Cecal cytomegalovirus infection following appendicectomy in a patient with ulcerative colitis

We report a patient who, 3 months after being diagnosed with ulcerative colitis, was admitted to hospital because of malaise and right lower abdominal pain. An open appendectomy was performed. Histological study showed ulcerative colitis affecting the appendix. After surgery, the patient presented a refractory outbreak of ulcerative colitis requiring treatment with steroids and cyclosporin A. Despite this treatment, the patient continued to pass abundant fresh blood associated with severe anemia. Colonoscopy showed only granular and congestive cecal mucosa. Biopsies showed intracytoplasmic inclusion bodies with immunohistochemical stains positive for cytomegalovirus (CMV) infection. Rectorrhagia and anemia quickly disappeared after beginning treatment with ganciclovir. Appendicular ulcerative colitis is not uncommonly associated with distal colitis. In addition, diffuse CMV infection complicating ulcerative colitis treatment is not unusual. By contrast, isolated, segmentary infection by CMV in the proximal colon is extremely rare. Until now, only three patients with localized CMV infection have been described, and all three cases occurred in the context of ileoanal anastomosis.     

Retroperitoneal fibrosis during the course of ulcerative colitis. A simple coincidence?

Retroperitoneal fibrosis has been described as a rare occurrence during the course of inflammatory bowel disease, mainly Crohn's disease. This is the third report on retroperitoneal fibrosis occurring during the course of ulcerative colitis. A 62-year-old male patient with a 5-year history of ulcerative colitis developed stenosis of the left ureter due to retroperitoneal fibrosis. Treatment consisted in surgically releasing the ureter from the mass and steroids. During a 2.5-year follow-up, renal function was stable and ulcerative colitis in remission. Important aspects of this case are the moderate course of ulcerative colitis, ultrasound confirmation of normal kidney structure before manifestation of fibrosis, hypertension diagnosed four years before retroperitoneal fibrosis, a non-functioning kidney at diagnosis, and reduction of retroperitoneal mass after steroid treatment. Retroperitoneal fibrosis, although a rare disease entity should be considered when a patient with ulcerative colitis develops otherwise unexplained renal insufficiency.     

Successful treatment of ulcerative colitis complicated by Sweet’s syndrome by corticosteroid therapy and leukocytapheresis

Ulcerative colitis is occasionally complicated by dermatological disorders presenting as extra-intestinal manifestations, including erythema nodosum and pyoderma gangrenosum. Sweet’s syndrome is considered to be a rare cutaneous disease in patients with ulcerative colitis. To date, only 17 cases of Sweet’s syndrome complicating ulcerative colitis have been reported in the English literature. Here, we report a case of a 41-year-old male who had been suffering from ulcerative colitis for 20 years. He was admitted to hospital with hematochezia, diarrhea and fever, and painful erythematous nodules on the face and arms. Histological examination of skin biopsies showed inflammatory cell infiltration composed mainly of neutrophils without evidence of necrotizing vasculitis, and the condition was diagnosed as Sweet’s syndrome. The patient was treated with prednisolone and leukocytapheresis and the erythematous nodules on the skin, as well as the abdominal symptoms and endoscopic findings of ulcerative colitis, immediately improved. In this paper we report on this case and review the literature concerning ulcerative colitis and Sweet’s syndrome.     

Sporadic adenoma in ulcerative colitis: endoscopic resection is an adequate treatment

BACKGROUND AND AIMS: In studies with small numbers of cases, it has been shown that endoscopic resection of adenomas in ulcerative colitis represents adequate treatment. In a larger study cohort with more prolonged follow up, we assessed the reliability of this finding. METHODS: Between 1988 and 2002, 148 consecutive patients, mainly from private gastroenterologists' practices, with ulcerative colitis were diagnosed as having an adenoma. In 60 patients, histological diagnosis was established in biopsies and in 87 patients in polypectomy specimens; one patient underwent proctocolectomy following diagnosis. The outcome of these patients was analysed after a mean follow up period of 6.0 (3.63) years. RESULTS: Among 60 patients, surprisingly without endoscopic treatment, 48.3% developed ulcerative colitis associated neoplasia in the same colon segment (23.3% low grade intraepithelial neoplasia; 8.3% high grade intraepithelial neoplasia; 16.7% carcinoma). Among 87 patients undergoing polypectomy of the adenoma, follow up revealed colitis associated neoplasia in other segments of colon in 4.6% of cases. CONCLUSION: Development of adenocarcinomas in a total of 6.7% of the overall patient group, and in 2.3% of those undergoing polypectomy, indicates that biopsy based diagnosis of an adenoma in ulcerative colitis must be considered to mandate endoscopic resection of the lesion; 40% of affected patients did not receive any form of endoscopic removal of the lesion. This shows that the most recent guidelines are not followed in a considerable number of patients with ulcerative colitis in private practice in Germany. Although polypectomy of the adenoma represents adequate therapy, further regular follow up examinations are nevertheless necessary.     

Development of intrahepatic cholangiocarcinoma after a 14-year follow-up of a patient with primary sclerosing cholangitis and ulcerative colitis

Intrahepatic cholangiocarcinoma (ICC) is one of the life‐threatening complications of primary sclerosing cholangitis (PSC). However, the incidence of ICC in Japanese PSC patients is low, and the association between the development of ICC and morbidity duration of PSC is largely unknown. Here, we describe a case of ICC that developed after a long‐term follow‐up of a patient with PSC and ulcerative colitis (UC). At the age of 10 years, the patient was first diagnosed with UC and its remission was achieved with systemic steroid therapy. Since then, he was routinely followed‐up. At the age of 19 years, laboratory tests showed abnormalities in liver function parameters, and the patient was diagnosed with PSC. Although treatment with ursodeoxycholic acid improved the abnormalities in serum levels of biliary enzymes and no PSC‐related symptoms were seen for 13 years, calculous cholecystitis frequently occurred in the patient since the age of 32 years. He developed ICC, which expressed some hepatic progenitor cell markers such as CD133, neural cell adhesion molecule, keratin 7, and keratin 19 at the age of 33 years. ICC was treated by curative partial hepatectomy and adjuvant chemotherapy with gemcitabine. Eight months later, however, the patient developed multiple metastases in the abdominal lymph nodes and lungs, and died 21 months after the onset of ICC. Here, we report a case of ICC that developed after a 14‐year follow‐up of a patient with PSC and UC.     

Diffuse gastroduodenitis associated with ulcerative colitis: Treatment by infliximab

Diffuse gastroduodenitis resembling ulcerative colitis in respect to macro‐ and microscopic findings occurs in ulcerative colitis, although it is rare. Reports of gastroduodenitis associated with ulcerative colitis treated with infliximab are rare. A 58‐year‐old man had tarry stool in March 2011. He had a history of ulcerative colitis that was diagnosed in 1984. He underwent subtotal colectomy in 1991. Endoscopy and radiography revealed diffuse friable mucosa throughout the duodenum and an ulcer in the middle of the descending portion, resulting in a narrow portion.In the stomach, numerous small aphthae were observed in the antrum. Biopsy specimens of the duodenum and antrum showed marked inflammatory cell infiltration in both areas and cryptitis in the duodenum. Standard induction therapy of infliximab was started in April. The ulcer in the descending portion became a scar without diffuse mucosal friability in September 2011.     

自身免疫性胆管炎合并溃疡性结肠炎1例

自身免疫性胆管炎合并炎症性肠病临床上较罕见.本文报道的1例自身免疫性胆管炎合并溃疡性结肠炎患者以尿黄伴皮肤瘙痒为首发症状,经实验室检查及肝穿刺活检诊断为自身免疫性胆管炎.随诊2年后出现腹泻,经肠镜病理活检确诊合并溃疡性结肠炎.本例无典型的溃疡性结肠炎的临床表现,便常规、肠镜及病理检查提高了诊断率.     

Two for one: coexisting ulcerative colitis and Crohn's disease.

Three cases of coexisting ulcerative colitis and Crohn's disease are presented. In the first case, the patient had a long-standing history of ulcerative proctitis before developing Crohn's colitis. In the two remaining cases, the patients presented initially with Crohn's disease of the ileum and, subsequent to resection, developed ulcerative colitis. Well-documented cases of patients diagnosed with both ulcerative colitis and Crohn's disease are rare. The literature on such cases is reviewed, and the controversy over whether ulcerative colitis and Crohn's disease are two distinct diseases is explored.     

Pulmonary Infiltrates and Skin Pigmentation Associated with Sulfasalazine

A patient with ulcerative colitis developed skin pigmentation and diffuse pulmonary shadowing without respiratory symptomatology, while taking sulfasalazine. The clinical picture and radiological abnormalities disappeared spontaneously on discontinuation of the drug. Histopathological studies from specimens taken by transbronchial biopsy showed bronchiolitis obliterans with fibrosing alveolitis. Sulfasalazine-induced lung disorder is an extremely rare entity which must be considered in all ulcerative colitis patients while on sulfasalazine therapy, despite the absence of pulmonary symptomatology.     

Association of primary sclerosing cholangitis,ulcerative colitis and coeliac disease in female siblings

Primary sclerosing cholangitis is a rare, cholestatic liver disease, most commonly affecting young men. The association of primary sclerosing cholangitis with other autoimmune disorders, although rare, indicates a genetic predisposition for this disease. We describe, for the first time, the association of primary sclerosing cholangitis, ulcerative colitis and coeliac disease in two sisters. Ulcerative colitis was mild and preceded liver disease in both patients. There were no symptoms of coeliac disease, and its silent form was diagnosed on the basis of serological tests. Both patients carried HLA molecules DR3 and DQ2. Although HLA DR4 was not found, there was a rapid progression of liver disease to cirrhosis and cholangiocarcinoma in one patient. The familial occurrence of primary sclerosing cholangitis, ulcerative colitis and coeliac disease supports the hypothesis of genetic predisposition for these diseases.     

Pulmonary vasculitis complicating childhood ulcerative colitis

A case of pulmonary vasculitis complicating ulcerative colitis is presented, and the literature is reviewed. Unexplained pulmonary problems complicating severe ulcerative colitis should prompt a review of rare etiologies to include pulmonary vasculitis, apical pulmonary fibrosis, and salicylazosulfapyridine-induced lung disease. Lung biopsy will establish a diagnosis of pulmonary vasculitis.     

Acute motor sensory polyneuropathy (AMSAN) complicating active ulcerative colitis with a patchy distribution

We report a case of acute motor and sensory neuropathy during a flare of ulcerative colitis. A 28-year-old male presented with a flare of distal ulcerative colitis despite treatment with mesalamine enemas and suppositories simultaneously with rapidly deteriorating weakness and needle sensation in both legs. Neurological assessment showed axonal sensorimotor polyneuropathy affecting mainly the lower limbs and to a lesser extent the upper limbs. Colonoscopy revealed moderately to severe active ulcerative colitis with a patchy distribution involving the rectum and the right colon. Vitamin and folic acid levels were normal. Virological, immunological and other laboratory tests were negative except for positive anti-ganglioside antibodies (anti-GM1). Ulcerative colitis and polyneuropathy improved when patient was treated with immunosuppressive therapy (corticosteroids, immunoglobulin and azathioprine). Peripheral polyneuropathy is a rare extraintestinal manifestation of ulcerative colitis and it is probably associated with an autoimmune pathogenetic mechanism.     

Secondary amyloidosis (AA-type) due to localized cutaneous vasculitis

We report a case of a 49 year old man, diagnosed soon after the outcome of casual proteinuria, of AA-type amyloidosis in relation to small and medium vessel cutaneous vasculitis without systemic involvement. This combination is a rare entity and only two cases of cutaneous hypersensibility vasculitis complicated with AA-type amyloidosis had been reported. We describe the results of the use of several immunosuppressive drugs during four years follow up with temporally total remission of the disease.     

Microscopic colitis: Is it a spectrum of inflammatory bowel disease?

Lymphocytic and collagenous colitis are forms of microscopic colitis which typically presents in elderly patients as chronic watery diarrhea. The association between microscopic colitis and inflammatory bowel disease is weak and unclear. Lymphocytic colitis progressing to ulcerative colitis has been previously reported; however there is limited data on ulcerative colitis evolving into microscopic (lymphocytic or collagenous) colitis. We report a series of six patients with documented ulcerative colitis who subsequently were diagnosed with collagenous colitis or lymphocytic colitis suggesting microscopic colitis could be a part of the spectrum of inflammatory bowel disease. The median duration of ulcerative colitis prior to being diagnosed with microscopic colitis was 15 years. We noted complete histological and/or symptomatic remission in three out of six cases while the other three patients reverted back into ulcerative colitis suggesting lymphocytic or collagenous colitis could present as a continuum of ulcerative colitis. The exact molecular mechanism of this histological transformation or the prognostic implications is still unclear. Till then it might be prudent to follow up these patients to assess for the relapse of inflammatory bowel disease as well as for dysplasia surveillance.     

Purpura und Nierenversagen bei einer Patientin mit Colitis ulcerosa

Autoimmune diseases are serious for patients and difficult for physicians as the treatment is not wholly satisfactory. The case of a patient with known ulcerative colitis, who presented with palpable purpura of the lower extremities, acute renal failure and an active urinary sediment is reported. The patient had elevated antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 and the renal biopsy showed a necrotizing crescentic pauci-immune glomerulonephritis. Treatment was carried out with cyclophosphamide and prednisone; however, both the ulcerative colitis and the ANCA vasculitis continued to cause severe clinical problems which necessitated adding infliximab to the regimen and resulted in an acceptable degree of remission.     

Granulocyte‐Monocyte Apheresis as an Adjuvant Therapy to Anti‐Tumor Necrosis Factor Drugs for Ulcerative Colitis

Biologic anti‐tumor necrosis factor (TNF) drugs have demonstrated their efficacy for the treatment of ulcerative colitis. Nevertheless, some patients will not respond to this therapy or will develop loss of response. Leukapheresis is the main non‐pharmacological therapy for some immune‐mediated diseases. The aim of our study was to describe our experience with this therapy in ulcerative colitis patients after loss of response to anti‐TNF treatment. Leukapheresis was indicated in four patients with left‐sided or extensive colitis because of partial response to biological therapy or secondary loss of response to it. All patients received 8 to 10 sessions in an intensive regimen. Globally, a decrease in the Mayo score was observed. The overall response rate was 50% with one patient who displayed sustained response. No patients have required colectomy during follow‐up. Adjuvant treatment with leukapheresis in patients with inadequate response to anti‐TNF treatment showed some beneficial effect, although of limited duration, in patients with ulcerative colitis.     

Spinal epidural lipomatosis caused by corticosteroid treatment in ulcerative colitis

Spinal epidural lipomatosis is the result of deposition of unencapsulated fat in the extradural space of the spinal canal. Most commonly, this rare condition is a complication secondary to corticosteroid treatment. We describe a 49-year-old patient with ulcerative colitis who developed paraparesis due to overgrowth of epidural fat tissue. This is the second patient with ulcerative colitis described in the literature who developed symptomatic epidural lipomatosis secondary to corticosteroid treatment. All internists tending patients with chronic diseases that require corticosteroid treatment should be aware of this rare clinical entity.     

Lobular panniculitis with small vessel vasculitis associated with ulcerative colitis

We report a rare case of lobular panniculitis with small vessel vasculitis, presenting with fever, cutaneous lesions, and systemic manifestations involving the visceral fat and associated with ulcerative colitis. The patient was treated with cyclophosphamide and prednisolone, which successfully cured the systemic disease, with resolution of the inflammatory infiltrates.     

Budd-chiari syndrome complicating restorative proctocolectomy for ulcerative colitis

PURPOSE: This is a case of hepatic vein thrombosis presenting in a delayed fashion after proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis. Search for a causative thrombotic condition resulted in the diagnosis of polycythemia vera, a myeloproliferative disorder associated with hypercoagulability. The polycythemia was masked by an iron deficiency associated with the ulcerative colitis. METHODS: The history, physical, diagnostic modalities, and treatment for this patient are described, and the literature of Budd-Chiari syndrome associated with ulcerative colitis is reviewed. RESULTS: Six cases of Budd-Chiari syndrome in the setting of ulcerative colitis are reported in the literature from 1945 to 1997. CONCLUSIONS: Hepatic vein thrombosis is a rare complication of ulcerative colitis. The diagnosis of Budd-Chiari syndrome demands a thorough search for a hematologic condition predisposing to thrombosis. Our patient had a myeloproliferative disorder, polycythemia vera, that is associated with a hypercoagulable state. The disorder was masked by an iron deficiency associated with the ulcerative colitis. Recognition of the entity will permit successful treatment.     

A rare extraintestinal manifestation of ulcerative colitis: Tracheobronchitis associated with ulcerative colitis

We describe a 44 year-old woman who presented with dyspnea and cough 5 years after initial diagnosis of ulcerative colitis. The evaluation of patient showed findings of large airway inflammation with tracheal wall thickening and mucosal irregularities. The etiology was investigated and was diagnosed as tracheobronchitis that is a rare extraintestinal manifestation of ulcerative colitis. She was treated with systemic corticostreoids and rapid clinical improvement was detected.