Enterocolic Lymphocytic Phlebitis Research Articles

FP4.12 - Enterocolic lymphocytic phlebitis masquerading as a malignant stricture in the cecum

Abstract Introduction Enterocolic lymphocytic phlebitis (ELP) is an idiopathic form of venous inflammation which is limited to the gastrointestinal tract. It is characterised by inflammation of the veins with no involvement of the arteries. Usually, It presents with gastrointestinal symptoms such as abdominal pain, and imaging may suggest malignancy. Case history A 56-year-old woman presented with fresh blood in her stools, abdominal pain and diarrhoea. All blood tests were normal; however, she had a raised CRP of 105 mg/L (normal < 5 mg/L). CT abdomen and pelvis showed thickening in the transverse colon and intussusception of the terminal ileum into the caecum. She was treated conservatively and discharged when symptoms resolved with an outpatient colonoscopy. The colonoscopy was incomplete due to an unresolvable sigmoid loop; CT colonography showed caecal wall thickening, which was suggestive of caecal malignancy. Following the MDT discussion, the patient was booked for a right hemi-colectomy. The right hemicolectomy specimen histology showed no malignancy, and lymphocytic phlebitis of the submucosal, subserosa and mesenteric veins, but the arteries were normal; hence, a diagnosis of enterocolic lymphocytic phlebitis was made. Conclusion ELP is a rare idiopathic form of venous inflammation localised to the gastrointestinal tract, which can occasionally present similar to bowel malignancy. The diagnosis is usually confirmed with a histopathology assessment. We present this case in light of its rarity to add to the literature on this entity.

Intestinal vascular diseases misdiagnosed as Crohn ​disease: analysis of 13 cases

Behçet ​disease (BD), enterocolic lymphocytic phlebitis (ELP), idiopathic myointimal hyperplasia of mesenteric veins (IMHMV), and mesenteric arteriovenous dysplasia/vasculopathy (MAVD/V) are rare vascular diseases (VDs) that cause bowel ischaemia, often presenting with a histological pattern resembling chronic enteritis, leading to diagnostic confusion with Crohn ​disease (CD). In this retrospective study, we compared the clinical and pathological characteristics of these VDs with those of CD. The study cohort comprised 13 patients misdiagnosed with CD but later identified as having VDs, including five, three, two, and three patients with BD, ELP, IMHMV, and MAVD/V, respectively. Moreover, 15 patients diagnosed with CD served as the control group. Data on disease history, patient demographics, symptoms and signs, endoscopic findings, clinical diagnosis, and follow-up status were collected, and the histological features of VDs were compared with those of CD. Despite substantial overlap in clinical and pathological characteristics between VDs and CD, several histological features were helpful in differentiating the two conditions. Intestinal stenosis, multisegmental disease, transmural inflammation, inflammatory polyps, submucosal lymphangiectasia, intramural abscesses, and epithelioid granulomas were significantly more frequent in patients with CD (p<0.001). In contrast, toxic ischaemic changes and vascular lesions were significantly more frequent in patients with VDs (p<0.001). The location of diseased vessels varied widely in patients with VDs, whereas vascular changes were limited to areas with more severe inflammation in those with CD. Furthermore, inflammatory cells within diseased vessels exhibited a polarised distribution in cases of CD, manifesting in two distinct patterns: pattern 1, where the inflammatory cells are densely populated in the outer layer of the vessel wall and sparsely populated in the inner layer; pattern 2, where the inflammatory cells are densely populated on the side with the ulcer and sparsely populated on the opposite side. Vascular occlusion, which was observed in all patients with VDs, was rare in those with CD. In summary, VDs and CD can be distinguished through careful histological analysis, particularly focusing on characteristics of vascular changes, in combination with medical history.

Lymphocytic Colitis With Enterocolic Lymphocytic Phlebitis

ABSTRACT Enterocolic lymphocytic phlebitis is a rare lymphocytic vasculitis afflicting the gastrointestinal veins without involving the arterial system. Lymphocytic colitis is a more common pathology described as lymphocytic inflammation of the colonic epithelium. Concurrence of both these pathologies is extremely rare. We describe a 53-year-old man presenting with chronic watery diarrhea, abdominal pain, and weight loss. Colonoscopic examination revealed normal-appearing mucosa, but biopsy findings revealed lymphocytic colitis with coexisting enterocolic lymphocytic phlebitis. The patient was started on oral budesonide and responded to the treatment with symptomatic and histopathological resolution.

Enterocolic lymphocytic phlebitis presenting as a caecal mass

Enterocolic lymphocytic phlebitis presenting as a caecal mass

Enterocolic lymphocytic phlebitis with marked myointimal hyperplasia and perivenous concentric fibrosis

Enterocolic lymphocytic phlebitis (ELP) is a rare enteropathy characterized by lymphocytic phlebitis of the mesenteric veins without arteritis. Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare disease similar to ELP, characterized by myointimal hyperplasia that constricts the lumen of veins, causing mucosal injury. A 62-year-old man with chief complaint of abdominal pain was treated by partial resection of the ileum after 3 months of conservative therapy. The pathologic diagnosis was ELP with prominent myointimal hyperplasia. Histologically, the lesion consisted of lymphocytic infiltration into the vein accompanied by prominent myointimal hyperplasia and perivenous concentric fibrosis, which are characteristics shared by ELP and IMHMV. The observations in this case suggest that some of ELP and IMHMV may belong to the same disease spectrum. Furthermore, perivascular concentric fibrosis was a remarkable observation that may contribute to differential diagnosis between ELP and "true" IMHMV.

Gastrointestinal Tract Vasculopathy: Clinicopathology and Description of a Possible "New Entity" With Protean Features.

Noninfectious gastrointestinal (GI) vasculopathic disorders are rare and are often overlooked in histopathologic examination or when forming differential diagnoses due to their rarity. However, involvement of the GI tract may lead to serious complications, including ischemia and perforation. Since awareness of the types of vasculopathy that may involve the GI tract is central to arriving at a correct diagnosis, we reviewed our institutional experience with GI tract vasculopathy in order to enhance diagnostic accuracy of these rare lesions. We report the clinical and histologic features of 16 cases (excluding 16 cases of immunoglobulin A vasculitis) diagnosed over a 20-year period. Of the 16 patients, 14 presented with symptoms related to the GI vasculopathy (including 2 presenting with a mass on endoscopic examination). The remaining 2 patients presented with incarcerated hernia and invasive adenocarcinoma. The vasculopathy was not associated with systemic disease and appeared limited to the GI tract in 8 patients. Eight had associated systemic disease, but only 6 had a prior diagnosis. The underlying diagnoses in these 6 patients included systemic lupus erythematosus (1), dermatomyositis (2), rheumatoid arthritis (1), eosinophilic granulomatosis with polyangiitis (1), and Crohn disease (1). One patient with granulomatous polyangiitis and 1 patient with systemic lupus erythematosus initially presented with GI symptoms. The 8 cases of isolated GI tract vasculopathy consisted of enterocolic lymphocytic phlebitis (4), idiopathic myointimal hyperplasia of the sigmoid colon (1), idiopathic myointimal hyperplasia of the ileum (1), granulomatous vasculitis (1), and polyarteritis nodosa-like arteritis (1). Isolated GI tract vasculopathy is rare, but appears to be almost as common as that associated with systemic disease. The chief primary vasculopathies are enterocolic lymphocytic colitis and idiopathic myointimal hyperplasia. Although the latter occurs predominantly in the left colon, rare examples occur in the small bowel and likely represent a complex, more protean disorder.

A Case of Enterocolic Lymphocytic Phlebitis

A Case of Enterocolic Lymphocytic Phlebitis

Atypical Presentation of a Rare Disease: Idiopathic Enterocolic Lymphocytic Phlebitis Mimicking Ulcerative Colitis

Enterocolic lymphocytic phlebitis (ELP) is a rare cause of ischemic bowel injury. It is characterized by phlebitis of the bowel wall and mesenteric veins, without arterial involvement. The etiology and pathogenesis of this condition remains poorly understood. A 33-year-old Caucasian male, presented with intermittent abdominal pain, and blood in stools for six months. He was reportedly diagnosed with ulcerative colitis and was started on mesalamine. He denied sick contacts, recent travel, tobacco and alcohol use. His physical examination revealed left lower quadrant abdominal tenderness, without guarding or rigidity. Bright red blood and tenderness to palpation were noted on rectal examination. His initial laboratory studies were unremarkable, except for hemoglobin level of 12.6 g/dl, and an elevated C-reactive protein level of 3.3 mg/dl. CT scan of the abdomen showed normal bowel wall thickness. A comprehensive laboratory work-up was negative for Anti-Nuclear Antibody (Ab), Anti-myeloperoxidase Ab, Anti-proteinase 3 Ab, Anti-mitochondrial Ab, Anti-smooth muscle Ab, Saccharomyces cerevisiae IgA/IgG Ab, celiac serology, anti- CCP Ab and rheumatoid factor. Complement and immunoglobin levels were within normal range. Stool infectious panel and stool calprotectin levels were negative. Colonoscopy revealed, moderate to severe proctosigmoiditis from the anorectal junction. Histopathologic examination showed regenerative mucosal changes without evidence of cryptitis, crypt abscesses, or granulomas. He underwent laparoscopic low anterior resection with diverting ileostomy, after failing treatment with steroids, azathioprine and infliximab for three months. Surgical pathology showed discrete ulcers that extended into submucosa, and lymphocytic cuffing of small submucosal and mesenteric veins suggestive of ELP. Flexible sigmoidoscopy during one month follow up showed, diffuse areas of severely congested, eroded, friable, dusky, and necrotic appearing mucosa. He underwent total proctocolectomy with end ileostomy for recurrence of ELP involving the entire colon. He has been asymptomatic for one year post-proctocolectomy. Discussion: ELP commonly presents as acute abdomen with hematochezia. Histological features can range from granulomatous phlebitis, necrotizing phlebitis, to myointimal and endothelial hyperplasia. Endoscopic appearance may lead to misdiagnosis, as deep submucosal biopsies are needed to diagnose ELP. Surgical resection of the affected bowel is curative.

A Case of Enterocolic Lymphocytic Phlebitis Mimicking Surgical Abdomen

A Case of Enterocolic Lymphocytic Phlebitis Mimicking Surgical Abdomen

Enterocolic lymphocytic phlebitis of the cecal pole and appendix vermiformis with increase of IgG4-positive plasma cells

Here we describe the clinicopathological course of a 20-year-old female patient with enterocolic lymphocytic phlebitis (ELP) of the appendix vermiformis and cecal pole with increase of IgG4-positive plasma cells. The patient presented with acute abdomen, suspicious of acute appendicitis. Diagnostic laparoscopy showed tumefaction of the cecal pole and appendix vermiformis. Histologic examination revealed mural thickening and a dense lymphoplasmocytic, partly obliterative infiltrate of the veins with sparing of the arteries, diagnostic of ELP. In addition, we found an elevated number of IgG4-positive plasma cells blended in with the lymphocytes. The IgG4-to-IgG ratio accounted for >40 %. This case meets the histopathological criteria requested for IgG4-related disease (IgG4-RD) and thus opens the possibility that ELP might be part of the IgG4-RD spectrum.

Enterocolic lymphocytic phlebitis preceding the development of inflammatory bowel disease: report of a case

Enterocolic lymphocytic phlebitis preceding the development of inflammatory bowel disease: report of a case

Enterocolic Lymphocytic Phlebitis Presenting as an Intussuscepting Cecal Mass

Enterocolic Lymphocytic Phlebitis Presenting as an Intussuscepting Cecal Mass

Vasculitides of the gastrointestinal tract

Systemic vasculitis is often not considered as a possible diagnosis by clinicians because of its low prevalence compared with other more common diseases. Vasculitis can affect any end organ, and it is therefore often missed early on in disease progression. Gastrointestinal (GI) manifestations of vasculitis are considered rare and the presentation is often nonspecific. However, if there is significant involvement of the major vessels of the gastrointestinal system, life-threatening sequelae, including perforation and bowel ischemia, may occur. This makes early and immediate management crucial to improve long-term morbidity and mortality. Diagnosis of various GI vasculitides often relies on correlation of clinical manifestations with pathology and additional investigations. This paper reviews the various vasculitides that affect the GI tract, including systemic lupus erythematosus, mixed connective tissue disease, Henoch Schönlein purpura, polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, microscopic polyangiitis, enterocolic lymphocytic phlebitis, and Behcet's disease. Segmental arterial mediolysis, mistakenly believed to be a vasculitis, is also discussed.

An incidental enterocolic lymphocytic phlebitis pattern is seen commonly in the rectal stump of patients with diversion colitis superimposed on inflammatory bowel disease

Aims:Enterocolic lymphocytic phlebitis (ELP) is an uncommon cause of bowel pathology and most frequently results in ischaemia. It is characterised by an artery-sparing, venulocentric lymphoid infiltrate that causes a phlebitis...

Enterocolic lymphocytic phlebitis and lymphocytic colitis: drug-related coexistent pathology

Dear Editor: Enterocolic lymphocytic phlebitis (ELP), a term coined in 1989, is an entity of unknown etiology and pathogenesis. This term is synonymous with mesenteric veno-occlusive disease, granulomatous phlebitis, necrotizing phlebitis, and myointimal hyperplasia. It is a spectrum of disease with lymphocytic phlebitis at one end of the spectrum, and thrombosis and myointimal hyperplasia at the other. ELP is a rare type of venulitis involving only the submucosal, subserosal, and mesenteric veins and venules of bowel wall. The arteries and arterioles, as well as the systemic circulation, are typically spared. Whilst a definitive cause has not been isolated, specific drugs have been associated with ELP. The entity of lymphocytic colitis (LC) was first described as “microscopic colitis” in 1980. An infiltrate of T lymphocytes within the epithelium in excess of 20 lymphocytes per 100 epithelial cells, features of epithelial injury, chronic inflammatory infiltrate in lamina propria, and absence of a prominent subepithelial collagen band, characterize LC histologically. The patients typically present with chronic diarrhea but the colon has a normal appearance endoscopically. LC was considered idiopathic until recently when infectious agents and drugs were proposed to be the possible triggers. We wish to report a 73-year-old man with a mechanical aortic valve (replaced twice), grade III left ventricular function, and a history of triple artery bypass graft, who presented with iron-deficiency anemia. Colonoscopy was performed to investigate the cause of the anemia and a large sessile polyp was found in ascending colon. Endoscopic removal of the polyp was unsuccessful, as the centre of the polyp could not be raised by saline instillation. Following a histopathological diagnosis of tubulo-villous adenoma with high-grade dysplasia, a right hemicolectomy was performed. It is noteworthy that the patient did not have any history of abdominal pain, diarrhea, alteration of bowel habits, or mass in abdomen at the time of presentation. The patient had been taking warfarin, aspirin, betablockers, angiotensin-converting enzyme inhibitor, statins, lasix, amiodarone, levothyroxine, and ferrous gluconate for several months for his cardiovascular complaints and irondeficiency anemia. The right hemicolectomy resection specimen was examined after formalin fixation and comprised an 18-cm segment of colon, 3 cm of terminal ileum, and an appendix measuring 6.5 cm in length. A polypoid mass, 2.5×2.5× 0.4 cm was identified in the proximal ascending colon. The rest of the resection specimen were grossly unremarkable, especially the mucosa which did not show evidence of ulceration, hemorrhage, or congestion. Histological examination of the mass was in keeping with the previous biopsy and the final diagnosis was tubulo-villous adenoma with isolated high-grade foci, no invasion, and complete excision. The grossly “normal” colon revealed thickening of the submucosal venous walls due to edema and a concentric, dense lymphocytic infiltrate. The lymphocytes permeated the entire thickness of the vessel wall and they did not show atypia. Several eosinophils were seen but neutrophils and plasma cells Int J Colorectal Dis (2009) 24:473–474 DOI 10.1007/s00384-008-0573-2

Enterocolic lymphocytic phlebitis: clinicopathologic features and review of the literature.

Enterocolic lymphocytic phlebitis (ELP) is a recently described entity and is of unknown etiology and pathogenesis. It is characterized by phlebitis of the bowel wall and mesentery, without arterial involvement or evidence of systemic vasculitis. The clinical presentation of ELP is varied, but it most commonly manifests with signs of an acute abdomen. Clinical, radiologic, and endoscopic findings are often conflicting and misdiagnosis is common as venous thrombosis is not suspected. The diagnosis of ELP is obtained histologically. There is a spectrum of histologic features associated with ELP, which includes lymphocytic phlebitis, necrotizing phlebitis, granulomatous phlebitis, and myointimal hyperplasia. Other features include venous thrombi and acute ischemic changes of the intestine. Surgical resection of the affected bowel is usually curative and recurrences are rare. The clinical and histopathologic features of ELP are reviewed.

Enterocolic Lymphocytic Phlebitis: Clinicopathologic Features and Review of the Literature

Enterocolic lymphocytic phlebitis (ELP) is a recently described entity and is of unknown etiology and pathogenesis. It is characterized by phlebitis of the bowel wall and mesentery, without arterial involvement or evidence of systemic vasculitis. The clinical presentation of ELP is varied, but it most commonly manifests with signs of an acute abdomen. Clinical, radiologic, and endoscopic findings are often conflicting and misdiagnosis is common as venous thrombosis is not suspected. The diagnosis of ELP is obtained histologically. There is a spectrum of histologic features associated with ELP, which includes lymphocytic phlebitis, necrotizing phlebitis, granulomatous phlebitis, and myointimal hyperplasia. Other features include venous thrombi and acute ischemic changes of the intestine. Surgical resection of the affected bowel is usually curative and recurrences are rare. The clinical and histopathologic features of ELP are reviewed.

Enterocolic Lymphocytic Phlebitis: Statistical Analysis of Histology Features in Viable and Ischemic Bowel

Enterocolic lymphocytic phlebitis is a rare cause of segmental ischemic enterocolitis. This artery-sparing transmural vasculitis is classically a circumferential phlebitis with perivenular lymphocyte cuffing and thrombi in the absence of systemic manifestations. Myointimal hyperplasia may represent a chronic phase of enterocolic lymphocytic phlebitis. Subclinical or early stage enterocolic lymphocytic phlebitis is not well delineated. We analyzed 600 submucosal and subserosal veins from both ischemic and intact bowel segments to discern if vascular morphology varied between sites. Crescentic and circumferential lymphocytic phlebitis is more common in viable bowel than in the ischemic segment. A nonsignificant trend was found for increased crescentic morphology between intact bowel remote from the ischemic focus compared with that adjacent to the ischemic focus. Hallmarks of ischemic bowel are necrotizing phlebitis and thrombi formation. Thrombophlebitis morphology is distinctly different in viable and ischemic bowel, changing from the classic lymphocytic to necrotizing lesions respectively.

Chronic Antral Ulcer Associated With Gastroduodenal Lymphocytic Phlebitis

Enterocolic lymphocytic phlebitis (ELP) is a rare cause of gastrointestinal ischemia. Unlike most vasculitic diseases affecting the gastrointestinal tract, ELP involves only the mural and mesenteric veins, which are surrounded by a lymphocytic and sometimes granulomatous infiltrate. The mesenteric arterial system and the systemic vasculature are characteristically spared. Most patients with ELP present with an acute abdomen that resolves following surgical resection of the involved bowel. ELP has been reported to involve the small bowel, colon, or both, but involvement of the upper gastrointestinal tract has not been previously described. Here we report a case of lymphocytic phlebitis that affected only the stomach and duodenum. The patient, a 68-year-old man, had a nonhealing gastric antral ulcer and underwent hemigastrectomy with vagotomy and Billroth II reconstruction. Both the resected stomach and duodenum showed characteristic lymphocytic and granulomatous infiltrates that involved the submucosal and mural veins, with associated obliteration of vascular lumina; the adjacent arteries were completely spared. The patient developed late postoperative complications including bile reflux gastritis and erosive esophagitis, but he had no recurrence of gastrointestinal ulceration or ischemia over a 2-year follow-up. We hypothesize that there may be more cases of upper gastrointestinal ELP than are diagnosed as such, in part because the diagnosis can be made only on surgical resections specimens.

Enterocolic Lymphocytic Phlebitis

Venous compromise as a cause of ischemic colitis and enteritis is infrequent. Enterocolic lymphocytic phlebitis, a rare cause of such compromise, to our knowledge has been reported in only three patients. We describe a patient with this condition who had hematochezia and a cecal mass that was mistaken endoscopically and radiologically for carcinoma and required resection. The mass, which was ulcerated, was due to intense submucosal edema. The patient had no history of taking hydroxyethylrutoside, a drug used in Europe in treating varicose veins that has been implicated in all three previously reported cases. This entity is characterized by a mixed T- and B-cell vasculitis affecting veins exclusively in the wall and mesentery of the colon and small bowel, without systemic involvement, leading to fibrointimal proliferation, thrombosis, venous occlusion, and subsequent edema and ischemic necrosis. It should be distinguished from other enterocolic phlebitides, including Behçet disease, systemic lupus erythematosus, and myointimal hyperplasia of mesenteric veins, and can be treated by local excision of the affected bowel.