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To assess the usefulness of FDG-PET/CT as a potential diagnostic tool for detecting underlying systemic diseases (SD) in patients with orbital inflammatory disorders (OID). All consecutive patients managed for new-onset OID between 2011 and 2018 in a tertiary referral center, who underwent FDG-PET/CT as part of the etiological diagnostic workup were evaluated. To quantify the incremental value of FDG-PET/CT over standard diagnostic workup, the Net Reclassification Index (NRI) and Integrated Discrimination Index (IDI) were used. Among the 22 patients enrolled, 11 (50%) had a positive FDG-PET/CT. After clinicobiological evaluation, FDG-PET/CT correctly reclassified 4(29%) of 14 patients with SD (p =.04) and 1(13%) of 8 with idiopathic orbital inflammation syndrome (p =.32). NRI and IDI were 0.41 ± 0.17 (p =.03) and 0.38 ± 0.08 (p <.001), respectively. FDG-PET/CT successfully detected asymptomatic lesions in all (n = 4) patients with lymphoma. FDG-PET/CT enabled accurate reclassification of more than one-quarter of patients with SD, especially extraorbital lymphomas. [ABSTRACT FROM AUTHOR]

Relapsing polychondritis (RP) is a rare systemic inflammatory disease primarily affecting the ears, nose and tracheobronchial tree cartilage, but also the cardiovascular system. Cardiovascular complications are the second cause of mortality in RP. We report the case of a woman with a corticosteroid-resistant RP-associated aortitis, who was successfully treated with tocilizumab (TCZ). The FDG-PET/CT was a useful tool for diagnosing aortitis and assessing the effect of biotherapy. We conducted a systematic literature review confirming this is the first case of rapid and sustained remission in a patient with corticosteroid-resistant RP-associated aortitis after TCZ treatment administered as a first-line immunotherapy. However, further studies are needed to confirm the beneficial effect of TCZ used in this life-threatening condition. [ABSTRACT FROM AUTHOR]

Angiotensin I-converting enzyme (ACE) is a well-known zinc-metallopeptidase that converts angiotensin I to the potent vasoconstrictor angiotensin II and degrades bradykinin, a powerful vasodilator, and as such plays a key role in the regulation of vascular tone and cardiac function. Increased circulating ACE (cACE) activity has been reported in multiple diseases, including but not limited to granulomatous disorders. Since 2001, genetic mutations leading to cACE elevation have also been described. This review takes advantage of the identification of a novel ACE mutation (25-IVS25 + 1G > A) in two Belgian pedigrees to summarize current knowledge about the differential diagnosis of cACE elevation, based on literature review and the experience of our centre. Furthermore, we propose a practical approach for the evaluation and management of patients with elevated cACE and discuss in which cases search for genetic mutations should be considered. [ABSTRACT FROM AUTHOR]

Background Type 2 diabetes is a risk factor for steatohepatitis and fibrosis. Non-invasive liver stiffness ( LS) and controlled attenuation parameter ( CAP) measurements by Fibroscan allow assessing liver fat and fibrosis. Aim To determine the prevalence of steatosis and significant fibrosis in a community-based diabetic population. Methods LS and CAP were measured in 705 patients using the standard 'M probe.' A second ' XL probe' was used, without CAP measurement, in case of failure with the 'M probe.' Results LS and CAP measurements were obtained in 437 patients (the M group), LS measurements ( LSM) with the XL probe being available in additional 232 patients. After the combined use of both probes, LSM failure and unreliable result were 1.6% and 5.6% respectively. Overall, 12.7% (n=85), 7.3% and 2.1% exhibited significant or advanced fibrosis or cirrhosis ( LSM≥8 kPa, ≥9.6 kPa, ≥13 kPa respectively), half of the patients with LSM≥8 kPa displayed normal liver tests. Significant and severe steatosis were measured in 75% and 24% of the M group patients. By multivariate analysis, factors associated with severe fibrosis were age, overweight, high GGT. Forty-seven patients with LSM≥8 kPa underwent liver biopsy; 93% had steatosis and 51% severe fibrosis. A significant correlation was found between LSM values and fibrosis score with an accuracy rate of 83%, 68% and 83% for LSM≥8 kPa, ≥9.6 kPa and ≥13 kPa respectively. Conclusions The prevalence of significant steatosis is very high and significant fibrosis affect 12.7% of the patients. Fibroscan is an effective procedure to screen for fibrosis and steatosis in diabetic patients. [ABSTRACT FROM AUTHOR]