Ana Arana-Guajardo

Wernicke Encephalopathy Presenting in a Patient with Severe Acute Pancreatitis

CONTEXT Acute pancreatitis can lead to prolonged fasting and malnutrition. Many metabolic changes, including thiamine deficiency, may lead to the well know pancreatic encephalopathy. In this condition however the thiamine deficiency is rarely suspected. CASE REPORT We report the case of a 17-year-old woman with severe acute pancreatitis who developed mental status changes and ophthalmoplegia. A magnetic resonance image showed hyperintensive signals in periventricular areas, medial thalamus, and mammillary bodies, findings consistent with the diagnosis of Wernicke encephalopathy. Thiamine treatment reversed neurological complications. CONCLUSION Wernicke encephalopathy secondary to thiamine deficiency should be considered as a possible cause of acute mental status changes in patients with acute pancreatitis and malnutrition. Prophylactic doses of thiamine could be considered in susceptible patients.

Aplicación de un modelo de predicción de progresión de artritis reumatoide en pacientes con artritis indiferenciada

INTRODUCTION Different prediction rules have been applied to patients with undifferentiated arthritis (UA) to identify those that progress to rheumatoid arthritis (RA). The Leiden Prediction Rule (LPR) has proven useful in different UA cohorts. OBJECTIVE To apply the LPR to a cohort of patients with UA of northeastern Mexico. METHODS We included 47 patients with UA, LPR was applied at baseline. They were evaluated and then classified after one year of follow-up into two groups: those who progressed to RA (according to ACR 1987) and those who did not. RESULTS 43% of the AI patients developed RA. In the RA group, 56% of patients obtained a score ≤ 6 and only 15% ≥ 8. 70% who did not progress to RA had a score between 6 and ≤ 8. There was no difference in median score of LPR between groups, p=0.940. CONCLUSION Most patients who progressed to RA scored less than 6 points in the LPR. Unlike what was observed in other cohorts, the model in our population did not allow us to predict the progression of the disease.

Vólvulo sigmoideo como presentación de una displasia neuronal intestinal tipo B en un adolescente

We report a 17-year-old man admitted to emergency department due to abdominal pain and distension. He referred a history of 18 months with constipation. Computerized tomography showed dilatation of the colon and coprostasis. The patient improved after disimpaction with enemas. Colonoscopy and anorectal manometry were reported normal. A full thickness rectal biopsy revealed normal ganglion cells. The patient was readmitted with a sigmoid volvulus. Sigmoidectomy and a termino-terminal anastomosis (descending colon-rectum) were performed (Figs. 1A and B). Histologically, neuronal intestinal dysplasia type B (NID-B) (NID) was reported (Fig. 1 C). Dehiscence and anastomatic leakage were found along with dilatation of the descending and transverse colon. Subtotal colectomy with a colostomy of the ascending colon and Hartmann’s pouch were performed. The resected segments showed NID-B.

Time Delay to Rheumatology Consultation Rheumatoid Arthritis Diagnostic Concordance Between Primary Care Physician and Rheumatologist

To the Editor: Rheumatology referral of patients with suspected arthritis impacts in the prognosis of rheumatoid arthritis (RA) because of earlier diagnosis and treatment initiation. An assessment in <12 weeks of symptom onset is associated with less joint destruction and a greater chance of achieving disease-modifying antirheumatic drugs (DMARD)–free remission as compared with a longer delay to assessment. The aim of the study was to evaluate the time to evaluation by the rheumatologist from the primary care provider (PCP) and their diagnosis concordance. We conducted a retrospective observational study from January to December 2013 in a Northern Mexico second-level public hospital. We evaluated the time to evaluation between rheumatologists and the referring PCP diagnosis and their concordance. We made a descriptive analysis and used κ coefficient to assess the level of concordance. We classified the patients’ main complaints at rheumatology consultation as follows: any pain in the upper extremities (hand, elbow, and shoulder), hip, lower extremities (knees and feet), and complaint of morning stiffness. The study was approved by the Institutional Review Board (Registry No. R-2014-1906-29). We evaluated 206 referrals; the most common cause of referral was RA. We found a mean time of 112.8 weeks (SD 187.6) (28.2 months [SD 46.9]) from onset of symptoms to evaluation by the rheumatologist, and a mean wait time of 9.5 weeks (SD 5.6) from PCP referral to the rheumatologist consultation, corresponding to 7.8% of latency of the disease. RA diagnosis concordance between PCP and rheumatologist was 0.131 (P = .041). Previously, Corominas et al delineated 4 steps to describe the delay from diagnosis to commencing treatment for RA: time from symptom onset to the first visit with the rheumatologist, time from referral to the first visit to rheumatology, time between symptom onset and final diagnosis, and time between first symptom to initiation of the first DMARD. In our study, the median time delay between symptom onset and rheumatologist evaluation was 28 months, in contrast with other authors who reported a median delay of 10 months. Even though 82% of patients were treated at a rheumatology clinic before 12 weeks, the wait time between seeing a PCP and seeing a rheumatologist was 9.5 weeks on average. Although diagnostic concordance was low between physicians, we observed that the presence of pain in the upper limbs (including hands, elbows, and shoulders) and morning stiffness were associated with a final diagnosis of RA (odds ratio = 10.5, 95% confidence interval = 1.3–81.4; and odds ratio = 3.04, 95% confidence interval = 1.69–5.45, respectively). This is consistent with other reports and triage strategies. In conclusion, we found a sizable delay in time to rheumatology consultation with low diagnostic concordance. We consider that the continuous education of not only doctors but also patients will improve early referral and therefore modify the time to diagnosis, treatment, and prognosis of rheumatic diseases.

Are RA patients from a non-endemic HCV population screened for HCV? A cross-sectional analysis of three different settings

INTRODUCTION In Mexico, other risk factors are associated with hepatitis C virus (HCV): prior heroin users, living alone, widower, and northern region residence. Rheumatoid arthritis (RA) patients are considered immunosuppressed and HCV testing is recommended before treatment. The aim of the study was to describe the characteristics of HCV testing in RA patients in three different medical care settings in a non-endemic area. METHODS A retrospective observational study was performed using medical records from 960 RA patients describing the indications for HCV testing. RESULTS The test was performed in 28.6% and the HCV overall frequency was 0.36%. Population characteristics were not associated with an increased risk of HCV infection; therefore, anti-HCV positivity was low. The main reason for testing was before starting biological agents. CONCLUSION Due to the low pre-test probability, testing for HCV infection should be personalized; i.e., according to disease prevalence in a particular geographical location and the individual risk factors.

Esophageal symptoms and their lack of association with high-resolution manometry in systemic sclerosis patients

BACKGROUND The esophageal involvement in systemic sclerosis (SSc) causes impact in the morbidity and mortality. High resolution manometry assesses esophageal involvement. Our aim was to categorize esophageal motor disorder in patients with SSc by HRM. METHODS We carried out an observational, descriptive and cross-sectional study. All patients underwent HRM as well as semi-structured interviews to assess frequency and severity of upper GI symptoms. Patients also completed the gastroesophageal reflux questionnaire (Carlsson-Dent). RESULTS We included 19 patients with SSc, 1 with morphea, and 1 with scleroderma sine scleroderma. Dysphagia and heartburn were the most frequent symptoms (61% each). We found an abnormal HRM in 15 (71.4%) patients. We found no statistically significant association between clinical or demographic variables and an abnormal HRM, or between any upper GI symptom and HRM findings. CONCLUSION We observed a high prevalence of esophageal symptoms and of HRM abnormalities. However, there was no clear association between symptomatology and HRM findings. HRM does not seem to accurately predict upper GI symptomatology.

CREST Syndrome: Clinical Expression of the Disease

A 54-year-old woman presented with a 2-year history of dyspepsia and intermittent dysphagia to solids and athralgias in hands and wrists. At physical examination, we found in the skin telangiectasia on the palate (Fig. A) and palms (Fig. B) and saltand-pepper skin in ears, distal forearm, and lower limb (Fig. C) and sclerodactyly. The tests showed positive antinuclear antibody 1:1280 with centromeric pattern, antitopoisomerase antibody negative, and anticentromere antibody positive 1:10,240. According to the American College of Rheumatology classification criteria, patients with CREST syndromemust meet at least 3 of the following clinical features: calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. We show some cutaneous presentations of the CREST syndrome and the need to look for them intentionally. In the case of telangiectasias, whose etiology is not fully understood, it is known to occur more in limited sclerosis patients such as in this case. In early stages, they are located in the fingers, hands,

Automated squeeze test (Gaenslen’s manoeuvre) to identify patients with arthralgia suspicious for progression to RA: improving time delay to rheumatology consultation

We read with interest the article by van Steenbergen et al 1 and the response by Mankia et al .2 In the former, a definition of suspicious arthralgia was proposed and in the latter a new approach to identifying individuals at risk of progression to rheumatoid arthritis (RA) was discussed. We agree with Mankia et al that general practitioners (GPs) are the first contact for those patients at risk. In other countries a delay in referring patients with RA has been seen, and we have similarly detected a delay of 28.2 (SD 46.9) …

THU0581 What we see, what we learn, and the prevalence of rheumatic diseases in our population: a diagnosis correlation study

Background The postgraduate program in rheumatology aims learning of musculoskeletal and autoimmune disorders. In México, objectively-structured clinical examination (OSCE) is applied in postgraduate certification processes by the Mexican Board of Rheumatology annually [1]. Peláez-Ballestas et al. described an epidemiological study (COPCORD, Community Oriented Program for the Control of Rheumatic Diseases) of 19,213 individuals in 5 regions in our country where they found a prevalence of musculoskeletal pain in 25.5%, osteoarthritis in 10.5%, back pain in 5.8%, rheumatic regional pain syndromes in 3.8%, rheumatoid arthritis (RA) in 1.6%, and fibromyalgia in 0.7% [2]. Objectives The aim of the study is to describe the student training in rheumatic diseases and correlate them with OSCE assessment and the prevalence of rheumatic diseases in our population. Methods An observational and analytical study was made between March 2014 to March 2015 in a single rheumatology training center at University Hospital. Student training was defined according to the times they evaluated patients with a determined diagnosis, this information was obtained by medical records. We categorize OSCE questions according to the rheumatic diagnosis. Finally, the two results were compared with prevalence of the rheumatic diagnosis according to COPCORD, which were registered according a score pain >4. We made descriptive statistics and a Spearmans Rho to evaluate the correlations of the diagnosis frequencies by each category. Results We reviewed 6279 medical records, 854 (13.6%) were of first-time evaluation. We had 5,400 (86.4%) women, with a mean age of 47.9 (SD 15.45) years. Descriptive statistics are in Table 1 and Figure 1, which included: medical consultations, OSCE assessment and a column with rheumatologic diagnosis according to COPCORD.Table 1 Resident MRC COPCORD n % n % n % RA 1776 28,28 35 15,28 307 5,93 OA 1061 16,90 9 3,93 2017 38,98 SLE 800 12,74 27 11,79 13 0,25 Soft tissue 554 8,82 10 4,37 2230 43,10 FM 476 7,58 5 2,18 0 0,00 Sjögren S 357 5,69 7 3,06 0 0,00 Osteoporosis 307 4,89 5 2,18 0 0,00 SSc 170 2,71 11 4,80 4 0,08 APS 111 1,77 10 4,37 0 0,00 Spondyloarthritis 111 1,77 5 2,18 27 0,52 Miositis 99 1,58 10 4,37 0 0,00 Juvenile Arthritis 87 1,39 3 1,31 0 0,00 PsA 86 1,37 5 2,18 0 0,00 Vasculitis ANCA 79 1,26 5 2,18 0 0,00 Raynaud 61 0,97 5 2,18 0 0,00 Crystal arthropaties 58 0,92 15 6,55 576 11,13 Connective tissue 45 0,72 0 0,00 0 0,00 The Spearman correlation coefficients of the 32 different diagnoses were: student training vs OSCE 0.492 (p=0.004), student training vs COPCORD 0.597 (p=0.01) and OSCE vs COPCORD 0.624 (p=0.01). Conclusions Although the most common musculoskeletal disease in our community did not obtain the frequency observed by students or evaluated in the OSCE, we observed a moderate correlation. We considered it is important to enhance the knowledge and improve the OSCE according to the most prevalent diseases to prepare the future rheumatologists. References Pascual Ramos V, et al. Reumatol Clin 2014; doi: 10.1016/j.reuma.2014.10.007. Peláez-Ballestas I, et al. J Rheumatol Suppl 2011;86:3–8. Disclosure of Interest None declared

AB0023 Association between mex-sledai and infections with mbl structural and promoter genotypes in mexican-mestizo patients

Background The mannose-binding lectin protein (MBL) is a multimeric molecule with a structure that is the analogue to the C1q protein. Deficient and low MBL concentrations in serum are due to the presence of mutations in the structural or promoter region Objectives To investigate the role of alleles and haplotypes of MBL2 gene in the clinical expression of systemic lupus erythematosus (SLE) and its association with infections in Mexican-mestizo patients. Methods An observational, cross-sectional, retrospective study. We included 74 SLE patients and 75 matched controls. All ≥16 years-old who met at least four 1982 or revised 1997 ACR criteria for SLE were included. The association of MBL locus haplotypes with disease activity and past history of infection was studied in those patients. Allele and haplotype determinations in the promoter and structural regions of the MBL2 gene were performed from genomic DNA isolated peripheral blood. Probes were sent to Invitrogen (Carlsbad, California) for synthesis. The disease activity was determined by MEX-SLEDAI. Infections were categorized arbitrarily if patients had ≥4 events. The associations between the codons, clinical activity, and having ≥4 infection events were by odds ratio. Results There were 13/73 (17.8%) SLE patients with ≥4 infections. The presence of homozygous C/C codon 57 was observed to be greater risk for SLE activity and present more than 4 infections. The significance of heterozygous HYLX promoter was observed only for the presence of infection. Table 1.Table 1. Association between MEX-SLEDAI and Infections with MBL structural and promoter genotypes in SLE patients MEX-SLEDAI p* Patients with Infections events ≥4, Total patients with infections events ≥4, p** OR median (IQR) n (%) n (%) Codon 52, n=71 0.68 12 (16.9) 0.44  A/A 3 (6) 10 (17.5)  A/D 3 (8) 0 (0)  D/D 1 (7) 2 (25) Codon 57, n=73 0.02 13 (17.8) 0.03 8.7 (1.2–58)  A/A 2 (6) 10 (14.7)  A/C 0 (0) 0 (0)  C/C 9 (0) 3 (60) Promoter, n=74 0.35 13 (17.6) 0.01  HYHY 3 (7) 2 (18.2)  LYLY 2 (8) 5 (21.7)  LXLX 1.5 (6) 1 (4.8)  LXLY 2.5 (5) 2 (13.3)  HYLY 5 (0) 1 (50)  HYLX 0 (0) 2 (100) MEX-SLEDAI: Mexican Systemic Lupus Erythematosus Disease Activity Index, IQR: interquartile range, OR: Odds ratio. *Kruskal–Wallis H test, ** Chi-square test. Conclusions MBL2 gene polymorphisms of the homozygous C/C in codon 57 of the structural region and heterozygous HYLX of the promoter region are associated with increased risk of a higher number of infections. Also, we observed that homozygous C/C in codon 57 was asociated to a higher MEX-SLEDAI. Disclosure of Interest None declared