE). In previously reported cases of colitis caused by SLE, intestinal vasculitis is implicated as the causative process, but is rarely confirmed histologically. We described a case of a 32-year-old man with increased activity of SLE, who presented with hematochezia and abdominal pain due to ischemic colitis with small vessel vasculitis which was proven by sigmoidoscopic biopsy. The clinical course of the patient was improved after steroid and conservative management.

Systemic Vasculitis:An Important and Underestimated Cause of Malignant Hypertension

Objectives:Malignant hypertension(MHT)is defi ned as severe hypertension accompanied by ischemic failure of one or more organs.The aims of this study were to evaluate the current clinical and etiologic profi les of MHT.Methods:As a retrospective study,we selected all patients admitted to our center from January 2013 to December 2016.Seventy patients with MHT were included.Results:The average age of the patients was 40 years,and more than half of the patients were male(78.57%).There were 24 patients with essential hypertension,accounting for 34.29%of the patients,and 46 with secondary hypertension,accounting for 65.71%of the patients.For secondary MHT,systemic vasculitis(25.57%)was the most common cause,followed by severe obstructive sleep apnea syndrome(15.71%),primary renal parenchymal hypertension(11.43%),primary aldosteronism(7.14%),and Cushing syndrome(1.43%)and nutcracker phenomenon(1.43%).Twenty patients with systemic vasculitis were characterized by severe hypertension accompanied by damage to two or more target organs of differing severity.The levels of white blood cells,hypersensitive C-reactive protein,serum creatinine,and 24-hour urinary protein were above their normal range.Conclusion:Systemic vasculitis may be one of the main causes of MHT,and has been underestimated in the past.In future clinical work,clinicians need to pay more attention to patients with systemic vasculitis.

Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome in a 77-year-old man: A case report

BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.

Systemic Lupus Erythematosus and Antiphospholipid Syndrome Related Retinal Vasculitis Mimicking Ocular Cysticercosis: a Case Report

MAKING accurate and timely diagnosis is often challenging when patients with a systemic disease first present with ocular manifestations.The possibility that vasculitis associated with systemic lupus erythematosus(SLE)and antiphospholipid syndrome(APS)can be misdiagnosed as cysticercosis has not been discussed in the literatures.

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis:Part 1

This is a review article describing some new and interesting aspects in the diagnosis and treatment in systemic vasculitis and demonstrating several cases that we have encountered.Particular emphasis will be put on Takayasu arteritis(also known as aortitis syndrome)most commonly observed in Japan and other oriental countries.CT and MRI better demonstrate the vessel wall abnormality of Takayasu arteritis than angiography.Importance of plain chest radiograph should also be kept in mind;it could provide a clue in the diagnosis of Takayasu arteritis in its early stage.

Classification,diagnosis and treatment of ANCA-associated vasculitis

Diagnosis of anti-neutrophil cytoplasmic antibodies(ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCAassociated vasculitis. Cyclophosphamide/azathioprine in combination with high dose steroids are used to induce and maintain remission of systemic vasculitis. The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis: Part 2

In part 1 of this article,clinical features and imaging findings of Takayasu arteritis(also known as aortitis syndrome)were described in detail.In part 2,treatment of Takayasu arteritis will be first described and discussed.This will be followed by description of clinical features and imaging findings of other systemic vasculitis.Comments on interventional radiology for systemic vasculitis will also be made.

Mononeuropathy multiplex associated with systemic vasculitis:A case report

BACKGROUND Vasculitis,a systemic disorder with inflammation of blood vessel walls,can develop broad spectrum of signs and symptoms according to involvement of various organs,and therefore,early diagnosis of vasculitis is challenging.We herein describe a patient who developed a special case of systemic vasculitis with mononeuropathy multiplex,rectal perforation and antiphospholipid syndrome(APS)presented with pulmonary embolism.CASE SUMMARY A 61-year-old woman visited hospital with complaints of myalgia and occasional fever.She was initially diagnosed as proctitis and treated with antibiotics,however,there was no improvement.In addition,she also complained right foot drop with hypesthesia,and left 2^(nd) and 3^(rd) finger tingling sensation.She underwent nerve conduction study for evaluation,and it revealed sensorimotor polyneuropathy in the left arm and bilateral legs.Subsequent sural nerve biopsy strongly suggested vasculitic neuropathy.Based on nerve biopsy and clinical manifestation,she was diagnosed with vasculitis and treated with immunosuppressive therapy.During treatment,sudden rectal perforation and pulmonary thromboembolism occurred,and further laboratory study suggested probable concomitant APS.Emergency Hartmann operation was performed for rectal perforation,and anti-coagulation therapy was started for APS.After few cycles of immunosuppressive therapy,tingling sensation and weakness in her hand and foot had been partially recovered and vasculitis was considered to be stationary.CONCLUSION Vasculitis can be presented with a variety of signs and symptoms,therefore,clinicians should always consider the possibility of diagnosis.

ANCA-Associated Vasculitis: Value of Apheresis in Initial Treatment

Introduction: Vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA) can be grouped with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MAP), and eosinophilic granulomatosis with polyangiitis (EGPA). Diagnosis of these rare pathologies is based on clinical presentation, the positivity of ANCA, and, if possible, histological proof of vasculitis. Our study describes a series of six cases of ANCA-associated vasculitis where due to the severity of symptoms apheresis sessions were started from the beginning of the therapy. Patients and methods: We conducted a retrospective, single-center observational, monocentric study on all patients treated by apheresis for ANCA vasculitis in the period January 01, 2016 to December 01, 2019. Results: We identified six cases of ANCA vasculitis treated by apheresis over a 3-year period. The mean age was 61 ± 19 years;M/F gender ratio was 1:1. Initial renal damage in all patients was rapidly progressive glomerulonephritis. Inflammatory syndrome occurred in all patients with average CRP of 82 mg/L. All patients had positive ANCA at diagnosis. Four patients required renal replacement therapy at the time of diagnosis. The induction regimen consisted of rituximab associated with IV boluses of methylprednisolone. The apheresis techniques used were the same for all patients, i.e. plasmapheresis. Outcomes were favorable for five patients;only one patient became dependent on hemodialysis. No mortality occurred. Conclusion: This study analyzed practices for the management of patients with ANCA vasculitis. No patient was treated with cyclophosphamide as a first approach but rituximab instead. Plasmapheresis was given because of symptoms severity at initial diagnosis.

Acute flare of systemic lupus erythematosus with extensive gastrointestinal involvement: A case report and review of literature

BACKGROUND Lupus mesenteric vasculitis(LMV)is a serious condition that may occur as an acute manifestation of gastrointestinal(GI)involvement and is not easily diagnosed by physicians.Delayed diagnosis and treatment of LMV may lead to rapid disease progression and can be life threatening.CASE SUMMARY A previously healthy 27-year-old woman presented with abdominal pain following a history of fatigue and consumption of cold water.Laboratory invest-igations,physical examinations,and enhanced abdominal computed tomography(CT)suggested systemic lupus erythematosus complicated by LMV.She received treatments,such as GI decompression,somatostatin,glucocorticoids,and immu-nosuppressants,and was evaluated using color ultrasonography.Twenty days later,the patient reported no stomach discomfort and was able to consume semi-liquid food.Laboratory investigations showed that inflammatory factors decreased to normal levels and complement levels increased slightly.One year after discharged,she recovered with methylprednisolone being tapered to 4 mg per day,mycophenolate mofetil to 0.75 g bid,and hydroxychloroquine to 0.2 g bid;however,only C3 complement level was slightly below the normal level.CONCLUSION Early diagnosis of LMV is essential for successful treatment;this depends on a combination of clinical manifestations,laboratory investigations,and imaging findings.Enhanced CT is preferred,but ultrasonography can be used for prompt screening and follow-up.

Panorama, Reasons for Seeking Care and Evolution of Systemic Autoimmune Diseases in Benin Hospitals in 2021

Introduction: Systemic autoimmune diseases have been poorly studied in sub-Saharan Africa and their frequency is not well known. This study provided an overview of the main reasons for the use of care and their evolution in the main hospitals in Benin. Methods: This was a multi-centric descriptive cross-sectional study conducted in the internal medicine, rheumatology, dermatology and nephrology departments of nine (09) public and private hospital centers in Benin over a 57-month period, from January 1st, 2017 to September 30th, 2021. It involved patients followed for a systemic autoimmune disease. The data was collected with a digital survey sheet and then processed and analyzed with the R software (version 3.4). Results: Two hundred and three (203) patients were recorded, which represents a hospital frequency of 0.13%. The mean age was 44 years and the sex ratio (M/F) was 0.29. Connective tissue disease accounted for 95.07% of systemic autoimmune diseases which were dominated by rheumatoid arthritis (40.9%) and systemic lupus (37.4%). Ten cases of vasculitis have been reported and dominated by Behçet’s disease (40%). The main reasons for seeking care were asthenia, weight loss and fever. Arthralgia and skin lesions are the main guiding signs. Six deaths (3.1%) were recorded among connective tissue disease and 1 death (10%) among vasculitis. Conclusion: In spite of being rare, systemic autoimmune diseases are a reality in Benin. A general population study would provide a better understanding of clinical characteristics and identify prognostic factors.

Two uncommon manifestations of leptospirosis:Sweet's syndrome and central nervous system vasculitis

To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide.The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries,at most clinical suspicion it is essential in the diagnosis of this disease.In this report,we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.

Gastrointestinal involvement in systemic lupus erythematosus:Insight into pathogenesis, diagnosis and treatment

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are com-mon in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lu-pus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by pro-tein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroen-teropathies. Most of these complications have good therapeutic responses to corticosteroids and immu-nosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and proki-netic medications are helpful in facilitating functional recovery and improving the outcome.

Endoscopic and radiographic features of gastrointestinal involvement in vasculitis

Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.

Systemic Diseases in Dakar (Senegal): Spectrum, Epidemiological Aspect and Diagnostic Time-Limit

Introduction: Systemic diseases have been the subject of few studies in the African literature and have probably been under-estimated. The objective of our study was to specify their spectrum, their epidemiological aspects and diagnostic delay in Internal Medicine Departments of Dakar (Senegal). Material and Method: It was a multicentric retrospective and descriptive study regarding all systemic diseases during 119 months from 1st January 2005 to 30 November 2014 in 5 hospital centers down Dakar. Systemic diseases were retained according to their international consensus criteria. Results: During the studying period, 726 patients were included with 632 women and 94 men (sex ratio of 0.14). The average age was 43.76 years. Inflammatory rheumatoid family history was noted in 10.06% of cases. Rheumatoid arthritis (RA) was the predominant affection, recorded on 564 patients, isolated or associated with other systemic diseases. It was followed in a decreasing order, in the systemic auto-immune diseases sub-groupe, by systemic lupus (56 cases), Sj?gren’s syndrome (32 cases), Systemic Sclerosis (26 cases), Idiopathic inflammatory myopathies (21 cases), Undifferentiated connective tissue diseases (20 cases), Anti Phospholipid’s syndrome (6 cases) and Mixed connective tissue disease (6 cases). A diagnosis of systemic vasculitis was recorded in 19 patients. The other systemic affections were represented by systemic sarcoidosis (8 cases), Adult-onset Still’s disease (03 cases), amyloidosis (02 cases) and 02 cases of systemic syndrome associated to immunodeficiency. The mean diagnostic delay duration before the diagnostic was 3.46 years. Conclusion: Systemic diseases in internal medicine are characterized by their diversity, the clear predominance of RA, and significant diagnostic delay.

Neurological Involvement in Systemic Lupus Erythematosus (SLE): Our Recent Experience

Introduction: The central, psychiatric and peripheral neurological manifestations of lupus are among the most severe visceral disorders and are grouped under the general term of “neuro-psychiatric systemic lupus erythematosus” (NPSLE). We conducted a cross-sectional observational study within our Department of Internal Medicine aimed at describing the clinical and evolutionary aspects of central neurological disorders of SLE, excluding lupus myelopathy. Patients and Methods: This was a retrospective and observational cross-sectional study carried out from 1 January 2015 to 31 October 2017, in the Department of Internal Medicine of Aristide le Dantec University Hospital in Dakar (Senegal). All patients hospitalized during this period who met the 1997 ACR classification criteria of SLE and who presented with a central neuropsychiatric syndrome attributable to SLE (as defined by ACR 1999) were included. Patients with isolated headache, acute myelitis or secondary neurological involvement attributable to a toxic, metabolic, infectious or tumour-related cause were excluded from our study. Results: During the study period, 10 patients with neuropsychiatric lupus involvement were treated at our institution, including 9 women and 1 man;the median age was 29 years (20 - 55 years). Neurological involvement occurred during the course of lupus evolution in 9/10 cases. The median time to SLE evolution was 18 months (0 - 60 months). Neuropsychiatric syndromes as defined by the 1999 ACR were commonly associated and more than half of our patients had multiple neuropsychiatric syndromes. There were 5 cases of confusion syndrome and coma, 4 cases of seizure, 3 cases of psychosis, 2 cases of acute cerebrovascular disease and 1 case of aseptic meningitis. Among the extra-neurological manifestations of SLE, haematological and dermatological involvements were common. Renal involvement affected half of the patients. The other manifestations were: polyarthritis in 3 patients, serositis in 2 patients, 5 cases of fever, 4 cases of deterioration of the general state, and one isolated case of ophthalmological involvement. Therapeutically, 8 patients received a bolus of methylprednisolone and 3 patients received a bolus of cyclophosphamide. Oral corticosteroids and hydroxychloroquine were administered to all patients, and azathioprine was administered in 2 patients. The evolution was favorable in 4 patients, other 2 patients maintained neurological sequelae and 2 patients were transferred to intensive care. Death was recorded in 4 patients. Conclusion: Neuropsychiatric manifestations of lupus are rare and sometimes severe, potentially life-threatening. In our patients, we have identified some of the most severe neurological syndromes according to the ACR nomenclature. The neurological involvement is exceptionally revealing, as these syndromes are often associated and integrated into a systemic context of lupus. The evolution is rapidly unfavorable and requires early diagnosis and optimal management.

感染性心内膜炎模拟系统性血管炎1例并文献复习

目的提高对感染性心内膜炎并发症的临床认识和鉴别诊断能力。方法回顾分析2022年8月15日本院收治的1例感染性心内膜炎患者的临床、实验室、影像学资料及治疗转归,结合国内外相关文献进行复习。结果感染性心内膜炎可表现出类似于原发性系统性血管炎的临床表现及实验室指标特征,从而增加疾病诊断的挑战性。动态复查心脏彩超有助于感染性心内膜炎早期诊断,糖皮质激素及免疫抑制剂对感染性心内膜炎临床症状改善作用有限,对改善实验室指标有一定作用,抗生素治疗及心脏瓣膜手术是关键性治疗手段。结论以多系统受累为临床表现的疑似系统性血管炎患者,需要警惕感染性心内膜炎的可能,糖皮质激素及免疫抑制剂在感染性心内膜炎模拟系统性血管炎治疗中患者有一定获益,早期诊断、规范治疗是改善感染性心内膜炎预后的关键因素。

右侧肢体偏瘫伴失语,左腰部疼痛——迷惑性极强的血管型Ehlers-Danlos综合征

作为一种罕见的遗传性结缔组织疾病,Ehlers-Danlos综合征(EDS)在人群中发病率为(0.1~1)/万。而作为EDS中预后最差的一类分型,血管型EDS(vEDS)的发病率在全部EDS分型中极低。本文报道一例vEDS患者,表现为一过性右侧肢体偏瘫伴失语,左腰部疼痛。通过基因检测最终诊断为vEDS,通过多学科会诊共同决策,为该患者制订后续的治疗方案。EDS分型复杂,表现多样,极易误诊,在此报告诊疗经验。

合并脊髓受累的瘤样原发性中枢神经系统血管炎一例

原发性中枢神经系统血管炎(PACNS)是一种发生在中枢神经系统的主要侵犯脑、脊髓及软脑膜的中小血管的血管炎性疾病。瘤样PNCAS是PACNS的少见亚型,常被误诊为颅内恶性肿瘤,脊髓受累在PACNS中更为罕见。PACNS因临床症状及影像学表现缺乏特异性,早期诊断困难。本文报道1例经病理确诊合并脊髓受累的瘤样PACNS,以提高对该病的认识。

儿童狼疮性肾炎合并神经精神狼疮的临床特征和危险因素

目的探究狼疮性肾炎(lupus nephritis,LN)合并神经精神狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE)患儿的临床特征,并分析LN患儿合并NPSLE的危险因素。方法纳入2017年5月至2024年1月在西安市儿童医院肾脏内科就诊的LN患儿119例,根据是否合并NPSLE分为NPSLE组(n=26)和非NPSLE组(n=93)。比较两组临床资料差异,分析LN患儿合并NPSLE的临床特征和危险因素。结果①本研究中,LN患儿合并NPSLE的发病率为21.8%,男女比例为1∶5.5,平均年龄为10岁6月;最常见的神经系统受累表现为精神异常,一般表现为盘形红斑,影像学表现为头颅MRI异常。②两组抗核抗体阳性、抗ds-DNA抗体阳性、抗SM抗体阳性、三系减少、仅贫血、血沉高(>15 mm/h)、低蛋白血症、Coombs试验阳性、血尿、大量蛋白尿指标等差异均有统计学意义(P<0.05)。③二元logistic回归分析显示,血尿、大量蛋白尿、抗SM抗体阳性是LN患儿合并NPSLE的独立危险因素。结论LN合并NPSLE的影响因素较多,其中血尿、大量蛋白尿、抗SM抗体阳性的LN患儿易并发神经精神改变。