Clinical and serological features of severe vasculitis in rheumatoid arthritis: prognostic implications.

Sixteen patients with classic rheumatoid arthritis (RA) complicated by severe vasculitis were studied and compared with a matched control group of 16 RA patients without vasculitis. Seven of the patients with vasculitis died within 4 to 120 months (median 32 months) after developing vasculitic symptoms. Gangrene of digits and extremities, bowel ulcers or bowel perforation, or both, and cardiac involvement were more common among the patients who died than among those with a more favourable course. The present data suggest that large vessel vasculitis in RA is associated with high frequency of arteriosclerotic vascular disease. The serum concentrations of complement components C3 and C4 were lower, and concentrations of IgM rheumatoid factor, complement activating rheumatoid factor, and C1q binding immune complexes (C1q solid and C1q fluid phase assay) were significantly higher among vasculitic patients than in the control group. Laboratory data provided little prognostic information with regard to rheumatoid vasculitis, with the exception that IgM and IgG rheumatoid factors were significantly higher among patients with fatal course of disease than in those who achieved remission.     

Isolated Colonic Leukocytoclastic Vasculitis Causing Segmental Megacolon: Report of a Rare Case

Abstract We report the case of a 44-year-old white man who presented with progressively worsening crampy abdominal pain and distention. Deterioration of his clinical picture along with leukocytosis and radiographic evidence of severe colonic dilation rendered exploratory laparotomy necessary. Greatly distended and inflamed transverse and descending colon were evident and an extended left colectomy was performed. Characteristic changes of leukocytoclastic vasculitis in the serosal and muscular layers of the resected colon were demonstrated at histopathologic examination. Systemic leukocytoclastic vasculitis, usually coexisting with Henoch-Schönlein purpura, commonly affects the small bowel with clinical evidence of ischemia or bleeding. Colon involvement is infrequently reported in the context of systemic disease. Isolated colonic leukocytoclastic vasculitis without extraintestinal manifestations is rare. A previously unreported case of localized leukocytoclastic vasculitis of the left colon resulting in the impressive presentation of megacolon, without the presence of any precipitating factor or associated systemic disease is presented here, with an overview of the related literature.     

Massive lower gastrointestinal bleeding as a presenting feature of Behcet's disease

This reports a 27 yr old female Patient with seronegative polyarthritis who presented with lower GI bleeding and subsequently developed evidence of cutaneous vasculitis (palpable purpura) and orogenital ulcers. The cause of the GI bleeding was multiple ulcerations in the terminal ileum, cecum ascending colon and transverse colon. Continued and massive bleeding necessitated surgical resection of the involved segment of gut which on histopathological examination confirmed the presence of vasculities. This patient was thus diagnosed as having Behcet's disease. Although major GI bleeding is a rare manifestation of Behcet's disease with vasculitis of the gut, one must consider this possibility if the patient has multi-system involvement with evidence of vasculitis involving the skin in the form of palpable purpura.     

Diffuse ulcerative lesions in the colon with intestinal Beh?et's disease

Ileocecal region is most commonly affected in intestinal Beh?et's disease. Localized deep ulcerations which have a punched-out or undermining appearance with well-defined margin placing in this region had been noted in many cases. 41 patients with intestinal Beh?et's disease were observed by colonofiberscope in Teikyo University Hospital from January 1982 to June 1988. In 10% (4 cases) of these patients, diffuse ulcerative lesions in the transverse colon and the distal colon were found. These diffuse lesions in the colon with Beh?et's disease were seemed to be much like those associated with inflammatory bowel disease as ulcerative colitis and Crohn's disease in morphological characteristics. The clinical symptoms of these four cases with Beh?et's disease including oral aphtha, genital ulcer, joint pain, skin eruptions, and eye involvement, preceded each colonic ulceration. The first two cases were affected with ulcerative lesion in the distal portion of the recto-sigmoid colon and the descending colon. And the last two were involved in the whole transversing colon by multiple deep ulcerations. Wide-spreading ulcerative lesion in the colon with Beh?et's disease is a rare condition in the Japanese, and only two papers has been reported previously. Because clinical manifestations between Beh?et's disease and inflammatory bowel disease are very similar in many points as oral aphthous ulcers, arthritis, eye involvement, skin eruptions, phlebitis, arterial occlusions, then, an overlapping field or a part of a spectrum of disease has been suggested by some authors. But, in the present circumstances, they are distinct disease entities, we think that these four cases are in an atypical condition with intestinal Beh?et's disease.     

Perforation of rectal diverticulum with amyloidosis secondary to rheumatoid arthritis: case report and review of the literature

We report a case of perforation of a rectal diverticulum with amyloidosis secondary to rheumatoid arthritis (RA), and review the clinicopathologic features in 21 Japanese amyloidosis patients with colorectal perforation. A 62-year-old woman with amyloidosis secondary to RA suddenly complained of abdominal pain. Computed tomography (CT) showed ascites and free air in the abdominal cavity, and many diverticula with calculi in the sigmoid colon. Emergent surgery was performed for acute peritonitis. We observed the perforation, 5 mm in diameter, of a diverticulum in the upper rectum, and many diverticula located in the upper rectum and sigmoid colon. Anterior resection of the rectum combined with sigmoidectomy was performed. The resected specimen showed many diverticula including fecaliths, approximately 7 mm in size. Histopathologically, many inflammatory cells had infiltrated around the perforation. On Congo red staining, amyloid deposits were observed in or around the small blood vessels of the lamina propria mucosa and submucosa of the rectum. Although colorectal perforation with amyloidosis secondary to RA is rare, this complication was associated with poor prognosis. Furthermore, in patients with amyloidosis, we should be careful regarding the management of colorectal diverticula.     

Gold Induced Enterocolitis: Case Report and a Review of the Literature

Abstract: The use of gold therapy in rheumatoid arthritis is not without risk. Although the better known side effects are bone marrow suppression and renal involvement, gold induced enterocolitis has been described. This paper reports a 59-year-old female recently treated with gold therapy for rheumatoid arthritis who developed bloody diarrhoea, toxic dilatation and perforation of her colon. The absence of features of inflammatory bowel disease or infection indicates gold as a possible causative agent. Pathogenesis, therapy and a review of the literature of gold induced enterocolitis are described.     

Severe colonic complications of pancreatic disease

INTRODUCTION: Colonic involvement in pancreatic disorders is rare but potentially fatal. Extension of contiguous inflammation or neoplasm, autodigestive effects of enzymes, or dissection of a pseudocyst or abscess may involve the colon producing obstruction, perforation, hemorrhage, or abdominal pain. RESULTS: Nine patients with pancreatic disease requiring colonic resection were identified. Cases included pancreatic abscess producing colonic necrosis (2). pancreatic carcinoma invading the colon (3). extension of pancreatitis producing a colonic stricture (3). and pseudocyst eroding into the splenic flexure (1). Presentation was varied, including rectal bleeding (2). clinical deterioration during severe pancreatitis (4). and large bowel obstruction (3). The 3 cases due to malignancy, 1 of which was recurrent, presented with primary large bowel symptoms suggesting intestinal obstruction rather than pancreatic disease. Typically, patients with severe acute pancreatitis had colonic pathology obscured and unrecognized initially because of the ongoing, fulminant inflammatory process. CONCLUSIONS: Recognition of large bowel involvement may be difficult because of nonspecific symptoms or be masked by the systemic features of a critical illness. Colonoscopy, contrast x-rays, or CT scan may be vital in selected cases to detect underlying pathology. Clinicians should be aware that acute or chronic pancreatitis or pancreatic carcinoma may compress, erode, or inflame the large bowel, resulting in life-threatening colonic necrosis, bleeding, obstruction, or perforation.     

Non-steroidal anti-inflammatory drug-induced small bowel injuries identified by double-balloon endoscopy

AIM: To clarify clinical features of the NSAID-induced small bowel lesions using a new method of endoscopy. METHODS: This is a retrospective study and we analyzed seven patients with small bowel lesions while taking NSAIDs among 61 patients who had undergone double-balloon endoscopy because of gastro-intestinal bleeding or anemia between September 2000 and March 2004, at Jichi Medical School Hospital in Japan. Neither conventional EGD nor colonoscopy revealed any lesions of potential bleeding sources including ulcerations. Double-balloon endoscopy was carried out from oral approach in three patients, from anal approach in three patients, and from both approaches in one patient. RESULTS: Ulcers or erosions were observed in the ileum in six patients and in the jejunum in one patient, respectively. The ulcers were multiple in all the patients with different features from tiny punched out ulcers to deep ulcerations with oozing hemorrhage or scar. All the patients recovered uneventfully and had full resolution of symptoms after suspension of the drug. CONCLUSION: NSAIDs can induce injuries in the small bowel even in patients without any lesions in both the stomach and colon.     

超声在结肠憩室诊断中的应用价值

目的通过对结肠憩室超声表现的分析，探讨结肠憩室临床诊断中超声的应用价值。 方法回顾性分析25例经手术病理和（或）结肠镜检查证实的结肠憩室病例，观察病灶的位置、形状、局部肠壁有无增厚，有无合并肌层缺如、病变周围网膜回声有无改变、有无渗液、是否合并粪石或气体等。 结果25例患者中，病灶共计67个，单发16例16个，多发9例51个（2~20个不等，仅1例多发憩室合并1个管状，余均呈大小不等囊袋状），升结肠15例、盲肠4例、降结肠2例、乙状结肠2例，结肠肝曲1例、升结肠伴降结肠1例，其中合并阑尾炎13例、腹膜炎3例、肠穿孔2例、系膜扭转1例、肠道出血1例、腹腔脓肿1例。超声表现为结肠旁囊袋状结构19例、呈囊袋状同时伴局部肠壁增厚4例、盲管状结构1例、局限性低回声凸起1例。合并肌层缺损13例、周围网膜回声增强16例、渗液4例、粪石8例、气体3例。 结论结肠憩室声像图多表现为结肠旁囊袋状结构，局部可伴肠壁增厚，部分伴肌层缺损，易合并粪石或气体，易并发阑尾炎，均伴有不同程度炎症表现，少数可合并穿孔，肠道超声检查能为临床明确诊断提供可视化的重要依据，尤其在排他性诊断中具有很重要的意义，值得临床推广应用。     

Endoscopic management of a rare granulation polyp in a colonic diverticulum

There are many case reports on colon diverticula that cause irritable bowel syndrome,constipation,bleeding,diverticulitis,stricture due to multiple recurrences of diverticulitis,and perforation.However,few articles have examined neoplasms that arise from a diverticulum,such as adenoma and adenocarcinoma,and there have been no reports of granulation polyps that arise from a colon diverticulum after recurrent diverticulitis.We observed a rare granulation polyp that arose from a diverticulum as a result of repeated episodes of local diverticulitis.Narrow band imaging magnified colonoscopy was very useful to diagnose the polyp as a granulation polyp because of the absence of a pit pattern on the surface of the polyp.We successfully resected the polyp using endoscopic mucosal resection.We inverted the diverticulum,and the resected stalk of the polyp was used to close the diverticulum with an over-thescope clip.If a granulomatous polyp could arise from a diverticulum,differential diagnosis between a colon neoplasm and a granulomatous polyp would not only be difficult but also necessary for suitable endoscopic treatment.     

Anatomical involvement and clinical features in 91 Japanese patients with Crohn's disease

Ninety-three patients with Crohn's disease who had not undergone bowel resection at the time of diagnosis (during the years 1969-1983) were selected for the study, to elucidate whether initial anatomical involvement correlates with clinical manifestations, complications, laboratory findings, and prognosis. Forty-one patients had isolated small intestine disease (44%), 37 ileocolic (40%), 13 colonic disease alone (14%), and two rectal or appendix involvement (2%). Statistically significant symptoms were fever and rectal bleeding with colon involvement, and number of liquid or very soft stools in 1 week with ileocolic or colon involvement. Statistically significant complications included intestinal obstruction with ileocolic disease, perianal fistulas, and arthritis with colonic disease. Increases in ESR, C-reactive protein, and white blood cell in patients with colon involvement were among the statistically significant laboratory findings. Gammaglobulin was significantly higher in patients with ileocolic or colon involvement than in those with small intestine disease. Red cell count and hemoglobin were significantly lower in patients with ileocolitis than in patients with small intestine involvement. Crohn's disease activity index (CDAI) was significantly higher in patients with ileocolic or colon involvement than in those with small intestine involvement only. These findings confirm that colonic involvement renders the disease more severe than involvement of the small intestine. Surgery was required for 22 patients (24%) during a 2.8-year follow-up, but the difference among the three groups was not significant.     

Laryngeal involvement in systemic lupus erythematosus.

Laryngeal involvement in systemic lupus erythematosus (SLE) can range from mild ulcerations, vocal cord paralysis, and edema to necrotizing vasculitis with airway obstruction. In this report, four cases showing the range of severity of this disease manifestation are presented, accompanied by a comprehensive review of the literature. The clinical course of 97 patients with laryngeal involvement with SLE are reviewed, of whom 28% had laryngeal edema and 11% had vocal cord paralysis. In the majority of cases, symptoms such as hoarseness, dyspnea, and vocal cord paralysis resolved with corticosteroid therapy. Other, less common causes of this entity included subglottic stenosis, rheumatoid nodules, inflammatory mass lesions, necrotizing vasculitis, and epiglottitis. The clinical presentation of laryngeal involvement in patients with SLE follows a highly variable course, ranging from an asymptomatic state to severe, life-threatening upper airway compromise. With its unpredictable course and multiple causations, this complication remains a diagnostic and therapeutic challenge to physicians involved in the care of patients with SLE.     

Hypertrophic osteoarthropathy and rheumatoid arthritis. Simultaneous occurrence in association with diffuse interstitial fibrosis

A patient is described who was treated with high-dose prednisone in an attempt to halt progressive respiratory insufficiency associated with diffuse interstitial fibrosis. On cessation of steroid therapy the patient was noted to have radiologic manifestations of hypertrophic osteoarthropathy (HOA) as well as clinical and laboratory features of rheumatoid arthritis (RA). Subsequently a diffuse vasculitis developed with bowel perforation and sepsis leading to death.     

Gastrointestinal Manifestations of Beh?et’s Disease

Behçet’s disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of life are mainly affected, with abdominal pain being the most common symptom. The ileocecal region is most commonly affected, with ulcerations that may penetrate or perforate. Rarely, the esophagus and stomach may have ulcerations. Bowel wall thickening is the most common finding on computed tomography (CT) scan. Pathology shows a vasculitis mainly involving the small veins or, alternatively, nonspecific inflammation. Corticosteroids, with or without other immunosuppressive drugs, are used for severe eye disease. Their use in intestinal disease is largely empirical. Surgery may be required for perforation. Behçet’s disease runs a chronic, unpredictable course with exacerbations and remissions which decrease in frequency and severity over time. Death is mainly due to major vessel disease and neurological involvement.     

Ulcerative colitis complicating seronegative HLA-A2-B27 rheumatoid arthritis with sacroiliitis.

The case is reported of a 50 year old man with longstanding seronegative rheumatoid arthritis who developed ulcerative colitis. The patient also had sacroiliitis and his tissue was typed as HLA-A2-B27 several years before the bowel disease began. A possible overlap between primary inflammatory bowel disease, complications to the treatment of rheumatoid arthritis with drugs, and gastrointestinal rheumatoid vasculitis is discussed.     

Endoscopic Features and Clinical Characteristics of Ulcerations With Isolated Involvement of the Small Bowel

BackgroundDouble-balloon enteroscopy (DBE) enables the detection of ulcerations in the small bowel. However, determining an etiological diagnosis remains challenging. This study was conducted to investigate the clinical and endoscopic features of ulcerations with isolated involvement of the small bowel (UIISB) to improve diagnostic ability.MethodsPatients (n = 565) who underwent DBE and presented with ulcerations in the small bowel at Nanfang Hospital from January 2005 to January 2018 were eligible. Medical records were retrospectively examined. Predictors to determine ulceration etiology were identified by logistic regression analysis.ResultsAfter excluding patients with extra-ulcerations in other sites (n = 306) and those without follow-up records (n = 50), 209 patients with UIISB were enrolled. Among them, 59.3% of the ulcers were in the ileum, 26.8% in the jejunum, and 13.4% in the jejunoileum. Initial symptoms included abdominal pain (54.1%) and obscure gastrointestinal bleeding (30.0%). The multiplicity of ulceration was categorized as a single (22.0%) or multiple (78.0%). Cases were diagnosed with Crohn’s disease (50.7%), chronic nonspecific inflammation (21.5%), diverticulum (9.1%), lymphoma (6.2%), gastrointestinal stromal tumor (4.3%), intestinal tuberculosis (1.9%), adenocarcinoma (1.4%), infective enteritis (1.4%), hemangioma (1.0%), cryptogenic multifocal ulcerous stenosing enteritis (1.0%), anastomotic ulcer (0.5%), intestinal duplication (0.5%), or neuroendocrine tumor (0.5%). Etiology identification indicated the if patients were aged 40 years or more, or had overt bleeding, single ulceration, and ulcer at jejunum, it as more prone to be neoplastic (P < .05).ConclusionWhen we manage patients with UIISB, Crohn’s disease should be first under consideration. Age≥40, overt bleeding, single ulceration, and ulcer at jejunum were reasonable indications for etiology of neoplasm or non-neoplasm.     

Laparoscopic-assisted bowel surgery

The use of laparoscopic surgical techniques is now being applied to a variety of operations traditionally performed in an open fashion. Twenty patients underwent laparoscopic-guided large and small bowel surgery at our institution from March 1991 to April 1992. The indications for surgery included polyps, obstruction, bleeding, and perforation, and pathologic diagnoses included benign polyps, lipomas, inflammatory bowel disease, perforation of a jejunal diverticulum, colonic arteriovenous malformations, and adenocarcinoma. Mobilization of the colon, ligation of the mesentery, and closure of the mesenteric defect were performed using the laparoscopic equipment. One trocar site was enlarged to 3 cm to deliver the bowel through the abdominal wall. All anastomoses were hand-sewn. Postoperative hospitalization ranged from 2 to 31 days (median, five days). No mortality was noted, and morbidity was 20 percent. We conclude that laparoscopic-guided bowel surgery is technically feasible and should translate into shorter hospitalization and less patient discomfort.     

A case of Non-Hodgkin's lymphoma in a patient with Crohn's disease]

Although adenocarcinoma is a well known complication of chronic inflammatory bowel disease, primary gastrointestinal lymphoma occurring in Crohn's disease is rare. A 40-year-old man with 10 year-history of Crohn's disease had multiple longitudinal ulcerative lesions on descending colon in follow-up colonoscopic examination. Microscopic examination of proximal descending colon revealed peripheral T cell lymphoma and other site of the descending colon was consistent with Crohn's disease. The patient reached complete remission of malignant lymphoma after three cycles of combined chemotherapy. He has been well for 10 months with sulfasalazine maintenance therapy but was admitted to the hospital due to spontaneous bowel perforation of ascending colon. Right hemicolectomy was done, but the patient died of post-surgical recurrent mesenteric abscess and sepsis. To the best of our knowledge, this is the first case of Non-Hodgkin's lymphoma complicating Crohn's disease in Korea which was confirmed by immunohistochemical studies.     

Rheumatoid aortitis: a rarely recognized but clinically significant entity

Aortitis as a feature of rheumatoid arthritis is considered rare. We have, however, identified 10 patients with aortitis from among 188 consecutive autopsy cases of rheumatoid arthritis. There were 5 men and 5 women with a mean duration of rheumatoid arthritis of 9.6 years. Nine were rheumatoid factor positive and had associated nodules. In addition to standard treatment regimens, 9 patients received corticosteroids. Although involvement of the thoracic aorta was most common, involvement of both the thoracic and abdominal aorta was present in 4 cases. Two patients had aneurysmal dilatation of the thoracic aorta and 1 of the abdominal aorta. Microscopic features of aortitis included necrosis of medial smooth muscle and elastica, with an inflammatory infiltrate comprising primarily lymphocytes and plasma cells. A panmural aortitis was seen in 3 cases. Rheumatoid granulomas were noted in the aortic wall in 5. The diagnosis of aortitis was not made until autopsy in any case. Aortitis was hemodynamically significant in 3 patients. Two had congestive heart failure secondary to thoracic aortitis and aortic valvulitis, and 1 had rupture of an abdominal aortic aneurysm at a site involved by aortitis. Seven patients had rheumatoid vasculitis with a mean of 10 organs involved. Six of these died of complications directly related to vasculitis, including 4 patients with coronary arteritis and associated myocardial infarction. Aortitis can be a feature of severe rheumatoid arthritis and is often associated with rheumatoid vasculitis. Hemodynamic compromise does occur and may be fatal.     

Esophageal involvement in Wegener's granulomatosis: a case report and review of the literature.

Wegener's granulomatosis is characterized by a granulomatous arteritis involving the upper and lower respiratory tracts, progressive glomerulonephritis and systemic symptoms attributable to small vessel vasculitis. Although multisystemic manifestations are frequent, involvement of the gastrointestinal tract is uncommon. Cases have been reported of intestinal perforation, ulceration and hemorrhage. A patient whose initial presentation of Wegener's granulomatosis was odynophagia secondary to esophageal vasculitis is described. Endoscopy revealed multiple punched out ulcerations in the esophagus, which resolved with standard therapy for systemic Wegener's granulomatosis. There are only two previous reports of symptomatic esophageal vasculitis in patients with Wegener's granulomatosis. These reports illustrate the need to consider odynophagia as a reflection of disease activity as opposed to complications of immunosuppressive therapy.