Polyarteritis nodosa revisited

Polyarteritis nodosa (PAN), the prototype of systemic vasculitis, is a rare condition characterized by necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules. Signs and symptoms of this disease are primarily attributable to diffuse vascular inflammation and ischemia of affected organs. Virtually any organ with the exception of the lungs may be affected, with peripheral neuropathy and symptoms from osteoarticular, renal artery, and gastrointestinal tract involvement being the most frequent clinical manifestations. A clear distinction between limited versus systemic disease and idiopathic versus hepatitis B related PAN should be done because there are differences in the implicated pathogenetic mechanisms, their treatment, and prognosis. Currently, corticosteroids plus cyclophosphamide is the standard of care for idiopathic PAN, in particular for patients with adverse prognostic factors (more severe disease), in whom this combination prolonged survival. In contrast for hepatitis B related PAN treatment consists of schemes that include plasmapheresis and antiviral agents.     

Vasculitis and the gastrointestinal tract

Vasculitis, defined as a non-infectious inflammatory disorder of blood vessels, can affect vessels of any type in any organ. The gastrointestinal (GI) tract may thus also be involved. In systemic disorders as mixed connective tissue disease (MCTD) and systemic lupus erythematodes (SLE), patients may present with symptoms of gastrointestinal disfunction such as motility disorders, caused by alterations in the connective tissue. True vasculitis however also occurs in the GI tract. Severe, occlusive damage often leads to ischemia that may result in ulceration and perforation. Non-occlusive vascular disease may lead to vascular leakage resulting in oedema and haemorrhage. Those patients often present with diarrhoea or symptoms of bleeding. GI involvement is frequent in Henoch-Sch?nlein purpura and also often noted in polyarteritis nodosa (PAN), microscopic polyangiitis, Wegener's syndrome and Churg-Strauss syndrome. Furthermore, GI vasculitis has also been described in giant cell arteritis, Takayasu's disease, Buerger's disease and leucocytoclastic vasculitides as essential mixed cryoglubulinemia, lupus vasculitis, rheumatoid disease, MCTD, drug-induced vasculitis and Beh?et's disease. The diagnosis and classification of vasculitis relies upon a combination of clinical, serological, haematological, radiological and histological findings. Establishing a precise diagnosis can be difficult but is important because treatment and prognosis can be highly variable.     

Successful treatment of gastrointestinal vasculitis due to systemic lupus erythematosus with intravenous pulse cyclophosphamide: a clinical case report and review of the literature

Gastrointestinal vasculitis in systemic lupus erythematosus (SLE) is quite rare and almost always accompanied by evidence of active disease in other organs, although occasionally it may be the presenting feature of the disease. Gastrointestinal involvement in SLE may present as lupus peritonitis, non-necrotizing pancreatitis, gastrointestinal vasculitis or surgical abdomen. Here we report a severe case of SLE which presented initially with fever of unknown origin. Severe distress, abdominal pain, the presence of occult blood in the stool and high acute-phase proteins were explained by a lupus peritonitis and intestinal vasculitis resembling inflammatory bowel disease. Whereas high-dose prednisone treatment did not prevent a severe relapse, we observed a sustained remission following i.v. cyclophosphamide pulse therapy. In the literature, only two similar cases are reported: one died despite a change in the therapy of a bowel perforation; our case was the second that improved under pulse cyclophosphamide. We suggest the use of cyclophosphamide after failure of steroids early in the course of SLE gastrointestinal vasculitis to prevent devastating complications.      

Fulminant amoebic colitis mimicking intestinal vasculitis in a patient with systemic lupus erythematosus

Colitis in patients with systemic lupus erythematosus (SLE) is quite rare. It can be caused by intestinal vasculitis, mesenteric vascular thrombosis, concomitant inflammatory bowel disease or infectious colitis. It is important to make an accurate and early diagnosis as the treatments for each condition differ and a delayed diagnosis can result in life-threatening complications. However, non-specific gastrointestinal symptoms make a timely diagnosis challenging. Amoebic colitis is a rare condition in patients with SLE. Here we present a case of fulminant amoebic colitis in a patient with SLE which was initially misdiagnosed as ischemic colitis due to intestinal vasculitis. Her colitis was complicated with multiple intestinal perforations, disseminated intravascular coagulation and acute respiratory distress syndrome; but in the end, the patient was successfully treated with metronidazole and paromomycin.     

Spontaneous retroperitoneal bleeding from renal microaneurysms and pancreatic pseudocyst in a patient with systemic lupus erythematosus

Visceral vasculitis and pancreatic pseudocyst are rare manifestations of systemic lupus erythematosus (SLE). We describe a patient with SLE who presented with spontaneous bilateral perinephric and retroperitoneal haematoma secondary to polyarteritis nodosa (PAN)-like vasculitis of the renal arteries, which subsequently evolved into systemic vasculitis with pancreatic pseudocyst formation.     

A case of systemic lupus erythematosus presenting with intestinal lymphangiectasia-associated protein-losing enteropathy accompanying hyperinflammation

Systemic lupus erythematosus (SLE) has the potential to affect virtually every organ; however, gastrointestinal system manifestations are relatively rare compared to other autoimmune diseases such as systemic sclerosis and inflammatory bowel disease. A 29-year-old female patient attended to the emergency room with abdominal distention, acute onset abdominal pain and constipation. She had watery chronic diarrhea (4-5 times/d) and weight loss (6 kg, 12%) for 4 months. While there was increased intestinal wall thickness, air-liquid levels were shown on abdomen computed tomography scan. The patient underwent abdominal surgery due to diagnosis of ileus. Ileocecal resection was performed and pathologic evaluation revealed intestinal lymphangiectasia. Autoimmune serology was performed with the following resulats: anti-nuclear antibody 1/3200 with homogenous pattern, anti-DNA antibody and anti-Sm/ribonucleoprotein antibodies were positive in addition to low complement levels (C3: 0.28 [0.9-1.8 g/L], C4: 0.06 [0.1-0.4 g/L]) indicating diagnosis of SLE. Development of intestinal involvement in SLE (lupus enteritis) is mainly grouped into 3 headings such as mesenteric vasculitis, pseudo-obstruction, and protein-losing enteropathy. Although the pathogenesis of intestinal lymphangiectasia remains unknown, it has been reported that immune complex-mediated visceral vasculitis may result in bowel wall and mucosal edema. To our knowledge this is the first case report accompanying hyperinflammatory response in addition to intestinal lymphangiectasia in SLE. On the other hand, clinicians should be alert for other reasons for hyperinflammatory syndromes rather than COVID-19, even during the pandemic.     

Systemic lupus erythematosus complicated with acute myocardial infarction and ischemic colitis

Acute myocardial infarction (AMI) is one of the most severe manifestations in patients with systemic lupus erythematosus (SLE). Ischemic colitis, mainly caused by intestinal vasculitis, is also one of the most serious, but uncommon, complications in SLE patients. "SLE vasculitis" simultaneously involving cardiac and gastrointestinal vessels has yet to be reported. This is the first report of SLE accompanying AMI, ischemic colitis and perforation of the digestive tract possibly due to SLE vasculitis, which was dramatically improved by treatment with high-dose glucocorticoid.     

Acute cholecystitis at initial presentation of polyarteritis nodosa

Polyarteritis nodosa (PAN) is a systemic vasculitis of unknown etiology. Although gastrointestinal involvement may be seen in half of the cases of PAN, vasculitis of the gallbladder at the disease onset is a rare presentation. We report a case of a 48-year-old man who was admitted due to acute cholecystitis. He had complained of myalgia, fever and weight loss for about one month prior to admission. At physical examination, mild hypertension and calf pain were noted. He underwent a cholecystectomy; histopathological evaluation disclosed necrotizing vasculitis suggestive of PAN. We emphasize that cholecystitis may be part of the initial presentation of systemic vasculitis.     

CD146在血管炎患者外周血白细胞表达的意义初探

目的探讨血管炎患者外周血白细胞CD146表达与临床活动性间的关系。方法流式细胞术检测39例活动期系统性血管炎患者[显微镜下多血管炎（MPA）13例,韦格纳肉芽肿（WG）9例,变应性肉芽肿性血管炎（CSS）2例,大动脉炎（TA）9例,白塞病（BD）4例,结节性多动脉炎（PAN）2例]及24例系统性红斑狼疮（SLE）患者外周血白细胞CD146表达,其中18例（MPA5例,WG4例,CSS2例,SLE4例,PAN2例,TA1例）患者经糖皮质激素和环磷酰胺治疗后于病情好转时再次检测。结果（1）与健康者相比,血管炎患者活动期中性粒细胞、淋巴细胞CD146表达增多,尤以中性粒细胞最多,差异均有统计学意义（P〈0．05）。（2）中性粒细胞CD146表达与淋巴细胞、单核细胞CD146表达相关（r值分别为0．66、0．853,P=0．000）,与病程、年龄、血沉、C反应蛋白、抗中性粒细胞胞浆抗体（ANCA）、PR3-ANCA、MPO-ANCA、血肌酐、伯明翰血管炎活动指数（BVAS）、系统性红斑狼疮疾病活动指数（SLEDAI）等无明显相关（r值分别为-0．108、-0．059、-0．073、-0．103、0．012、-0．5、-0．232、0．001、-0．08、0．089,P〉0．5）。（3）18例患者经治疗后好转期中性粒细胞、淋巴细胞CD146表达多数呈逐渐减少的趋势（P〈0．05）。结论CD 146在血管炎患者活动期外周血白细胞尤其是中性粒细胞中表达明显升高,随着糖皮质激素和免疫抑制剂治疗病情好转后呈下降或转阴趋势,其在血管炎发病机制中的意义有待深入研究。     

Choroidopathy of systemic lupus erythematosus

PURPOSE: To describe the ocular and systemic manifestations associated with systemic lupus erythematosus (SLE) choroidopathy. METHODS: Three new cases of choroidopathy in patients with active SLE were described. Twenty-five published cases of lupus choroidopathy were summarized. RESULTS: There have been 28 cases of lupus choroidopathy (47 involved eyes) that have been reported in the English literature since 1968, including the three current cases. Only two of the patients were male. The choroidopathy was bilateral in 19 patients (68%). All 28 patients (100%) had active systemic vascular disease at the onset of their choroidopathy; 18 (64%) had nephropathy and 10 (36%) had central nervous system (CNS) lupus vasculitis. All but one of the patients had a known diagnosis of SLE at the onset of choroidopathy. 30 of the 47 involved eyes had presenting visual acuity of 20/40 or better; 14 eyes showed improvement in visual acuity with therapy. 23 patients (82%) had resolution of their choroidopathy when their systemic disease was brought under control. Despite treatment, 4 of the 28 patients (14%) died from complications of SLE. CONCLUSIONS: Although less known than retinopathy, lupus choroidopathy may be more common than generally appreciated. It usually serves as a sensitive indicator of lupus activity. The presence of SLE choroidopathy is generally indicative of coexistent (although sometimes occult) nephropathy, CNS vasculitis, and other SLE visceral lesions. Immunomodulation of the systemic disease can lead to improvement and resolution of the systemic vasculitis as well as the choroidopathy.     

Vasculitis-induced colonic strictures

PURPOSE: The clinical presentations of gastrointestinal involvement from systemic vasculitis are diverse. Colonic involvement from systemic vasculitis is unusual. We report the first case of a symptomatic colonic stricture associated with rheumatoid vasculitis and another associated with systemic lupus erythematosus. METHODS: The clinical, radiologic, and histologic features of two cases of symptomatic colonic strictures secondary to colonic involvement with vasculitis are described. The literature covering gastrointestinal involvement from vasculitis in these conditions is reviewed. RESULTS: Surgical resection of the colonic strictures was required in both patients and had a satisfactory outcome. CONCLUSIONS: These cases provide further evidence of the protean clinical presentations of intestinal involvement in systemic vasculitis. Although immunosuppression has been shown to be of value in the treatment of vasculitis affecting the gastrointestinal tract, surgical resection is required for established strictures.     

A case of systemic lupus erythematosus presenting with rectal ulcers as the initial clinical manifestation of disease

Gastrointestinal involvement is often seen in patients with systemic lupus erythematosus (SLE). All parts of the gastrointestinal tract may be affected. However, rectal involvement at onset is rare. We describe here a case of SLE in which rectal ulcers due to vasculitis occurred as the initial manifestation of the disease without involvement of any other organ. The ulcers worsened, along with the appearance of lupus nephritis 5 years later When steroid therapy was initiated, there was rapid clinical and radiographic improvement. Our case suggests that rectal ulcer is a rare but important complication of SLE and can represent the initial and sole clinical manifestation of the disease.     

Systemic lupus erythematosus vasculitis: A current therapeutic overview

Opinion statement The development of systemic lupus erythematosus (SLE) vasculitis is of prognostic value. The earlier the vasculitis is treated, the better the prognosis for SLE. Cutaneous vasculitis is common in SLE, whereas visceral vasculitis is rare. Skin SLE vasculitis is successfully treated with antimalarials, but its discontinuation may result in an SLE flare even among patients in remission. When visceral SLE vasculitis is encountered, or when a disease state is perceived to be life-threatening, a more aggressive therapy is warranted. A combination of medications, plasmapheresis, and intravenous immunoglobulin treatment, along with high-dose steroids and cytotoxic drugs, are typically employed in the treatment of severe SLE vasculitis. Finally, patients with SLE vasculitis may benefit from a number of autoimmune disease therapies currently under investigation, such as switching cytokine responses from Th1 to Th2, and the manipulation of toll-like receptors, chemokines, and FcR receptors. Specific B-cell therapies (eg, anti-Blys, B-cell depletion) may also emerge as potential treatments for SLE vasculitis.     

系统性红斑狼疮合并肠假性梗阻12例临床分析

目的 分析系统性红斑狼疮(SLE)合并肠假性梗阻病人的临床表现及实验室检查，提高对SLE合并肠梗阻的认识。方法对12例SLE合并肠梗阻的病人进行回顾性分析，收集整理其临床资料及有关实验室检查，分析其发病情况、临床累及脏器、实验室检查、病程、治疗及预后等特点。结果发病年龄2l～56岁，平均36岁，平均病程31个月。死亡5例。以肠梗阻为首发症状者2例。有肾脏累及者9例，血液系统累及者6例，中枢神经系统累及者2例，循环系统累及者8例。其他：胃肠出血4例，腹水4例。2例以肠梗阻为首发症状者入院前分别误诊为溃疡性结肠炎和肠炎。2例曾行剖腹探查并部分小肠切除术，病理报告均提示肠道血管炎。结论SLE合并肠梗阻患者病情较重，常伴有其他脏器累及，病死率较高。以肠梗阻为首发症状的SLE患者易被误诊。有肠梗阻症状并有其他脏器累及者应排除有无SLE可能。肠道血管炎是引起肠梗阻的主要原因。     

Polyarteritis nodosa and microscopic polyangiitis: Etiologic and diagnostic considerations

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized vessels with clinical manifestations resulting from ischemia and infarction of affected tissues and organs. Although the cause of most cases of PAN and the related disorder microscopic polyangiitis (MPA) remains largely unknown, there has been significant progress in understanding the pathogenesis of vascular inflammation. The diagnostic approach to PAN and MPA should be individualized and based on specific organ involvement. Because no test or clinical finding reliably indicates the presence or absence of PAN or MPA, diagnosis requires integration of clinical findings, angiography, and biopsy data.     

Successful treatment of pediatric systemic polyarteritis nodosa with cholestatic hepatitis

Polyarteritis nodosa (PAN) is a rare vasculitis in children. We report a case of 5-year-old boy with systemic PAN and cholestatic hepatitis. He had fever, abdominal pain, and gastrointestinal bleeding. Angiography revealed multiple microaneurysms in the renal, hepatic, and superior mesenteric arteries. Clinical manifestations improved slowly after immunosuppressive therapy, but liver enzyme and bilirubin levels elevated gradually. Liver biopsy findings revealed marked centrizonal canalicular cholestasis, bile duct damage, and intact hepatocyte, but there was no evidence of viral hepatitis or vasculitis. Levels of liver enzymes and bilirubin improved after two cycles of cyclophosphamide therapy. We thought that the possible etiology of elevated liver enzyme and bilirubin levels might be a manifestation of PAN.     

Acute gastrointestinal distress syndrome in patients with systemic lupus erythematosus

Cases of mesenteric vasculitis in systemic lupus erythematosus (SLE) are well described, however, individual patient with recurrent mesenteric vasculopathy producing repeated episodes with each exacerbation similar in character and quality has not been reported previously in the literature. We describe two SLE patients whose condition was complicated by repeated stereotypic, CT confirmed, episodes of lupus enteritis characterized by dramatic intestinal wall edema. Moreover, each flare was accompanied by significant hypocomplementemia and was rapidly reversible suggesting an acute gastrointestinal distress syndrome (AGDS) as a result of leukoaggregation and a gut capillary leak syndrome.     

Calciphylaxis in a patient with end-stage renal disease secondary to systemic lupus erythematosus associated with acral gangrene and mesenteric ischemia

A patient with end stage renal disease secondary to systemic lupus erythematosus (SLE) ultimately required amputation of the four extremities and developed mesenteric ischemia. The patient presented with widespread medial calcification involving various small to medium sized arteries, although no noticeable secondary hyperparathyroidism was observed. We speculated that SLE associated with systemic vasculitis and uremic milieu over a number of years may represent the perfect preexisting condition for calcific arteriolopathy to occur following which several factors including chronic administration of corticosteroids, photosensitivity in lupus, and significant weight loss may have contributed to acral gangrene and mesenteric ischemia.     

Coronary artery disease in systemic lupus erythematosus: A review of the literature

CONTEXT: Coronary artery occlusive disease is a common though underappreciated complication of systemic lupus erythematosus (SLE), typically a disease of young women. A case of a premenopausal patient with SLE and an acute myocardial infarction is presented, and the etiology and management of coronary artery disease in SLE reviewed. OBJECTIVES: To review the incidence, risk factors, pathology and treatment of coronary artery disease in systemic lupus erythematosus. DATA SOURCES: MEDLINE search of articles in English-language journals from 1980 to 2000. The index words "systemic lupus erythematosus" and the following co-indexing terms were used: "coronary artery disease," "atherosclerosis," "vasculitis," "anticardiolipin antibodies," "antiphospholipid syndrome." SELECTION SYNTHESIS AND ABSTRACTION: Papers identified were reviewed and abstracted by the authors with a presentation of a summary. RESULTS: The prevalence of coronary artery disease among women with SLE between the ages of 35 and 44 years is at least 50-fold greater than among age-matched control subjects. Of these, coronary atherosclerosis accounts for the vast majority of cases; vasculitis of the coronary arteries and other causes generally believed to be more typical of SLE are comparatively rare. CONCLUSIONS: The evidence suggests that SLE is a significant risk factor for coronary atherosclerosis independent of the classic risk factors of hypertension, tobacco use, and hyperlipidemia.     

Systemic manifestations of Parvovirus B19 infections

PURPOSE: Parvovirus B19 (B19) causes many clinical disorders, of which the most common are erythema infectiosum, aplastic crisis complicating chronic hemolytic anemia, and hydrops fetalis. In young adults, the skin eruption caused by B19 is accompanied by polyarthritis and polyarthralgia in 60% of the cases. Rheumatoid factors and other antibodies including antinuclear antibodies, anti-ADN, and antiphospholipids can be produced in the wake of B19 infection. CURRENT KNOWLEDGE AND KEY POINTS: These features may simulate systemic diseases as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) (lupus-like eruption over the cheeks, cytopenia, etc.) or vasculitis (purpura, renal involvement). In addition, there have been a few reports of SLE, vasculitis and other connective tissue diseases developing shortly after a B19 infection associated with virus clearance suggesting that B19 can act as a trigger of systemic disease. However, studies in large series indicate that in fact B19 is probably an extremely rare cause of RA, SLE or vasculitis. FUTURE PROSPECTS AND PROJECTS: In fundamental studies B19 interacts with inflammatory cells by regulation of cytokines. More recently, two studies suggest that viral infection due to B19 may affect the course of SLE, leading to specific biological subsets. These preliminary findings require confirmation to elucidate the significance of the presence of B19 in systemic disease.