Homozygous mutations in the 15-hydroxyprostaglandin dehydrogenase gene in patients with primary hypertrophic osteoarthropathy

Mutations in HPGD have recently been reported to cause primary hypertrophic osteoarthropathy (PHO), a rare genetic disease characterized by digital clubbing, pachydermia, and periostosis. We screened HPGD mutations in six patients from three unrelated Turkish families with PHO, in which we showed one previously reported, p.A140P, and one novel, p.M1L, homozygous mutations. Both mutations co-segregated with the phenotype in all three families and were absent in 100 Turkish controls. These results confirm the presence of biallelic HPGD mutations in patients with PHO in an independent series from a different population.     

The beneficial effect of azathioprine on maintenance of remission in severe ulcerative colitis

Background. The search is on to find more effective drug regimens for patients with severe ulcerative colitis, as conventional drugs such as sulfasalazine and its congeners fail to prevent relapse in a significant number of patients. Azathioprine has also been reported to be useful as a steroid-sparing drug in patients who suffer from frequent relapses. As these drugs when used individually fail to sustain remission in a significant number of patients, we evaluated the combination of these two drugs. Methods. Thirty-five newly diagnosed patients with severe ulcerative colitis were randomized into two groups; group A (combination therapy) received sulfasalazine and azathioprine, while group B (sulfasalazine monotherapy) received sulfasalazine and placebo. In addition, all the patients received steroids initially to achieve clinical remission. The patients were followed-up for a period of 1 year. The therapeutic outcome was measured by the number of patients who suffered relapse in each group. Results. All the patients completed the 1-year study period. While 4 patients (23.5%) in group A suffered relapse of disease, 10 (55.6%) in group B suffered relapse, the difference being statistically significant. The relapse-free period was also significantly longer in group A. Conclusions. Combination therapy (sulfasalazine and azathioprine) is more effective than sulfasalazine and placebo in the maintenance of remission in patients with severe ulcerative colitis. Received: November 20, 2000 / Accepted: September 14, 2001     

Evaluation of liver fibrosis by transient elastography (Fibroscan®) in patients with inflammatory bowel disease treated with methotrexate: a multicentric trial

Abstract

Background. Methotrexate is an effective treatment for inflammatory bowel disease (IBD). However, long-term treatments have been associated with the development of liver fibrosis. FibroScan® is a noninvasive, safe, and effective technique to evaluate liver fibrosis. Aim. To evaluate the presence of significant liver fibrosis by transient elastography (FibroScan®) in IBD patients treated with methotrexate. Methods. Cross-sectional study including IBD patients treated with methotrexate from different hospitals. Clinical and analytical data, duration of treatment, and cumulative dose of methotrexate were obtained. Liver stiffness was assessed by FibroScan®. The cutoff value for significant liver fibrosis (according to METAVIR) was F ≥2: 7.1 kPa. Results. In the study, 46 patients were included, 30 women (65%), with a mean age of 43 ± 10 years. 31 patients had Crohn's disease (67.4%), 13 ulcerative colitis (28.3%), and 2 indeterminate colitis (4.3%). The mean cumulative dose of methotrexate was 1242 ± 1349 mg, with a mean treatment duration of 21 ± 24 months. The mean value of liver stiffness was 4.7 ± 6.9 kPa. There were 35 patients (76.1%) with F01, 8 patients (17.4%) with F = 2, and 3 patients with F ≥3 (6.5%). There were no differences in liver stiffness depending on sex, age, type of IBD, or cumulative dose of methotrexate. Conclusions. (1) Development of advanced liver fibrosis in IBD patients treated with methotrexate is exceptional. (2) There were no differences in liver stiffness depending on the type of IBD or the cumulative dose of methotrexate. (3) FibroScan® may be potentially useful for evaluation and follow-up of liver fibrosis in methotrexate-treated patients.     

Prognostic value of p53 mutations in patients with locally advanced esophageal carcinoma treated with definitive chemoradiotherapy

Purpose. A significant correlation has been found between p53 mutation and response to chemotherapy or radiotherapy. To determine the prognostic value of p53 mutation in patients with locally advanced esophageal carcinoma treated with definitive chemoradiotherapy, p53 mutation was analyzed using the biopsied specimens taken for diagnosis. Methods. Concurrent chemoradiotherapy was performed for 40 patients with severe dysphagia caused by esophageal squamous cell carcinoma associated with T3 or T4 disease. Chemotherapy consisted of protracted infusion of 5-fluorouracil, combined with an infusion of cisplatinum. Radiation treatment of the mediastinum was administered concomitantly with chemotherapy. The p53 gene mutation was detected by fluorescence-based polymerase chain reaction single-strand conformation polymorphism (PCR-SSCP) methods. DNA sequences were determined for DNA fragments with shifted peaks by SSCP methods. Results. Of the 40 patients, 15 had T3 disease and 25 had T4 disease; 11 patients had M1 lymph node (LYM) disease. Of the 40 patients, 13 (33%) achieved a complete response. The median survival time was 14 months, and the 2-year survival rate was 20%. Among the 40 tumor samples, p53 mutation was detected in 24 tumors (60%). The survival rate in the 24 patients with p53 mutation did not differ significantly from that in the 16 patients without p53 mutation. In contrast, the 15 patients with T3 disease survived longer than the 25 patients with T4 disease (P = 0.016); however, the survival rate in the 11 patients with M1 LYM disease did not differ significantly from that in the 29 patients without M1 LYM disease. Conclusion. Concurrent chemoradiotherapy is potentially curative for locally advanced esophageal carcinoma, but p53 genetic abnormality has no impact on prognosis. Received: December 6, 2000 / Accepted: January 26, 2001     

Infecciones en enfermedades autoinmunes sistémicas

IntroductionInfections are a major cause of morbidity and mortality in patients with systemic autoimmune diseases. The aim of the present study is to describe the frequency of infections in a historical cohort of the SAD polyclinic of the Maciel Hospital, according to the type of disease and treatment received.Material and methodsAn analytical, retrospective and observational study was conducted in 339 patients with SAD attended at the outpatient clinic in the period from January 1, 2012 to February 28, 2019. Infectious complications were analysed according to treatment and disease.Results339 cases, median age 56, mostly female. Most cases presented SLE (30.1%) and RA (23.6%), followed by antiphospholipid syndrome (20.4%) and Sjögren's syndrome (12.1%). Hydroxychloroquine (66%), followed by corticosteroids (55.5%) were the most frequently used treatments. Thirteen point three percent received biological therapies: 46.9% of the cases presented some infectious complication, 95% were non-opportunistic. Respiratory infections were the most frequent (48.6%) followed by urinary infections (31.7%) and skin and soft tissue infections (17.6%). On comparing the infected and non-infected groups, significant differences were found in the following variables: methotrexate, mycophenolate, corticoids, biological therapies, combination of drugs, active disease, RA and cases with overlap. The use of hydroxychloroquine and sulfasalazine was associated with a lower risk of infection in patients with RA.ConclusionsInfections are a frequent complication in patients with RA, due to the immune disturbances of the disease itself and prescribed treatments, mainly corticoids and biologicals. The importance of screening and infection prophylaxis before starting treatment is stressed.     

Emergency anticoagulation treatment for cirrhosis patients with portal vein thrombosis and acute variceal bleeding

Abstract

Objective. To determine the safety and efficacy of anticoagulation treatment for portal vein thrombosis in cirrhosis patients with acute variceal bleeding, with patient eligibility determined by contrast ultrasonography findings. Materials and methods. This prospective study included 23 consecutive cirrhosis patients (63.8 ± 11.8 years old, 12 males and 11 females) with emergency admission for acute variceal bleeding with or without portal vein thrombus. Eligibility for anticoagulation treatment was determined by positive intra-thrombus enhancement on contrast ultrasonography (perflubutane microbubble agent, 0.0075 mL/kg) performed before endoscopy. Low-molecular-weight heparin was administered after hemostasis was achieved by band ligation. Repeated band ligation or injection sclerotherapy combined with argon plasma coagulation was performed for variceal disappearance. Results. Hemostasis was achieved in all 10 patients with active bleeding. Five of these patients had portal vein thrombus, and all showed positive intra-thrombus enhancement on contrast ultrasonography. Anticoagulation treatment of these five patients resulted in complete recanalization of the portal vein within 2–11 days. There were no significant differences in the number of endoscopic treatment sessions or the length of hospital stay between the groups with and without thrombosis, and no complications including rebleeding were reported. Long term, none of the patients who continued oral anticoagulation treatment had recurrence of thrombosis (4/5). Variceal recurrence occurred only in the non-thrombosis group (2/18) during the follow-up period (median: 351 days). Conclusions. Early anticoagulation treatment in cirrhosis patients with portal vein thrombosis and acute variceal bleeding may be safe, tolerated, and effective in cases with positive intra-thrombus enhancement on contrast ultrasonography.     

Molecular analyses of the HGO gene mutations in Turkish alkaptonuria patients suggest that the R58fs mutation originated from central Asia and was spread throughout Europe and Anatolia by human migrations

Summary: Alkaptonuria (AKU) is a rare metabolic disorder of phenylalanine catabolism that is inherited as an autosomal recessive trait. AKU is caused by loss-of-function mutations in the homogentisate 1,2-dioxygenase (HGO) gene. The deficiency of homogentisate 1,2-dioxygenase activity causes homogentisic aciduria, ochronosis and arthritis. We present the first molecular study of the HGO gene in Turkish AKU patients. Seven unrelated AKU families from different regions in Turkey were analysed. Patients in three families were homozygous for the R58fs mutation; another three families were homozygous for the R225H mutation; and one family was homozygous for the G270R mutation. Analysis of nine intragenic HGO polymorphisms showed that the R58fs, R225H and G270R Turkish AKU mutations are associated with specific HGO haplotypes. The comparison with previously reported haplotypes associated with these mutations from other populations revealed that the R225H is a recurrent mutation in Turkey, whereas G270R most likely has a Slovak origin. Most interestingly, these analyses showed that the Turkish R58fs mutation shares an HGO haplotype with the R58fs mutation found in Finland, Slovakia and India, suggesting that R58fs is an old AKU mutation that probably originated in central Asia and spread throughout Europe and Anatolia during human migrations.     

The Frequency of Monocyte Chemoattractant Protein-1 Gene Polymorphism in Obstructive Sleep Apnea Syndrome

Purpose

In obstructive sleep apnea syndrome (OSAS) many proinflammatory cytokines are released from activated endothelial cells due to repeated decreases in arterial oxygen saturation. Some of these proinflammatory cytokines are involved in the etiology of coronary artery disease (CAD). Although the association between OSAS and CAD is known, risk factors for CAD have not been determined in this patient group. Monocyte chemoattractant protein-1 (MCP-1) is a proinflammatory cytokine that plays a key role in the development of atherosclerosis. In this study, we compared the frequency of MCP1 rs1024610-rs1024611 single-nucleotide polymorphisms (SNPs) in OSAS patients with no comorbidity, OSAS patients with no comorbidity except CAD, and healthy individuals.

Material and Methods

The study included 301 subjects. Two hundred one patients with OSAS (OSAS only and OSAS + CAD groups) and 100 healthy control subjects underwent polysomnography. MCP1 rs1024610 and rs1024611 mutation frequencies were determined.

Results

Body mass index, apnea–hypopnea index, triglyceride levels, and mean oxygen desaturation were significantly higher in the OSAS patients than in the healthy population (p < 0.05). In MCP1 rs1024611 SNP analysis, homozygous mutation was significantly more common in the OSAS + CAD group than in the OSAS and control groups (p < 0.001). MCP1 rs1024610 SNP analysis showed no significant differences among the study groups.

Conclusion

OSAS patients with homozygous MCP1 rs1024611 SNP are at higher risk for CAD. The MCP1 rs1024610 SNP was not associated with incidence of CAD. Patients with OSAS and MCP1 rs1024611 homozygous mutation are more susceptible to CAD and early detection and treatment may significantly reduce mortality and morbidity.

     

Guía de práctica clínica 2021 para el diagnóstico,el tratamiento y el seguimiento de pacientes con espondiloartritis periférica. Asociación Colombiana de Reumatología

BackgroundPeripheral spondyloarthritis is a chronic inflammatory disease whose clinical presentation is related to the presence of arthritis, enthesitis and/or dactylitis. This term is used interchangeably with some of its subtypes such as psoriatic arthritis, reactive arthritis, and undifferentiated spondyloarthritis.ObjectiveTo develop and formulate a set of specific recommendations based on the best available evidence for the diagnosis, treatment, and monitoring of adult patients with peripheral spondyloarthritis.MethodsA working group was established, clinical questions were formulated, outcomes were graded, and a systematic search for evidence was conducted. The guideline panel was multidisciplinary (including patient representatives) and balanced. Following the formal expert consensus method, the GRADE methodology “Grading of Recommendations Assessment, Development and Evaluation” was used to assess the quality of the evidence and generate the recommendations. The clinical practice guideline includes ten recommendations related to monitoring of disease activity (n=1) and treatment (n=9).ResultsIn patients with peripheral spondyloarthritis, the use of methotrexate or sulfasalazine as the first line of treatment is suggested, and local injections of glucocorticoids are conditionally recommended. In patients with failure to cDMARDs, an anti TNFα or an anti IL17A is recommended. In case of failure to bDMARDs, it is suggested to use another bDMARD or JAK inhibitor. In patients with peripheral spondyloarthritis associated with inflammatory bowel disease, it is recommended to start treatment with cDMARDs; in the absence of response, the use of an anti TNFα over an anti-IL-17 or an anti-IL-12-23 is recommended as a second line of treatment. In patients with psoriatic arthritis, the combined use of methotrexate with a bDMARD is conditionally recommended for optimization of dosing. To assess disease activity in Psoriatic Arthritis, the use of DAPSA or MDA is suggested for patient monitoring.ConclusionsThis set of recommendations provides an updated guideline on the diagnosis and treatment of peripheral spondyloarthritis.     

Differences in colonic disease activity in patients with ulcerative colitis with and without primary sclerosing cholangitis

PURPOSE: A small group of patients with ulcerative colitis (UC) also suffer from primary sclerosing cholangitis (PSC). Genetic and immunologic differences exist between UC patients with and without concomitant PSC. Furthermore, UC patients with PSC are more prone to developing colonic dysplasia/aneuploidy compared with patients with UC only. Because colonic disease activity and treatment with sulfasalazine have been found to be of independent importance for development of colonic carcinoma in UC, this study aims to determine if differences exist concerning colonic disease activity in UC patients with and without PSC. METHODS: Twenty-nine PSC patients with total colitis were matched to two UC patients with total colitis but without liver disease. Case records and questionnaires were used to gain information on pharmacologic treatment and disease activity. RESULTS: Observation time was 20 (PSC group) and 23 years (UC only). Number of patients taking prophylactic treatment did not differ between groups. Patients with UC only had received treatment with systemic and local corticosteroids significantly more often than UC patients with PSC (P < 0.05 and P < 0.02). Patients with UC only were hospitalized because of colonic activity significantly more often (P < 0.02). Number of patients undergoing colectomy because of disease activity or number of patients with chronic continuous symptoms did not differ between the two groups. CONCLUSION: UC in patients with PSC runs a milder course than UC in patients without this complication, although the number of patients taking prophylactic treatment was the same. If lower disease activity reflects differences in pathogenesis of UC in patients with PSC or if it can explain increased risk to develop colonic malignancy in patients with both PSC and UC needs further elucidation.Supported by grants from Nanna Svartz Scholarship, Stockholm, Sweden.     

Interleukin-1 targeting treatment in familial Mediterranean fever: an experience of pediatric patients

Abstract

Objectives. The aim of this report was to evaluate and discuss treatment of pediatric familial Mediterranean fever (FMF) patients with anti-interleukin1 (IL-1) agents.

Methods. Refractory or colchicine unresponsive FMF was described as severe and frequent attacks and/or having high acute phase reactance levels despite having a maximum dose of colchicine (2 mg/day). Disease course, adverse effects, duration of follow-up, treatment protocols, responses to the therapies were discussed.

Results. Eight patients (6 male, 2 female) having refractory FMF were identified. Mediterranean fever (MEFV) gene analyses revealed homozygous M694V mutations in six patients and heterozygote M694V mutations in one patient and no mutation in one patient. They were all treated with anakinra and/or canakinumab. The use of anti-IL-1 drugs was beneficial to all patients. None of them had any severe adverse effects due to the therapy.

Conclusions. Anakinra and canakinumab were effective in patient refractory to colchicine treatment as shown both in our series and in the literature. Therefore, controlled trials are needed to evaluate the safety and long-term efficacy of IL-1 targeting agents in colchicine resistant patients.     

Early response to certolizumab pegol predicts long-term outcomes in patients with active rheumatoid arthritis: results from the Japanese studies

Abstract

Objectives. A post-hoc analysis was performed to determine the relationship between the timing and magnitude of DAS28(ESR) response and long term outcomes in Japanese patients after 1 year of CZP treatment.

Methods. Our analysis included 82 J-RAPID trial patients treated with CZP 200 mg and methotrexate, and 116 HIKARI trial patients treated with CZP 200 mg alone or with disease-modifying agents other than methotrexate. Remission rates and changes in mTSS at year 1 were compared to the DAS28(ESR) response at week 12 of CZP treatment.

Results. After 1 year of treatment, remission was achieved in 41.3% of the J-RAPID and 34.9% of the HIKARI patients with a week 12 DAS28(ESR) response of ≥ 1.2. In comparison, patients with a DAS28(ESR) response of < 1.2 at week 12 only had a < 7% probability of achieving remission and displayed higher change in mTSS after 1-year treatment.

Conclusions. The likelihood of remission and extent of radiographic progression after 1 year was associated with the week 12 DAS28(ESR) response. The DAS28(ESR) response at 12 weeks could be beneficial for identifying patients that are unlikely to respond to prolonged CZP treatment.     

Current prevalence and characteristics of cervical spine instability in patients with rheumatoid arthritis in the era of biologics

Abstract

Objectives. Cervical spine instability (CSI) is commonly involved in patients with rheumatoid arthritis (RA). Although the treatment for RA has dramatically changed due to methotrexate and biologics, it is unclear whether this change contributes to the prevalence of CSI or not. Our objectives were to update the current prevalence of CSI and to investigate the factors associated with CSI.

Methods. A cross-sectional study of patients with RA was conducted in our outpatient clinic. Clinical information and symptoms related to CSI were obtained. Plain radiography and magnetic resonance imaging were performed. CSI included atlantoaxial subluxation (AAS), vertical subluxation (VS), and subaxial subluxation (SAS).

Results. Two hundred and twenty patients were analyzed, 93 (42%) of whom had CSI. A ≥ 10-year disease duration, Steinbrocker stage III, and three or more narrowed disc spaces from C2/3 to C6/7 were significantly associated with CSI. A neck pain VAS was associated with VS, but not with AAS and SAS. In contrast, methotrexate and biologics had no effect on CSI.

Conclusion. The prevalence of CSI in this study was lower compared to previous reports before the approval of biologic, although we failed to detect the effect of biologics. Further prospective studies are needed to elucidate the efficacy.     

Trial of intravenous pulse cyclophosphamide and methylprednisolone in the treatment of severe systemic-onset juvenile rheumatoid arthritis

Objective. Not uncommonly, some children with systemic-onset juvenile rheumatoid arthritis (JRA) have persistently active disease with joint destruction and profound growth delay despite maximum treatment with known medications. Based on previous observations of improvement in synovitis following intravenous (IV) cyclophosphamide (CYC) and methylprednisolone (MP) treatments, a group of children with severe systemic-onset JRA was treated in an attempt to control active synovitis and to allow tapering of corticosteroids. Methods. Four patients with systemic-onset JRA were continued on a daily regimen of nonsteroidal antiinflammatory agents and prednisone, with a weekly subcutaneous dose of methotrexate (1 mg/kg). In addition, 1 patient continued receiving sulfasalazine and 1 patient remained on a regimen of sulfasalazine and hydroxychloroquine. Patients received 6–10 monthly treatments of IV CYC (500–1,000 mg/m²) and MP (30 mg/kg; 1 gm maximum) accompanied by IV mesna and large amounts of IV fluids. Subsequent treatments were given once every 2–3 months. Results. After 12–20 IV pulses of CYC, all patients showed improvement, and 3 achieved remission of disease. All were able to discontinue corticosteroid use and all had an increase in linear growth. Conclusion. Monthly IV pulse CYC treatments can be useful to control disease in selected children with severe, destructive JRA.     

Efficacy of golimumab for preventing large joint destruction in patients with rheumatoid arthritis as determined by the ARASHI score

Objectives: The objective of this study is to investigate the inhibitory effect of golimumab on large joint destruction in patients with rheumatoid arthritis.

Methods: We recruited 45 patients with rheumatoid arthritis and evaluated the radiographic severity of large joint destruction using the assessment of rheumatoid arthritis by scoring of large joint destruction and healing in radiographic imaging (ARASHI) score. We evaluated 450 large joints including the elbow, shoulder, hip, knee, and ankle at baseline and 52 weeks after treatment with golimumab. Rapid radiographic progression (RRP) and rapid radiographic improvement (RRI) were calculated and the correlation between large joint destruction and clinical factors was analyzed.

Results: The mean age of the study population was 61.29?±?14.71 years old, and most patients (91.1%) were female. The mean disease duration was 12.6?±?12.48 years. The cohort included patients in all clinical stages of disease as defined by the Steinbroker criteria (I:7, II:10, III:9, IV:19) as well as clinical classes 2 (n?=?18), 3 (n?=?26), and 4 (n?=?1) and the mean disease activity score-CRP (DAS28-CRP) was 4.431?±?1.044. Patients were treated with methotrexate (mean dose 6.44?±?1.78?mg/week), prednisolone (PSL) (mean dose 1.078?±?1.871?mg/d), and golimumab (44.4% of 100?mg). RRP was evident in 20% of the large joints treated with golimumab, and, therefore, golimumab was effective at inhibiting large joint destruction in 80% of joints. RRI was evident in 33.3% of large joints following golimumab treatment. We also observed that EULAR response criteria significantly correlated with the ARASHI change score at 52 weeks after treatment. The total ARASHI status score significantly correlated with the Sharp–van der Heijde score, but not with the delta total sharp score. Multiple regression analyses revealed that the total ARASHI change score was only correlated with EULAR response criteria significantly.

Conclusions: Golimumab therapy was effective at inhibiting large joint destruction of RA patients who have good clinical response, including higher improvement of the shoulder and ankle joints than other large joints.     

Homozygosity for the CARD15 frameshift mutation 1007fs is predictive of early onset of Crohn's disease with ileal stenosis,entero-enteral fistulas,and frequent need for surgical intervention with high risk of re-stenosis

Objective. The identification of CARD15 as a susceptibility gene for Crohn's disease (CD) offers new possibilities for patient classification and risk assessment. The purpose of this study was to carry out a CARD15 sequence analysis in a large single-center IBD cohort and to investigate the impact of different genotypes on disease phenotypes. Material and methods. A total of 445 unrelated patients with IBD (68.1% CD, 28.5% ulcerative colitis (UC), 3.4% indeterminate colitis (IC)) were included in the study. Clinical data were recorded by detailed questionnaire and analysis of the charts. CARD15 variants (R702W, G908R, 1007fs (frameshift)) were identified by DNA sequence analysis. Results. CARD15 variants were found in 142 inflammatory bowel disease (IBD) patients (31.9%) including 120 CD patients (39.6%). In CD, the presence of two CARD15 variants was associated with ileal disease (p=0.008 versus wild-type (wt); OR 4.04; 95% CI 1.36–11.96) and a fibrostenotic phenotype (p=0.002 versus wt; OR 5.47; 95% CI 1.61–18.58). Subgroup analysis of 19 patients (4.3%) homozygous for the CARD15 variant 1007fs (3020ins C) revealed an association with onset of CD at an early age (p=0.014 versus wt), ileal involvement (p=0.001), and intestinal stenoses in all patients (p=0.001) frequently requiring surgery (73.7%; p=0.093). Of these patients 78.6% developed re-stenoses after surgical resection; 52.6% of the homozygotes were diagnosed as having entero-enteral fistulas. Conclusions. Patients homozygous for the 1007fs mutation had an early disease onset with long-segment ileal stenoses and entero-enteral fistulas. They frequently needed surgical intervention and had a high risk of re-stenosis. Genotyping therefore appears to be an important diagnostic tool in identifying severely affected patients requiring individualized treatment strategies at an early stage of the disease.     

Management of inflammatory bowel disease in pregnancy

Background and AimsInflammatory bowel disease (IBD) is a chronic disease affecting mainly young people in their reproductive years. IBD therefore has a major impact on patients’ family planning decisions. Management of IBD in pregnancy requires a challenging balance between optimal disease control and drug safety considerations.This article aims to provide a framework for clinical decision making in IBD based on review of the literature on pregnancy-related topics.MethodsMedline searches with search terms ‘IBD’, ‘Crohn's disease’ or ‘ulcerative colitis’ in combination with keywords for the topics fertility, pregnancy, congenital abnormalities and drugs names of drugs used for treatment of IBD.ResultsIBD patients have normal fertility, except for women after ileal pouch-anal anastomosis (IPAA) and men under sulfasalazine treatment. Achieving and maintaining disease remission is a key factor for successful pregnancy outcomes in this population, as active disease at conception carries an increased risk of preterm delivery and low birth weight.Clinicians should discuss the need for drug therapy to maintain remission with their patients in order to ensure therapy compliance. Most IBD drugs are compatible with pregnancy, except for methotrexate and thalidomide. If possible, anti-TNF therapy should be stopped by the end of the second trimester and the choice of delivery route should be discussed with the patient.ConclusionsDisease control prior to conception and throughout pregnancy is the cornerstone of successful pregnancy management in IBD patients.     

Prevalence of cardiovascular disease in subjects hospitalized due to chronic obstructive pulmonary disease in Beijing from 2000 to 2010

Objectives To investigate the overall prevalence of cardiovascular disease (CVD) in subjects hospitalized for chronic obstructive pulmonary disease (COPD), and explore the prevalence of the major CVD complications and trends in patients with COPD over a 10-year period. Methods Medical records in the PLA General Hospital, Beijing Union Medical College Hospital, and Beijing Hospital from 2000/01/01 to 2010/03/03 were retrospectively reviewed. A total of 4960 patients with COPD were reviewed in the study (3570 males, mean age, 72.2 ± 10.5 years; 1390 females, mean age, 72.0 ± 10.4 years). Results The prevalence of CVD in COPD patients was 51.7%. The three most prevalent CVDs were ischemic heart disease (28.9%), heart failure (19.6%), and arrhythmia (12.6%). During the 10-year study period, the prevalence of various CVDs in COPD patients showed a gradual increasing trend with increasing age. There was higher morbidity due to ischemic heart disease (P < 0.01) in male COPD patients than in the female counterparts. However, heart failure (P < 0.01) and hypertension (P < 0.01) occurred less frequently in male COPD patients than in female COPD patients. Furthermore, the prevalence of ischemic heart disease decreased year by year. In addition to heart failure, various types of CVD complications in COPD patients tended to occur in younger subjects. The prevalence of all major types of CVD in women tended to increase year by year. Conclusions The prevalence of CVD in patients hospitalized for COPD in Beijing was high. Age, sex and CVD trends, as well as life style changes, should be considered when prevention and control strategies are formulated.     

Recurrent posterior scleritis and orbital myositis as extra-intestinal manifestations of Crohn's disease: Case report and systematic literature review

BackgroundOcular episcleritis and uveitis are well-recognised extra-intestinal manifestations of Crohn's disease. Orbital myositis is rare: to our knowledge it has been associated with Crohn's disease in thirteen cases. Posterior scleritis, orbital myositis and Crohn's disease have been reported as coexisting in only two cases.Methods and resultsWe describe a third case, that of a 31-year old female with Crohn's colitis for 8 years, complicated by enteropathic arthritis and pyoderma gangrenosum. She presented with intense and intractable periorbital pain, particularly at night and worse on eye movements. B-scan ultrasonography confirmed posterior scleritis and treatment with high dose oral steroids (up to 60 mg prednisolone) was initially effective, but subsequently failed to control the inflammation. There was only a partial response to infliximab. Five months after presentation, diplopia developed, with failure of abduction of the left eye. MRI scan of the orbits confirmed orbital myositis involving the left lateral and medial rectus muscles. Pulsed intravenous methylprednisolone and six cycles of intravenous cyclophosphamide over a three month period resulted in complete resolution of inflammatory symptoms.ConclusionsThis case highlights a rare combination of ocular abnormality secondary to Crohn's disease and reports successful resolution with aggressive immunosuppressive therapy.