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Background: Tularemia is a zoonotic infection caused by Francisella tularensis , an aerobic, facultative intracellular coccobacillus, encountered especially in the Northern hemisphere. F. tularensis is a pathogen of humans and hundreds of animal species. Patients and methods: A Belgian traveler returning from an adventurous vacation in Central Europe presents fever, flu-like symptoms, a skin ulcer with a necrotic center resembling an eschar on the left thigh and painful left inguinal lymphadenopathy. An enzyme linked immunosorbent assay developed by the National Reference Laboratory for Tularemia, Sciensano, Belgium, detected elevated Ig G antibodies against F. tularensis , while the rest of the serologies were negative. Results: A highly likely case of ulceroglandular tularemia is described and the differential diagnosis is discussed. Conclusion: The incidence of tularemia has been increasing throughout Europe in recent years. Physicians should be aware of this disease, its diversity of reservoirs, transmission routes and clinical presentations. [ABSTRACT FROM AUTHOR]

Chikungunya virus (CHIKV) is an arthropod-borne virus that is transmitted by Aedes mosquitoes, and its main features are high fever and (debilitating) arthritis. Infection with CHIKV, as well as other viruses, has been associated with hypercoagulable states and may be linked with the development of venous thrombosis. In fact, the development of deep venous thrombosis has been described in CHIKV infection. We present a case of superficial thrombophlebitis of the thoracic wall, known as Mondor's disease, associated with CHIKV infection. To our knowledge, this probable association has never been described before. [ABSTRACT FROM AUTHOR]

Objective Previous studies have shown that the majority of patients with PMR have increased 18F-fluorodeoxyglucose (FDG) uptake around the shoulders, hips and processes of the cervical and lumbar spine on PET. The specificity of these findings for PMR is, however, not known. Methods We prospectively included 99 consecutive patients with a possible clinical diagnosis of PMR. All patients underwent 18F-FDG-PET scanning before treatment with glucocorticoids was started. The clinical suspicion of PMR was quantified by the treating physician on a scale from 1 to 5. FDG uptake was scored visually in 12 articular regions (scores 0–2) and a total skeletal score was calculated reflecting the FDG uptake in these 12 articular regions. Receiver operating characteristics analysis was performed to determine the optimal clinical and total skeletal score for diagnosing PMR. The gold standard for a diagnosis of PMR was the judgement of an experienced clinician after at least 6 months of follow-up. Results Sixty-seven patients were diagnosed with PMR while 32 patients got another diagnosis. A clinical score of 4 or more had a sensitivity of 67.2%, specificity of 87.5%, positive predictive value (PPV) of 91.8% and a negative predictive value (NPV) of 56.0% for the diagnosis of PMR. A total skeletal score of 16 or more had a sensitivity, specificity, PPV and NPV of, respectively, 85.1, 87.5, 93.4 and 73.7%. Conclusion 18F-FDG-PET before starting glucocorticoid therapy improves the diagnostic accuracy compared with a clinical scoring system in patients with clinical suspicion of PMR. [ABSTRACT FROM AUTHOR]

In patients presenting with nasal septum perforation, the differential diagnosis between ANCA-associated vasculitis and cocaine-induced midline destruction (CIMD) can be challenging. We describe the case of a 28-year old man who presented with a nasal septum perforation. He admitted the use of cocaine and showed no other symptoms of systemic inflammation. Perinuclear anti-neutrophilic cytoplasmatic antibodies (p-ANCAs) came back positive, as did anti-proteinase 3-antibodies. Further testing revealed antibodies to human neutrophil elastase (HNE), typically found in CIMD but rarely in ANCA-associated vasculitis. The combination of an atypical ANCA-pattern and the detection of HNE-antibodies led to the diagnosis of CIMD. In conclusion, HNE antibodies can be used to distinguish between CIMD and ANCA-associated vasculitis. [ABSTRACT FROM AUTHOR]

Abstract: Background and aims: Granulomas are a characteristic microscopic finding in Crohn''s disease. Their clinical significance is controversial and their pathogenesis is unknown, but impaired processing of bacterial components has been suggested. Autophagy is a fundamental process involved in the elimination of intracellular bacteria. Genetic variants in autophagy genes IRGM and ATG16L1 have been associated with susceptibility to Crohn''s disease. We therefore investigated whether variants in autophagy genes contribute to granuloma formation. Methods: Surgical specimens from 464 clinically well-documented Crohn''s patients were reviewed and scored for the presence and distribution of granulomas. All patients were genotyped for the CD-associated SNPs in ATG16L1 and IRGM as well as for 77 haplotype tagging SNPs in 13 additional autophagy genes. Results: Granulomas were found in 75% of the patients. Their frequency increased with more distal involvement of the GI tract. Granuloma positive patients were significantly younger at the time of diagnosis and surgery, and were more likely to smoke. We identified associations between granulomas and autophagy gene variants ATG4A (rs5973822), FNBP1L (rs17109951) and ATG4D (rs7248026; rs2304165; rs10439163). Conclusion: These findings suggest that granuloma formation is a marker of a more aggressive disease course, and that variants in autophagy genes ATG4A, ATG2A, FNBP1L and ATG4D, may contribute to granuloma formation. [Copyright &y& Elsevier]

Abstract: Background and aims: Infliximab (IFX) and iron sucrose (FeS) are of high value in inflammatory bowel disease (IBD). We aimed to assess the relative role of both therapies in IBD related anaemia and their safety when used in combination. Methods: IBD patients with anaemia receiving a first series of FeS infusions in addition to IFX were prospectively followed. We investigated serum kinetics of erythropoietin (EPO), soluble transferrin receptors (sTFRs) and vascular endothelial growth factor (VEGF). Results: Data analysis included 87 patients of whom 49.4% achieved the target Hb level of 12.0 g/dL. IFX resulted in a significant increase of EPO and sTFR compared to baseline pre-IFX levels (p =0.029 and p =0.005 respectively) and after a 12-week combined FeS and IFX treatment, EPO and sTFR levels dropped significantly compared to pre-FeS levels (p <0.001 for both). Infusion related adverse events were recorded in 2 IFX treated patients (2.3%, 0.7% of the infusions) and were mild. Disease activity and quality of life were not affected. Conclusions: In anaemic IBD patients treated with IFX, combined administration of FeS is safe. Infliximab significantly increases serum EPO and sTFR levels resulting in an increased functional iron deficiency, which is restored after combined treatment with I.V. iron sucrose. [Copyright &y& Elsevier]