Jorge Reis

Crohn's disease in a southern European country: Montreal classification and clinical activity

Background: Given the heterogeneous nature of Crohns disease (CD), our aim was to apply the Montreal Classification to a large cohort of Portuguese patients with CD in order to identify potential predictive regarding the need for medical and/or surgical treatment. Methods: A cross‐sectional study was used based on data from an on‐line registry of patients with CD. Results: Of the 1692 patients with 5 or more years of disease, 747 (44%) were male and 945 (56%) female. On multivariate analysis the A2 group was an independent risk factor of the need for steroids (odds ratio [OR] 1.6, 95% confidence interval [CI] 1.1–2.3) and the A1 and A2 groups for immunosuppressants (OR 2.2; CI 1.2–3.8; OR 1.4; CI 1.0–2.0, respectively). An L3+L34 and L4 location were risk factors for immunosuppression (OR 1.9; CI 1.5–2.4), whereas an L1 location was significantly associated with the need for abdominal surgery (P < 0.001). After 20 years of disease, less than 10% of patients persisted without steroids, immunosuppression, or surgery. The Montreal Classification allowed us to identify different groups of disease severity: A1 were more immunosuppressed without surgery, most of A2 patients were submitted to surgery, and 52% of L1+L14 patients were operated without immunosuppressants. Conclusions: Stratifying patients according to the Montreal Classification may prove useful in identifying different phenotypes with different therapies and severity. Most of our patients have severe disease. (Inflamm Bowel Dis 2009)

Estimating the prevalence of inflammatory bowel disease in Portugal using a pharmaco-epidemiological approach.

To estimate inflammatory bowel disease (IBD) prevalence in Portugal from 2003 to 2007, and to obtain disease, sex and age specific estimates.

Reversible Henoch–Schönlein purpura complicating adalimumab therapy

The tumour necrosis factor antagonists have demonstrated efficacy in the induction of remission and its maintenance in numerous chronic inflammatory conditions. These agents are generally well tolerated but with the increasing number of patients receiving anti-tumour necrosis factor-α (anti-TNFα) therapy, more adverse reactions are expected to occur. Cutaneous eruptions complicating treatment with anti-TNFα agents are common, occurring in around 20% of patients. Most reactions are mild-to-moderate and rarely warrant treatment withdrawal. We herein present a case of Henoch-Shönlein purpura (HSP) vasculitis following treatment with the monoclonal anti-TNFα antibody adalimumab for ileo-colic Crohns disease. The reaction occurred after 18 months of adalimumab therapy and discontinuation of the anti-TNFα resulted in rapid improvement of the condition. The causal relationship has become even more likely when the purpura reappeared after restarting adalimumab. The patient started infliximab, with disease control and no cutaneous side effects. To the best of our knowledge, this is the second case report of HSP complicating adalimumab therapy. Although adalimumab is theoretically less related to immune-mediated reactions, clinicians must be aware that adverse side effects may still occur. This is the first case that shows that infliximab can be safely used in patients with adalimumab related HSP. We discuss the literature and potential causal mechanisms and propose possible approaches to its management.

Ulcerative colitis in a Southern European country: a national perspective

Background:The incidence, prevalence, and even the clinical behavior of ulcerative colitis (UC) are highly variable in different world regions. In previous studies, Portugal was reported as having a milder clinical behavior. The aim of this study was to apply the Montreal Classification in a large group of UC Portuguese patients in order to describe their clinical characteristics and evaluate variables potentially useful for outcome prediction. Methods:A cross‐sectional study based on data collected from a nationwide online registry was undertaken. Results:In all, 2863 patients with UC were included. Twenty‐one percent had ulcerative proctitis, 52% left‐sided colitis, and 28% extensive colitis. Sixty percent of patients had taken steroids, 14% immunosuppressors, 1% biologicals, and 4.5% were submitted to surgery. Patients with extensive colitis had more severe activity, needing more steroids, immunosuppressors, and surgery. At the time of diagnosis 61% were less than 40 years old and 5% less than 16. Younger patients also had a more aggressive initial course. Thirty‐eight percent of patients had only taken salicylates during the disease course and were characterized by a lower incidence of systemic symptoms at presentation (3.8% versus 8.8%, P < 0.001), fewer extraintestinal manifestations (7.7% versus 24.0%, P < 0.001), and a higher prevalence of proctitis (32.1% versus 10.0%). Conclusions:A more aggressive phenotype was found in extensive colitis and in the initial course of younger patients, with an increased need for steroids and immunosuppressors. In addition, a significant percentage of patients, particularly with proctitis, showed a milder clinical evolution and were maintained in remission only with salicylates. (Inflamm Bowel Dis 2009)

Can Red Cell Distribution Width Be Used as a Marker of Crohn's Disease Activity?

Introduction Recently, it has been suggested an association between red cell distribution width (RDW) and Crohns disease activity index (CDAI), but its use is not yet performed in daily clinical practice. Objectives To determine whether RDW can be used as a marker of Crohns disease (CD) activity. Methods This was a cross-sectional study including patients with CD, observed consecutively in an outpatient setting between January 1st and September 30th 2013. Blood cell indices, erythrocyte sedimentation rate (ESR), and C-reactive protein were measured. CD activity was determined by CDAI (active disease if CDAI ≥ 150). Associations were analyzed using logistic regression (SPSS version 20). Results 119 patients (56% female) were included in the study with a mean age of 47 years (SD 15.2). Twenty patients (17%) had active disease. The median RDW was 14.0 (13–15). There was an association between RDW and disease activity (p = 0.044). After adjustment for age and gender, this association remained consistent (OR 1.20, 95% CI 1.03–1.39, p = 0.016). It was also found that the association between RDW and disease activity was independent of hemoglobin and ESR (OR 1.36, 95% CI 1.08–1.72, p = 0.01) and of biologic therapy (OR 1.19, 95% CI 1.03–1.37, p = 0.017). A RDW cutoff of 16% had a specificity and negative predictive value for CDAI ≥ 150 of 88% and 86%, respectively. Conclusion In this study, RDW proved to be an independent and relatively specific marker of CD activity. These results may contribute to the implementation of this simple parameter, in clinical practice, aiming to help therapeutic decisions.

Is there a proximal shift in the distribution of colorectal adenomas

Introduction Several studies have shown a proximal shift of colorectal cancer (CRC) during the last decades. However, few have analyzed the changing distribution of adenomas over time. Aim The aim of this study was to compare the site and the characteristics of colorectal adenomas, in a single center, during two periods. Methods We conducted a retrospective, observational study in a single hospital of adenomas removed during a total colonoscopy in two one-year periods: 2003 (period 1) and 2012 (period 2). Patients with inflammatory bowel disease, familial adenomatous polyposis, hereditary non-polyposis colorectal cancer syndrome, or history of CRC were excluded from the study. The χ2 statistical test was performed. P values less than 0.05 were considered statistically significant. Results During the two considered periods, a total of 864 adenomas from 2394 complete colonoscopies were analyzed: 333 adenomas from 998 colonoscopies during period 1 and 531 adenomas from 1396 colonoscopies during period 2. There was a significant increase in the proportion of adenomatous polyps in the proximal colon from period 1 to 2 (30.6% to 38.8% (p = 0.015)). Comparing the advanced features of adenomas between the two periods, it was noted that in period 2, the number of adenomas with size ≥1 cm (p = 0.001), high-grade dysplasia (p = 0.001), and villous features (p < 0.0001) had a significant increase compared to period 1. Conclusion Incidence of adenomatous polyps in the proximal colon as well as adenomas with advanced features has increased in the last years. This finding may have important implications regarding methods of CRC screening.

Endometriosis: A Rare Cause of Large Bowel Obstruction

Large bowel obstruction can result in significant morbidity and mortality, especially in cases of acute complete obstruction. There are many possible causes, the most common in adults being colorectal cancer. Endometriosis is a benign disease, and the most affected extragenital location is the bowel, especially the rectosigmoid junction. However, transmural involvement and acute occlusion are very rare events. We report an exceptional case of acute large bowel obstruction as the initial presentation of endometriosis. The differential diagnosis of colorectal carcinoma may be challenging, and this case emphasizes the need to consider intestinal endometriosis in females at a fertile age presenting with gastrointestinal symptoms and an intestinal mass causing complete large bowel obstruction.

Actinomycosis Causing Recurrent Perianal Fistulae

Actinomycosis is a rare but easily curable infection that should be considered in the differential diagnosis of perianal fistulizing disease. We present the case of a 26-year-old woman with complex perianal fistulae, including trans-sphincteric and suprasphincteric fistulous tracts and a rectovaginal fistula, requiring multiple abscess drainages, seton placement, and antibiotic courses, with little improvement. After extensive investigation, Actinomyces spp. was identified in anal cytology. The patient underwent a 6-week course of intravenous penicillin followed by oral amoxicillin, with remarkable improvement. This case illustrates the importance of pursuing less common diagnoses in refractory complex perianal disease, such as actinomycosis.

Isolated Visceral Angioedema Induced by Angiotensin-Converting Enzyme Inhibitor

Visceral angioedema is a rare complication of therapy with angiotensin-converting enzyme (ACE) inhibitors. Clinical presentation includes nausea, vomiting, abdominal pain and diarrhea. Early detection of this entity can prevent recurrent episodes and unnecessary invasive procedures, including surgery. This article describes a 46-year-old-woman who presented to the emergency department with abdominal pain, associated with nausea and vomiting. She had been taking ramipril for 15 days. A computed tomography was performed which revealed thickening of a jejunal segment, with submucosal edema. ACE inhibitor-associated angioedema was suspected and the medication was discontinued, with resolution of symptoms in 48 h. After 7 months of follow-up, the patient is asymptomatic. Despite of its rarity, ACE inhibitor-induced small-bowel angioedema should be included in the differential diagnosis when patients receiving ACE inhibitor therapy present with abdominal complaints.

Acute Hepatitis in the DRESS Syndrome

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe, idiosyncratic reaction characterized by diffuse maculopapular rash, facial edema, lymphadenopathy, fever, eosinophilia and/or other leukocyte abnormalities, and involvement of internal organs as liver, kidney, heart and lung. Diagnosing this entity is specifically complicated due to the multiplicity of organs involved. DRESS syndrome must be recognized promptly and the causative drug withdrawn in order to improve patient outcomes. Indeed, it is a potentially life-threatening condition, with a reported mortality between 5 and 20%. We describe a case of a 22-year old woman admitted to our hospital with acute diffuse, pruritic rash associated with crampy abdominal pain, vomiting, diarrhea and fever three weeks after starting sulfasalazine therapy. Initially, laboratory parameters revealed normal white blood cell count and normal liver enzymes, but during hospitalization, eosinophilia developed and liver enzymes, including transaminases and cholestatic parameters, dramatically increased. The diagnostic of DRESS syndrome was made and sulfasalazine was withdrawn and as there were signs of disease severity, systemic corticotherapy was initiated, with gradually improvement of the rash and symptoms resolution. The patient was discharged home after thirty days of hospitalization.