A "Mix and Match" in Hemochromatosis-A Case Report and Literature Focus on the Liver.

Hemochromatosis is a genetic disorder characterized by increased iron storage in various organs with progressive multisystemic damage. Despite the reports dating back to 1865, the diagnosis of hemochromatosis poses a challenge to clinicians due to its non-specific symptoms and indolent course causing significant delay in disease recognition. The key organ that is affected by iron overload is the liver, suffering from fibrosis, cirrhosis or hepatocellular carcinoma, complications that can be prevented via early diagnosis and treatment. This review aims to draw attention to the pitfalls in diagnosing hemochromatosis. We present a case with multiorgan complaints, abnormal iron markers and a consistent genetic result. We then examine the relevant literature and discuss hemochromatosis subtypes and liver involvement, including transplant outcome and treatment options. In summary, hemochromatosis remains difficult to diagnose due to its symptom heterogeneity and rarity; thus, further education for practitioners of all disciplines is useful in facilitating its early recognition and management.

Impaired ankle-brachial index in antiphospholipid syndrome: Beyond the traditional risk factors.

INTRODUCTION: The patients with antiphospholipid syndrome (APS) associate an increased risk of atherosclerosis. OBJECTIVE: To determine the predictors of an abnormal ankle-brachial index (ABI), surrogate measure of atherosclerosis, in patients with APS. METHODS: The ABI was measured according to standard recommendations in 106 patients. Traditional cardiovascular risk factors were assessed in all cases. A large spectrum of APS antibodies was determined in 73 patients. RESULTS: A total of 106 patients diagnosed with APS were included. 28.3% patients included were found to have low ABI. Anti-beta 2-glycoprotein I (aß2GPI) IgG antibodies [4.00 (1.00-79.00) vs 3.00 (0.00-29.00) U/mL, P = 0.02] and antiprothrombin (aPT) IgM antibodies [4.50 (0.00-82.00) vs 3.00 (0.00-14.00) U/mL, P = 0.05] titers were found to be higher in patients with abnormal ABI. However, after multivariate regression analysis, only the aß2GPI IgG titer remained predictor of low ABI (P = 0.04). CONCLUSIONS: aß2GPI IgG associated with impaired ABI in patients with APS. This relation might reflect their involvement in the atherosclerosis occurrence.

Circulating Reticulocalbin 1 and Reticulocalbin 3 in Systemic Sclerosis Patients: Results of a Case Control Study.

BACKGROUND: Proteomic candidate biomarkers for systemic sclerosis (Ssc) useful for appropriate patient evaluation and follow-up were identified in mass-spectrometry studies; however, most of these biomarkers were not evaluated and confirmed on independent patient samples. Up-regulation of reticulocalbin 1 (RCN1) and reticulocalbin 3 (RCN3) in the dermal fibroblast secretome originating from Ssc patients was previously described. The aim of the study was to evaluate circulating RCN1 and RCN3 as candidate biomarkers for Ssc clinical expression. METHODS: 40 consecutive Ssc patients and 20 gender and age matched controls were included. Serum RCN1 and RCN3 was evaluated using commercial ELISA kits. RESULTS: Serum RCN1 and RCN3 were not statistically significant different between Ssc patients and healthy controls. Serum RCN1 and RCN3 were correlated in both Ssc and healthy control groups (p < 0.001). Serum RCN1 was positively correlated with Ssc disease activity score (EUSTAR, p = 0.02) and remained associated with EUSTAR after adjusting for disease duration in multivariate analysis. 6 Ssc patients (15%) had elevated RCN1 values compared to reference values obtained from healthy control samples. These patients had higher prevalence of digital ulcers, higher disease activity scores, and tended to have esophageal hypomotility, calcinosis, telangiectasia, and diffuse Ssc subtype. CONCLUSIONS: RCN1 and RCN3 expression was not statistically significantly different to healthy controls. However, RCN1 was associated with disease activity score and could be used as a stratification biomarker for Ssc patients, as patients with high RCN1 shared a particular disease pattern.

Trace Element Deficiency in Systemic Sclerosis-Too Much Effort for Some Traces?

Trace elements are essential for several physiological processes. To date, various data have suggested that inadequate levels of trace elements may be involved in the pathogenesis of different chronic diseases, including immune-mediated ones, or may develop during their course. Systemic sclerosis (SSc) is a complex autoimmune multisystemic disease, primarily characterized by microvascular dysregulation, the widespread activation of the immune system and tissue fibrosis. According to the latest reports regarding the pathogenesis of SSc, the main pathophysiological processes-inflammation, vasculopathy and fibrosis-may include various trace element derangements. The present literature review aims to update the available data regarding iron, zinc, copper and selenium status in SSc as well as to underline the possible implications of these trace elements in the complexity of the pathogenic process of the disease. We observe that the status of trace elements in SSc plays a crucial role in numerous pathogenic processes, emphasizing the necessity for proper monitoring and supplementation. The reported data are heterogenous and scarce, and future studies are needed in order to draw clearer conclusions about their complete spectrum.

Criteria and Non-Criteria Antiphospholipid Antibodies and Cancer in Patients with Involuntary Weight Loss.

Cancer patients have higher prevalences of antiphospholipid antibodies (aPLs), occasionally associated with thrombotic events. A cross-sectional study regarding the presence of criteria (IgG/IgM anti-cardiolipin-aCL, anti-ß2 glycoprotein I-aß2GPI) and non-criteria (IgG/IgM anti-phosphatidylserine-aPS, anti-phosphatidylethanolamine-aPE, anti-prothrombin-aPT) aPLs in 146 patients with involuntary weight loss was performed. None of the patients had thrombotic events during the study. Out of the 36 cancer patients, 33 had non-hematologic malignancies. In the cancer subgroup, 60% of the patients had at least one positive aPL, with significantly more patients being positive for aß2GPI IgG compared with the non-cancer subgroup-p = 0.03, OR = 2.23 (1.02-4.88). When evaluating the titres, aCL IgG/IgM, aß2GPI IgG, aPE IgG, and aPS IgG had significantly higher values in cancer patients, the best cancer predictor being aß2GPI IgG-AUC 0.642 (0.542-0.742). Gastrointestinal cancer patients were studied separately, and aCL IgM positivity was significantly higher-p = 0.008, OR = 6.69 (1.35-33.02). Both the titres of aCL IgM (p = 0.006) and aPS IgM (p = 0.03) were higher in the gastrointestinal cancer subgroup, with aCL IgM being the best predictor for gastrointestinal cancer development-AUC 0.808 (0.685-0.932). Despite criteria and non-criteria aPLs being frequent in cancer, their connection with thrombosis in these patients is probably dependent on other important risk factors and needs further research.

Criteria and Non-Criteria Antiphospholipid Antibodies in Antiphospholipid Syndrome: How Strong Are They Correlated?

The place of non-criteria antiphospholipid antibodies (aPLs) in the diagnosis of antiphospholipid syndrome (APS) is still debatable. The aim of this research was to evaluate the correlations between the titres of non-criteria aPLs (anti-phosphatidylethanolamine (aPE), anti-phosphatidylserine (aPS), and anti-prothrombin (aPT) antibodies), and the ones of the already studied criteria aPLs (anti-cardiolipin (aCL) and anti-ß2 glycoprotein I-aß2GPI antibodies). Altogether, 72 APS (30 primary and 42 secondary) patients were included in our study. High correlation coefficients (rs) were found between aPS IgM and aCL IgM, overall (0.77, p < 0.01), as well as in the primary (0.81, p < 0.01), and secondary (0.75, p < 0.01) APS subgroups. Low or statistically insignificant correlations were observed between IgG/IgM isotypes of aPT and aCL, or aß2GPI, in the entire study population, and when evaluating the subgroups. Therefore, moderate correlations were mainly identified between the tested non-criteria antibodies and the criteria ones, suggesting little added value for the use of the tested non-criteria aPLs, with the exception of aPT, which seems to have different kinetics and might be a promising APS diagnostic tool.

Chronic Complete Distal Aortic Occlusion and Pulmonary Embolism-Atypical Antiphospholipid Syndrome?

Complete aortic occlusion is a rare pathology with various possible etiologies. According to current data, it is most frequently caused by atherosclerosis. However, thrombosis or vasculitis could also be involved. We present the case of a 42-year-old female with chronic complete distal aortic occlusion, associated pulmonary embolism and positive antiphospholipid antibodies. The patient had an obstetric history suggestive of antiphospholipid syndrome (APS). She presented with typical intermittent claudication symptoms persisting for approximately five years at the time of admission. Arteriography revealed complete infrarenal aortic occlusion and the presence of collateral arteries. Aortoiliac bypass surgery was performed. This case emphasizes an unusual, yet possible, etiology of chronic aortic occlusion-most probably, combining atherosclerosis and chronic thrombosis-in a relatively young patient, in which the diagnosis was significantly delayed due to the peculiar association of traits.

Refractory Takayasu's Arteritis with Severe Coronary Involvement-Case Report and Literature Review.

This report presents the case of a female patient diagnosed with Takayasu arteritis from childhood, with severe, refractory coronary involvement, leading to two acute coronary syndromes and multiple anginous episodes. Consequently, the patient suffered aorto-bicarotid bypass two times, multiple interventional procedures with stent implantation, balloon angioplasty, and up to ten repeated in-stent restenosis that required reinterventions, despite being on maximal immunosuppressive treatment. In recent years, various studies have been reported that aim to best characterize this particular type of vascular damage and to indicate optimal therapeutic options for treatment. The latter should be based on the activity of the underlying disease; however, no reliable markers are available in TA. The management of TA patients with coronary involvement continues to be a challenge and requires both drug and interventional techniques to avoid life-threatening events.

Ferritin above 100 mcg/L could rule out colon cancer, but not gastric or rectal cancer in patients with involuntary weight loss.

BACKGROUND: A tenth of patients with involuntary weight loss (IWL) have gastrointestinal cancer. Ferritin is the first parameter to be modified during the process leading to iron deficiency anaemia, therefore it should be the most sensitive. The aim of this study was to assess the ability of ferritin to rule out gastrointestinal cancer in patients with involuntary weight loss. METHODS: All consecutive patients with IWL admitted in a secondary care university hospital were prospectively studied. Ferritin, haemoglobin with erythrocyte indices and serum iron were recorded for all patients. The reference standard was bidirectional endoscopy and/or 6 months follow-up. RESULTS: 290 patients were included, a quarter had cancer, of which 22 (7.6%) had gastrointestinal cancer (8 gastric cancer, 1 ileum cancer, 13 colorectal cancer). Ferritin had the best area under the curve (AUC), both for gastrointestinal cancer (0.746, CI: 0.691-0.794), and colorectal cancer (0.765, CI: 0.713-0.813), compared to the other parameters of iron deficiency. In the diagnosis of colorectal cancer, ferritin with a cut-off value of 100 mcg/L had a sensitivity of 93% (CI: 69-100%), and negative likelihood ratio of 0.13, with a negative predictive value of 99% (96-100%), while for gastrointestinal cancer, the sensitivity was lower (89%, CI: 67-95%), with a negative likelihood ratio of 0.24. There were three false negative patients, two with gastric cancer, and one with rectal cancer. CONCLUSION: In patients with involuntary weight loss, a ferritin above 100mcg/L could rule out colon cancer, but not gastric or rectal cancer.

COVID-19 Vaccine Does Not Increase the Risk of Disease Flare-Ups among Patients with Autoimmune and Immune-Mediated Diseases.

BACKGROUND: Reports describing post-vaccine autoimmune phenomena, in previously healthy individuals, increased the concerns regarding the risk of disease flare-ups in patients with immune diseases. We aimed to assess the potential risk of disease flare-up, after receiving the COVID-19 (Coronavirus disease 2019) vaccine, during a follow-up period of 6 months. METHODS: We performed a prospective cohort study, enrolling the patients with autoimmune- and immune-mediated diseases who voluntarily completed our questionnaire, both online and during hospital evaluations. Based on their decision to receive the vaccine, the patients were divided into two groups (vaccinated and non-vaccinated). Participants who chose not to receive the vaccine served as a control group in terms of flare-ups. RESULTS: A total of 623 patients, 416 vaccinated and 207 non-vaccinated, were included in the study during hospital evaluations (222/623) and after online (401/623) enrolment. There was no difference concerning the risk of flare-up between vaccinated and non-vaccinated patients (1.16, versus 1.72 flare-ups/100 patients-months, p = 0.245). The flare-ups were associated with having more than one immune disease, and with a previous flare-up during the past year. CONCLUSIONS: We did not find an increased risk of flare-up following COVID-19 vaccination in patients with autoimmune-/immune-mediated diseases, after a median follow-up of 5.9 months. According to our results, there should not be an obvious reason for vaccine hesitancy among this category of patients.

COVID-19 Impact and Vaccination Willingness among Romanian Patients with Autoimmune/Immune-Mediated Diseases.

BACKGROUND: During the COVID-19 pandemic, patients with immune diseases are a vulnerable population. We aimed to evaluate their access to medical care, as well as their awareness and willingness to obtain the vaccine after a year of the SARS-CoV-2 pandemic. METHODS: A cross-sectional, multicenter study was conducted on a questionnaire basis, handled both online as well as in person. RESULTS: 651 patients with autoimmune or immune mediated diseases were enrolled. More than half (339/641 [53%]) reported difficulties in obtaining medical care throughout the pandemic and 135/651 ([21%]) of them were confirmed with COVID-19; 442/651, ([68%]) expressed their willingness to be vaccinated against SARS-CoV-2. The factors associated with an increased probability of vaccination were the male gender (OR = 2.01, CI95% 1.2-3.7, p = 0.001), the patient's opinion that she/he was well informed (OR = 3.2, CI 95% 2.1-6.01, p < 0.001), physician's advice (OR = 2.1, CI 95% 1.3-3.5, p < 0.001), and flu vaccination in the past (OR = 1.5, CI 95% 1.1-2.3, p < 0.001), while those associated with a decreased probability of vaccination were COVID-19 disease in the past medical history (OR = 0.7, CI 95% 0.3-0.95, p = 0.02), and the opinion that patients with autoimmune diseases are at increased risk for adverse reactions (OR = 0.7, CI95% 0.53-0.89, p = 0.001). CONCLUSIONS: Given the fact that considering themselves informed regarding vaccination is the most important factor in order to be immunized against SARS-CoV-2, effective information campaigns would substantially increase willingness.

Correlation between Osteoprotegerin Levels and Antiphospholipid Syndrome Parameters.

OBJECTIVE: To identify the osteoprotegerin (OPG) correlates with antiphospholipid syndrome (APS) parameters. METHODS: Our cohort included 40 patients with primary APS disease associated with systemic lupus erythematosus (SLE) (mean age, 43.7 years; 87% female). Data on cardiovascular risk factors and specific clinical events in APS were collected. Then we tested OPG and 10 criteria and noncriteria antiphospholipid antibodies (aPLs) on preserved specimens in all cases. RESULTS: A total of 26 patients (65%) had high serum OPG levels. Patients with high OPG were mostly overweight. In patients with SLE, the OPG levels were associated with anti-double-stranded DNA (anti-dsDNA) and anti-Sm titers. However, we did not find significant correlations of the OPG with any of the 10 aPLs tested. Also, we found no relationship regarding venous APS events. CONCLUSION: In APS, high OPG levels are not linked to serum aPL expression.

Different effects of anti-TNF-alpha biologic drugs on the small bowel macroscopic inflammation in patients with ankylosing spondylitis.

BACKGROUND & AIMS: Considering the ability of anti-TNF alpha drugs to lower the burden intestinal inflammation in patients with inflammatory bowel disease (IBD), and the similarity between IBD and ankylosing spondylitis (AS) regarding inflammatory intestinal involvement, we aimed to investigate the impact of anti-TNF alpha biologic therapy on subclinical intestinal inflammation in AS patients. METHODS: Between January 2008 and December 2013, 38 AS patients and 23 controls were enrolled in the study and investigated with small bowel videocapsule endoscopy examination and ileocolonoscopy. Each tertile of the small bowel (proximal, mid and distal) was assessed by calculating the Lewis score based on the image stream. RESULTS: The Lewis scores were significantly higher in the AS group compared to controls (580.9 ± 818 vs. 81 ± 121, p<0.001). 16 patients (42.1%) were on anti-TNF alpha therapy (Adalimumab (n = 5), Infliximab (n = 5) or Etanercept (n = 6)).31.3% of them used NSAIDs simultaneously, compared with 77.3% of the other patients (p<0.01). Their Lewis scores were lower compared to the other patients for the entire small bowel (306 ± 164 vs. 790 ± 1038, p = 0.015), its proximal and distal tertiles (238 ± 154 vs. 560 ± 543, p = 0.021, and 140 ± 189 vs. 300 ± 220, p = 0.027, respectively). The Lewis score was also lower in patients receiving Adalimumab/Infliximab compared to those on Etanercept for the entire bowel and its distal tertile (262 ± 165 vs. 380 ± 148, p = 0.069 and 62 ± 101 vs. 273 ± 236, p = 0.060, respectively). CONCLUSION: Anti-TNF alpha therapy in patients with AS reduces the subclinical intestinal inflammation, but the magnitude seems to depend upon the class anti-TNF alpha agent used (Clinical Trials. gov NCT00768950).

Extended Antiphospholipid Antibodies Screening in Systemic Lupus Erythematosus Patients.

BACKGROUND: The antiphospholipid syndrome (APS) is one of the most encountered autoimmunity in systemic lupus erythematosus (SLE) patients and pathogenesis of these two seems to be intricate. AIM: To investigate the association of antiphospholipid antibodies (APLAs) titer with the presence of secondary APS diagnosis in SLE patients. METHODS: 65 patients fulfilling the 2012 Systemic Lupus Collaborating International Clinics (SLICC) SLE's criteria were included. The APS diagnosis was sustained according to the 2006 Sydney APS's criteria. Three groups of patients were defined: SLE patients with secondary APS, SLE with history of positive "criteria" APLAs but without APS clinical features, respectively SLE patients without positive APLAs or clinical APS criteria. An extended APLAs panel was searched in all cases: both IgM and IgG of anticardiolipin antibodies (aCL), anti-P2 glycoprotein I antibodies (aß2GPI), antiphosphatidylethanolamine antibodies (aPE), antiphosphatidylserine antibodies (aPS), respectively antiprothrombin antibodies (aPT). Results. Only the aß2GPI, both IgM and IgG serotypes, had significantly higher titers in patients with SLE and secondary APS compared to no APS (with/ without positive APLAs): median (min; max) 7.0 (0.0-300.0) vs. 1.0 (0.0-28.0) vs. 1.0 (0.0-12.0), respectively 3.0 (0.0-79.0) vs. 1.0 (0.0-3.0) vs. 1.0 (0.0-12.0) (p<0.001, Kruskal-Wallis test)]. Also, in regression logistic models, only the aß2 GPI (IgG and IgM ) were identified as risk factors for secondary APS diagnosis in the SLE patients: OR(95%CI) 5.9 (2.2-15.7), respectively 1.3 (1.1-1.5). In regard with the SLE markers, the IgG serotypes of the "non-criteria" APLAs analyzed (aPS, aPT, aPE) were correlated with the antiDNA titers while the IgM serotypes inversely associated with the complement C3 levels. CONCLUSIONS: IgG aß2 GPI are accompanied by almost 6-fold increase risk of secondary APS when screening SLE patients. On the contrary, the "non-criteria" APLAs do not seem associated with the APS diagnosis in SLE patients. Some correlates of the "non-criteria" APLAs with the antiDNA and complement C3 levels were also observed.

Cancer and involuntary weight loss: failure to validate a prediction score.

BACKGROUND: Many patients who have involuntary weight loss have cancer. The Hernandez prediction rule includes 5 variables (elevated levels of alkaline phosphatase and lactate dehydrogenase, low albumin, high white blood cell count, and age >80 years). The purpose of this study was to evaluate the validity of the prediction rule. METHODS: We prospectively evaluated 290 consecutive inpatients and outpatients who had involuntary weight loss. Clinical, hematologic, and biochemical parameters were determined. There were 259 patients who had follow-up at 6 months to determine the cause of involuntary weight loss, and 31 other patients were lost to follow-up. The 5 variables were introduced into a regression logistic model with cancer as a dependent variable. RESULTS: Cancer was diagnosed in 72 of the 290 patients (25%) who had involuntary weight loss. Bivariate analysis showed that serum albumin, C-reactive protein, erythrocyte sedimentation rate, alkaline phosphatase, iron, lactate dehydrogenase, white blood cell count, hemoglobin, and ferritin levels were associated with cancer (range of area under the receiver operating characteristic curve, 0.589 to 0.688). Multivariate analysis showed that albumin, erythrocyte sedimentation rate, iron, white blood cell count, and lactate dehydrogenase levels were associated with cancer. When dichotomized, only low albumin (odds ratio, 2.6, CI [1.3-5.2]) and high alkaline phosphatase (odds ratio, 2.3, CI [1.7-4.7]) were associated with cancer. The area under the receiver operating characteristic curve of the 5-variable prediction rule was only 0.70 (95% confidence interval, 0.61-0.78). The negative predictive value of this model with 3 variables (age >60 y, alkaline phosphatase, and albumin level) increased from 85% to 95% when all tests were negative. CONCLUSIONS: In patients who had involuntary weight loss, those who have cancer are likely to have ≥1 abnormal laboratory test. The 5-variable prediction rule had a significantly lower accuracy than originally reported. Further evaluation of the 3-variable modification of the prediction rule may be useful.

Small bowel inflammatory involvement in Behçet's disease associated spondyloarthritis is different from other spondyloarthritides. A prospective cohort study.

BACKGROUND & AIMS: Behçet's disease presents some similar clinical features with seronegative spondyloarthritides (SpA), raising the question whether a subgroup of patients with Behçet's disease belong in fact to the latter. As the already known inflammatory involvement at the level of the small bowel in SpA patients could not be extrapolated for patients with Behçet's disease associated SpA (BehSpA), the present study aims to investigate and compare it in these diseases. METHODS: 54 consecutive patients with a form of SpA, and 7 patients with BehSpA were enrolled and submitted to videocapsule endoscopy (VCE) examination. After reviewing the VCE findings, calculation of the score of small bowel mucosal inflammatory change (Lewis) was performed for each patient. The comparison was made with a control group, sex and age-matched to the patients in the study groups. RESULTS: The Lewis score differed in the groups considered for analysis (mean of 439, 179 and 81 in the SpA, BehSpA and the control group, respectively, with p = 0.04 for the comparison SpA vs. BehSpA and 0.05 for the comparison BehSpA vs. controls). C reactive protein (CRP) level was markedly reduced in the BehSpA group vs. the other SpA group (2.08 and 14.02 mg/L, respectively; p = 0.053). CONCLUSION: The significant difference in intestinal inflammatory involvement in BehSpA versus the other SpAs, as well as the significantly lower serum levels of CRP, as revealed by the present study, clearly draw a line between the two disease entities.

Serum cytokines and cancer in involuntary weight loss.

BACKGROUND: Tumor necrosis factor α (TNF-α), interleukin 1ß (IL-1ß), and IL-6 may be associated with involuntary weight loss in patients with and without cancer. However, results of previous studies have been conflicting. We evaluated patients who had involuntary weight loss to determine cytokine levels and the correlation of these cytokines with weight loss, the association with inflammation, and the potential for use in cancer diagnosis. MATERIALS AND METHODS: In 290 consecutive patients with involuntary weight loss (74 patients [26%] with cancer and 216 patients [74%] without cancer), erythrocyte sedimentation rate (ESR), and serum levels of C-reactive protein, TNF-α, IL-1ß, and IL-6 were determined. RESULTS: Higher ESR and levels of C-reactive protein, TNF-α, IL-1ß, and IL-6 were associated with cancer. The levels of TNF-α, IL-1ß, and IL-6 did not correlate with the amount of weight loss. In multivariable analysis, only ESR was associated with cancer. CONCLUSIONS: In patients with involuntary weight loss, TNF-α, IL-1ß, and IL-6 were associated with cancer but were not weight loss mediators.

Neuromyelitis optica--complication or comorbidity in primary Sjögren's syndrome?

We report two cases of neuromyelitis optica (NMO) associated with primary Sjögren's syndrome (pSS), comparing the clinical and laboratory features of these predominant neurological patients and reporting their different outcome. NMO - a severe demyelinating disorder of the central nervous system - primarily affects the spinal cord and optic nerves, resulting in longitudinally extensive transverse myelitis and/or optic neuritis. Our patients had a late pSS diagnosis, due to the absence of sicca syndrome and specific Sjögren serology in the early stages of their diseases, when the neurological symptoms prevailed. Many NMO patients have an accompanying autoimmune disease, most commonly Sjögren syndrome and systemic lupus erythematosus or a related profile of non-organ-specific autoantibodies. Neurologic involvement occurs in approximately 20% of patients with pSS, usually preceding the diagnosis (in 75-80% of the cases) [1,2]. The frequency of both neurologic manifestations (revealing pSS) and negative autoimmune serology, especially in the event of CNS involvement, could explain why underlying pSS is misdiagnosed [3,4]. Screening for pSS should be systematically performed in cases of acute or chronic myelopathy and/or cranial nerve involvement, mainly because these patients have a severe outcome. The presence of the anti-aquaporin4 antibodies, besides anti-Ro and anti-La, in both reported cases, is intriguing and raises the question of whether we are facing two distinct diseases or the NMO is just complicating an unusually less expressive Sjögren's syndrome subtype.

Utility of routine hematological and inflammation parameters for the diagnosis of cancer in involuntary weight loss.

INTRODUCTION: A quarter of patients with involuntary weight loss (IWL) have cancer. Inflammation and anemia are associated with cancer, and recent studies showed that red blood cell distribution width (RDW) is a predictor of mortality, including cancer-related death. The aim of this study was to assess the ability of routine hematological and inflammation parameters to diagnose cancer in patients with IWL. MATERIALS AND METHODS: A total of 253 consecutive patients with IWL admitted in a secondary care university hospital were included. Routine hematological and inflammatory parameters (hemoglobin level, mean corpuscular volume, RDW, serum iron level, erythrocyte sedimentation rate, C-reactive protein level, and ferritin level) were recorded for all patients. The investigative workup was not standardized, but the patients were followed up for 6 months to avoid misclassification concerning the final diagnosis. RESULTS: All parameters, excepting mean cellular volume, were statistically associated with cancer. The areas under the curve were 0.708 (95% confidence interval [CI], 0.627-0.790) for C-reactive protein level, 0.690 (95% CI: 0.620-0.760) for erythrocyte sedimentation rate, 0.651 (95% CI, 0.566-0.735) for serum iron level, 0.607 (95% CI, 0.526-0.687) for hemoglobin level, 0.598 (95% CI, 0.518-0.679) for ferritin level, 0.594 (95% CI, 0.517-0.671) for RDW, and 0.561 (95% CI, 0.474-0.649) for mean cellular volume. In the multivariable analysis, only erythrocyte sedimentation rate remained associated with cancer. CONCLUSIONS: In patients with IWL, the hematological and inflammation parameters were statistically different in patients with cancer and in those without cancer. However, in clinical practice, they were modest diagnostic tests for cancer.

NSAID-induced deleterious effects on the proximal and mid small bowel in seronegative spondyloarthropathy patients.

AIM: To investigate the small bowel of seronegative spondyloarthropathy (SpA) patients in order to ascertain the presence of mucosal lesions. METHODS: Between January 2008 and June 2010, 54 consecutive patients were enrolled and submitted to a video capsule endoscopy (VCE) examination. History and demographic data were taken, as well as the history of non-steroidal anti-inflammatory drug (NSAID) consumption. After reading each VCE recording, a capsule endoscopy scoring index for small bowel mucosal inflammatory change (Lewis score) was calculated. Statistical analysis of the data was performed. RESULTS: The Lewis score for the whole cohort was 397.73. It was higher in the NSAID consumption subgroup (P = 0.036). The difference in Lewis score between NSAID users and non-users was reproduced for the first and second proximal tertiles of the small bowel, but not for its distal third (P values of 0.036, 0.001 and 0.18, respectively). There was no statistical significant difference between the groups with regard to age or sex of the patients. CONCLUSION: The intestinal inflammatory involvement of SpA patients is more prominent in NSAID users for the proximal/mid small bowel, but not for its distal part.