Search

Competing Interests: Conflicts of interest The GDG adhered to BAD’s policy for declaration of interests for guideline authors. Further details may be requested by contacting guidelines@bad.org.uk. The following interests were declared over the duration of the guideline development: RM is member of an advisory board for Janssen (nonspecific). RJM is (i) a member of advisory boards for AbbVie, Chugai, Novartis, Pfizer and Roche (specific); (ii) a speaker at international meetings funded by Chugai/Roche, Eli Lilly and Pfizer (specific); (iii) a director for the National Centre for Behçets Syndrome (specific); (iv) a chief investigator for the BioBehçet’s trial (NIHR EME grant) (specific); and (v) a grant holder and chief investigator for the phase II trial ‘Secukinumab in Behçet’s’, with funding provided to Liverpool University Hospitals NHS Trust (specific). PB (i) receives consultancy or lecturing fees from Sobi, Novartis and Roche (nonspecific); (ii) is a local principal investigator for the EMERALD trial (Sobi) (nonspecific); (iii) is chief investigator of the KDCAAP trial (nonspecific); and (iv) is a trustee of a patient-led charity for Kawasaki disease (nonspecific). AFÇ (i) participates on advisory boards and talks at meetings for irritable bowel disease funded by AbbVie, MSD, UCB Pharma, Takeda, Pfizer, Centurion, Janssen and Mgen (specific); (ii) is head of the Turkish IBD Association, which receives annual funding from MSD, AbbVie, Takeda, UCB Pharma, Centurion, Mgen, Pfizer and Janssen (specific); and (iii) receives funding to their clinic from MSD, AbbVie, Takeda, UCB Pharma, Centurion, Mgen and Janssen, which provides medical equipment used for Behçets (specific). SSK (i) is chairperson for Janssen (nonspecific); (ii) received speaker fees from LEO Pharma, Janssen and L’Oreal (nonspecific); and (iii) has a salary partly funded by a grant awarded by the Women’s Dermatology Society and GLODERM (The International Alliance of Global Health Dermatology) (nonspecific). CEP is the UK’s chief investigator for the apremilast clinical trial in paediatric Behçets funded by Amgen (specific). JS (i) is a shareholder with Welbeck Health Partners in OneWelbeck Skin and Allergy Centre (nonspecific) and (ii) is a subinvestigator for a multicentre open-label phase I study to evaluate the safety, tolerability, pharmacokinetics and early signs of effectiveness of induction of antigen-specific immune tolerance with TPM203 in pemphigus vulgaris (nonspecific). PS (i) is a member of an advisory board for Janssen regarding guselkumab in psoriatic arthritis (nonspecific); (ii) is a member of an advisory board for UCB regarding bimekizumab in psoriatic arthritis (nonspecific); (iii) is a member of an advisory board for Galapagos (nonspecific); (iv) received sponsorship from Amgen for an osteoporosis conference (nonspecific); and (v) received an honorarium from SpA academy (nonspecific). RW is Vice Chair of Behçets UK (specific). MC-J, IC, APC, SC, LF, JH, SH, ASMJ, SK, HM, HP, AP-G, CW, MH, LM, MFMM and AMC have no conflicts of interest to declare.

Background: Coronavirus disease 2019 pandemic was expected to have traumatic effects and increase the anxiety levels of inflamma- tory bowel disease patients., Methods: We aimed to investigate the psychosocial effects of the coronavirus disease 2019 pandemic on patients with inflammatory bowel disease by revealing the risk perception for present disease, coping strategies, follow-up characteristics, and treatment adher- ence. This is a cross-sectional, web-based survey study including 798 inflammatory bowel disease patients who were followed at our outpatient clinic and 303 volunteer who did not have any known chronic diseases and were not health professionals were included as the control group., Results: In this study, 281 of the patients were diagnosed with Crohn's disease and 215 with ulcerative colitis. The mean age of patients with Crohn's disease, ulcerative colitis, and the control group were 40.9 ± 13.1, 42.3 ± 12.7, and 39.9 ± 11.6, respectively. Here, 119 (42%) of the Crohn's disease cases, 116 (54%) of the ulcerative colitis cases, and 170 (56%) of the control group were male. Among the 3 groups, coronavirus disease 2019-related post-traumatic stress disorder rates (Impact of Event Scale-Revised > 33) and State-Trait Anxiety Inventory of current status of anxiety-related anxiety rates were not statistically different while State-Trait Anxiety Inventory of anxiety tendency-related constant anxiety was higher in inflammatory bowel disease patients than the control group (P < .017)., Conclusion: Inflammatory bowel disease patients with anxiety have a lower quality of life, and this may worsen the clinical course of the disease. Coronavirus disease 2019 is a major source of stress for such a vulnerable population. During the pandemic, psychological support and mental health awareness should be made accessible to all individuals.

Introduction: Trastuzumab emtansine (TDM-1) is an antibody-drug conjugate effective in human epidermal growth factor receptor-2 - expressing advanced breast cancer. Pulmonary complications of TDM-1 are rarely reported. TDM-1-associated interstitial lung disease is referred to as pneumonitis., Case Report: A 47-year-old female patient who underwent modified radical mastectomy and axillary lymph node dissection operations due to a palpable mass in the right breast and axillary region. The patient who had received multiple chemotherapy was last receiving TDM-1 treatment. Fatigue, dyspnea, and tachypnea were detected for the first time on 20 days after the 6 th treatment., Menagement and Outcome: In our case, we first considered metastasis, pneumonia and fungal infection based on radiological findings, but the lack of response to the treatments and the results of the investigations suggested drug-induced pneumonia and steroid treatment was started. Our case had a complete radiological recovery and complete response to sterod therapy. In such cases, it is important to first exclude infections and metastasis. In cases of drug-induced pneumonia, the first treatment option is systemic corticosteroids and generally responded well., Discussion: Unlike other cases of interstitial pneumonia, lung imaging of our case was resembling a metastasis, pneumonia and fungal infection. With increasing use of TDM-1, we will have more experience in both efficacy and complications of TDM-1. Although TDM-1 is a well-tolerated drug, clinicians should be aware of rare pulmonary complications and prepared to respond appropriately.

The COVID-19 pandemic, caused by the novel severe acute respiratory syndrome coronavirus 2, has resulted in high mortality and morbidity worldwide and is still a growing problem. Inflammatory bowel disease (IBD) is a chronic inflammatory disease for which a substantial number of patients are treated with immunosuppressive medications, either occasionally or long-term. Despite the accumulating evidence, there is still a lack of knowledge about the impact of COVID-19 on IBD patients, especially those who are under immunosuppressive treatment. Moreover, following the emergence of several COVID vaccines, there are concerns regarding vaccine effectiveness and possible side effects in such patients. In this context, we tried to briefly summarize the accumulating evidence and recommendations for the management of IBD in the context of the COVID-19 pandemic.

Background/aims: This study aimed to determine the predictors of endoscopic recurrence in a cohort of patients with Crohn's disease (CD) with prior intestinal resections., Materials and Methods: The charts of the patients with CD were reviewed in a retrospective manner. Eighty-three patients were eligible for the final analysis. Demographic features of these patients and time between resection and colonoscopy, presence of any macroscopic residual disease in the remnant intestine, and postoperative medications were noted. Rutgeerts score was used to define postoperative endoscopic recurrence., Results: The patients' mean age±SD at their final colonoscopy was 42.81±11.99 yr; and 37 of 83 patients (45%) were female. The mean follow-up time between resection and the final colonoscopy was 51.16±51.08 months. A total of 51 of 83 patients (61%) were in endoscopic remission (i0, i1); whereas 32 (39%) had an endoscopic recurrence (i2, i3, i4). History of multiple resections (χ2=6.12; p=0.013) and the presence of any postoperative residual disease in the remnant intestine (χ2=5.86; p=0.015) were risk factors; whereas the regular use of azathioprine (AZA) was significantly more common among patients without recurrence (χ2=4.515; p=0.034). In an age-sex adjusted Cox regression analysis history of multiple resections, presence of any postoperative residual disease proved to be independent risk factor for endoscopic recurrence, whereas the regular use of AZA proved to be ineffective., Conclusion: In a retrospective long-term follow-up cohort of resected patients with CD, having multiple resections for CD and the presence of any residual synchronous disease after ileocolonic resection were identified as risk factors for endoscopic recurrence; the latter was never reported in previous studies.

Background/aims: The aim of the present study was to compare the demographic features and long-term outcomes of patients with inflammatory bowel disease (IBD) with or without ankylosing spondylitis (AS)., Materials and Methods: Among 1640 IBD (Crohn's disease and ulcerative colitis), 76 patients with IBD+AS were identified. The study group consisted of 76 patients with IBD with synchronous AS. The control group consisted of patients with only IBD, and those were selected according to their registry sequence number being the previous and next case to the diseased case with IBD+AS. The primary endpoint was to compare the rate of intestinal resections between both groups (IBD vs. IBD+AS)., Results: Among 76 patients with IBD+AS, 52 (68%) first presented with IBD, 11 (15%) with AS, and the remaining 13 (17%) had both diagnoses at the same time. The mean follow-up time was significantly longer in patients with IBD+AS (43.4 vs. 27.8 months; p=0.01). Twenty-two percent of patients with IBD and 14% of those with IBD+AS had an intestinal resection (p=NS). Biologic and systemic corticosteroid treatments were significantly more common among patients with IBD+AS (32% vs. 7% for biologics, p<0.0001 and 44% vs. 28% for corticosteroids, p=0.042). Age-sex-adjusted regression analysis for both groups disclosed IBD duration as the only independent predictor for resection (R2=0.178; p=0.016)., Conclusion: The present study shows that up to 5% of patients with IBD may have AS. Patients with IBD+AS do not have a worse disease outcome than solo patients with IBD.

Objective: To assess the efficacy and safety of treatment modalities for major organ involvement of Behçet's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS., Methods: A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included., Results: We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A., Conclusion: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.

Objectives: Crohn's disease is a chronic inflammatory bowel disease that requires lifelong multidisciplinary management. Seventy percent of patients affected by Crohn's disease will require at least one surgical procedure over their lifetime. The aim of this retrospective study was to present our series of patients suffering from Crohn's disease who were scheduled for surgery by a multidisciplinary team., Material and Methods: The data were retrieved from a review of 950 patients with Crohn's disease treated at our institution between March 2000 and March 2016. Only patients with intestinal Crohn's disease were included into the study. A multidisciplinary team assessed the decision to perform surgery., Results: There were 203 patients who underwent surgery included in this study. One hundred and sixty-six were intestinal and 37 were perianal Crohn's disease. The mean age was 36±11.5 (range, 12-75) years. Ninety-two were stricturing, 45 were fistulizing, and 12 were inflammatory. The most commonly affected site was the ileocecal region (n=109, 65.7%), and the most common surgical procedure was the ileocecal resection (n=109, 65.6%). Laparoscopic approach was the procedure of choice in 56 (33.7%) patients. Of the patients enrolled, the most common early (<30 days) complications observed were the wound infection as the first (n=11) and anastomotic leak as the second (n=10). The mortality rate was 2.4% (n=4)., Conclusion: Multidisciplinary approach to Crohn's disease may decrease the surgical complications and recurrence rates leading to a better treatment.

Behçet disease (BD) is a variable vessel vasculitis that can involve several organs and systems. Gastrointestinal (GI) involvement has an acute exacerbating course with ulcers, most commonly in the ileocolonic area. These ulcers can be large and deep, causing perforation and massive bleeding. This article highlights the current knowledge on the epidemiology, clinical findings, diagnosis, and management of GI involvement of BD, with emphasis on recent findings., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose of Review: Gastrointestinal system can be involved in primary and secondary vasculitides. The recent data regarding the pathophysiology, clinical findings, diagnosis, management, and outcome of gastrointestinal involvement in different types of vasculitis are reviewed., Recent Findings: Diagnosis of gastrointestinal vasculitis may be difficult and relies mostly on imaging, because biopsy samples are hard to obtain and superficial mucosal biopsies have a low yield. There are conflicting reports on the association of antineutrophilic cytoplasmic antibodies (ANCA) type with the frequency of gastrointestinal involvement in ANCA-associated vasculitis. Pancreatitis is a rare but serious complication of ANCA-associated vasculitis. Terminal ileitis may be observed in immunoglobulin A vasculitis and can be hard to distinguish from Crohn's disease. High fecal calprotectin levels can indicate active gastrointestinal involvement in both immunoglobulin A vasculitis and Behçet's syndrome. Refractory gastrointestinal involvement in Behçet's syndrome can be treated with thalidomide and/or TNF-α antagonists. The outcome of mesenteric vasculitis in systemic lupus erythematosus can be improved with high-dose glucocorticoids and cyclophosphamide or rituximab., Summary: Gastrointestinal system can be commonly involved in immunoglobulin A vasculitis, ANCA-associated vasculitis, polyarteritis nodosa, and Behçet's syndrome and can be an important cause of morbidity and mortality. Treatment depends on the type of vasculitis and is usually with high-dose corticosteroids and immunosuppressives.

Objectives: Behçet's disease (BD) can be life threatening and may be refractory to corticosteroids and immunosuppressives. There has been some experience with haematopoietic stem cell transplantation (HSCT) in BD either for severe, refractory disease or for a haematological condition. The objectives of this study were to describe a BD patient undergoing HSCT and to evaluate the outcomes of BD patients who underwent HSCT., Methods: We report a BD patient with refractory gastrointestinal (GI) involvement who had HSCT for concomitant myelodysplastic syndrome (MDS). We also performed a systematic literature search regarding HSCT for either refractory disease or concomitant haematological conditions in BD patients., Results: A 30-year-old woman with refractory GI BD involvement with trisomy 8 MDS underwent a successful myeloablative allogeneic HSCT resulting in complete resolution of both BD and MDS. Additionally we identified 14 manuscripts providing data on 19 patients with BD who had HSCT. Among these 20 patients, including ours, refractory disease was the indication of transplantation in 9, while 11 patients were transplanted because of accompanying haematological conditions. Transplant indications for the nine patients (four male, five female) with refractory BD were neurological involvement in five, pulmonary artery aneurysm in two, GI disease in one and not reported in one patient. Three patients with neurological disease, both patients with pulmonary artery aneurysm and the patient with intestinal involvement achieved complete remission of their disease. Six patients transplanted for haematological conditions, including the presented case, also had GI involvement of BD. All of these patients achieved complete remission of GI findings after HSCT., Conclusion: When considering HSCT, the potential adverse events and complications, which can be fatal, need to be kept in mind., (© The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)