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Background There is a small but measurable increased risk of lymphoma in inflammatory bowel disease [IBD], with a suggestion that primary intestinal lymphoma [PIL] in IBD is associated with inflamed tissue and immunosuppressant use, mainly thiopurines. Methods This multicentre case series was supported by the European Crohn's and Colitis Organisation [ECCO] and performed as part of the Collaborative Network of Exceptionally Rare case reports [CONFER] project. Clinical data were recorded in a standardized case report form. Results Fifteen patients with intestinal lymphoma from eight centres were included (12 males, 11 patients with Crohn's disease [CD], mean age 47.8 [±16.4 SD, range 26–76] years at lymphoma diagnosis). Lymphoma type was diffuse large B-cell lymphoma [DLBCL] in eight, Hodgkin's disease in two, mucosa-associated lymphoid tissue [MALT] lymphoma in three, and single cases of immunoblastic lymphoma and indolent T-cell lymphoma. Lymphoma was located within the IBD-affected area in ten patients. At lymphoma diagnosis, nine patients had a history of azathioprine or anti-tumour necrosis factor [TNF] use. Lymphoma was diagnosed at a mean time of 10.4 [±7.07, 1–24] years after IBD diagnosis in 11 patients, prior to IBD in two and concurrently in two. Sustained remission over a median follow-up time of 6.5 [1.5–20] years was achieved in ten patients after treatment; five of them had started biological therapy [including anti-TNFs, vedolizumab and ustekinumab] for active CD subsequent to their PIL treatment. Conclusion In this small case series, two-thirds of patients developed lymphoma in the IBD-affected area, and almost two-thirds had a history of thiopurine or anti-TNF use. Biologics were restarted without recurrence of lymphoma in half of the remitters. [ABSTRACT FROM AUTHOR]

Background and Aims Crohn's disease [CD] can involve any part of the gastrointestinal tract. We aimed to characterize the clinical, endoscopic and histological features and treatment outcomes of CD patients with oesophageal involvement. Methods We collected cases through a retrospective multicentre European Crohn's and Colitis Organisation CONFER [COllaborative Network For Exceptionally Rare case reports] project. Clinical data were recorded in a standardized case report form. Results A total of 40 patients were reported (22 males, mean [±SD, range] age at oesophageal CD diagnosis: 25 [±13.3, 10–71] years and mean time of follow-up: 67 [±68.1, 3–240] months). Oesophageal involvement was established at CD diagnosis in 26 patients [65%] and during follow-up in 14. CD was exclusively located in the oesophagus in two patients. Thirteen patients [32.2%] were asymptomatic at oesophageal disease diagnosis. Oesophageal strictures were present in five patients and fistulizing oesophageal disease in one. Eight patients exhibited granulomas on biopsies. Proton-pump inhibitors [PPIs] were administered in 37 patients [92.5%]. Three patients underwent endoscopic dilatation for symptomatic strictures but none underwent oesophageal-related surgery. Diagnosis in pre-established CD resulted in treatment modifications in 9/14 patients. Clinical remission of oesophageal disease was seen in 33/40 patients [82.5%] after a mean time of 7 [±5.6, 1–18] months. Follow-up endoscopy was performed in 29/40 patients and 26/29 [89.7%] achieved mucosal healing. Conclusion In this case series the endoscopic and histological characteristics of isolated oesophageal CD were similar to those reported in other sites of involvement. Treatment was primarily conservative, with PPIs administered in the majority of patients and modifications in pre-existing inflammatory bowel disease-related therapy occurring in two-thirds of them. Clinical and endoscopic remission was achieved in more than 80% of the patients. [ABSTRACT FROM AUTHOR]

Background Genital granulomatosis [GG] is a metastatic form of Crohn's disease [CD], characterised by granulomatous inflammation of the genital skin without contact with the gastrointestinal tract. Little is known about GG, as most publications are case reports or small series, and only sporadic in male cases. Methods and Aims Cases of GG were retrospectively collected through the Collaborative Network For Exceptionally Rare case reports project of the European Crohn′s and Colitis Organisation. Results A total of 43 patients [9 males, 34 females] were diagnosed as having GG, mostly as oedema and/or ulcers. Histological confirmation of granulomas was obtained in 70% of the cases. CD location was colonic or ileocolonic in 97% and perianal disease was documented in 57%. There was no significant difference between males and females in CD phenotype or genital lesions. GG was the first manifestation of inflammatory bowel disease [IBD] in one-third of the patients; these patients were younger at the time of GG occurrence and they all were non-smokers. GG occurred in the absence of gastrointestinal disease activity in 30% of the cases. Ten out of 11 patients [91%] responded to systemic corticosteroid treatment, 5/9 patients responded to immunomodulators, and 9/11 patients responded to anti-tumour necrosis factor alpha [TNF-α] agents. Conclusions GG is a rare extraintestinal manifestation of CD. It mainly occurs among women, in the setting of colonic involvement of CD, and perianal disease is often associated. Most cases are successfully managed with systemic corticosteroids or anti-TNF agents. [ABSTRACT FROM AUTHOR]