In patients with generalized vasculitis, the withdrawal of cyclophosphamide and the substitution of azathioprine after remission did not increase the rate of relapse. Thus, the duration of exposure to cyclophosphamide may be safely reduced.
Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterised by severe hypophosphataemia and osteomalacia, with renal phosphate wasting that occurs in association with tumour. The epidemiology likewise aetiology is not known. The clinical presentation of TIO includes bone fractures, bone and muscular pains, and sometimes height and weight loss. TIO may be associated with mesenchymal tumours which may be benign or malignant in rare cases. Mesenchymal tumour itself may be related to fibroblast growth factor 23 (FGF23), which is responsible for hypophosphataemia and phosphaturia occurring in this paraneoplastic syndrome. Hypophosphataemia, phosphaturia and elevated alkaline phosphatase are the main laboratory readings that may lead to more precise investigations and better diagnosis. Finding the tumour can be a major diagnostic challenge and may involve total body magnetic resonance imaging, computed tomography and scintigraphy using radiolabelled somatostatin analogue. The treatment of choice for TIO is resection of a tumour with a wide margin to insure complete tumour removal, as recurrences of these tumours have been reported. We provide here an overview on the current available TIO case reports and review the best practices that may lead to earlier recognition of TIO and the subsequent treatment thereof, even though biochemical background and the long-term prognosis of the disease are not well understood. This review also includes a 4-year-long history of a patient that featured muscular pains, weakness and multiple stress fractures localised in the hips and vertebra with subsequent recovery after tumour resection. Because the occurrence of such a condition is rare, it may take years to correctly diagnose the disease, as is reported in this case report.
Systemic sclerosis (SSc) is characterized by thickening and fibrosis of skin and internal organs that is associated with vascular damage. SSc may lead to arterial dysfunction and premature aging of the arteries. However, its relationship with parameters of arterial wall dysfunction has not been fully explored. To determine if carotid-radial pulse wave velocity (PWV), aortic augmentation index (AIx) and endothelial function are altered in SSc patients, 17 consecutive patients with SSc and 34 age- and gender-matched controls were included in our study. PWV and AIx were assessed non-invasively by applanation tonometry. The endothelium-dependent flow-mediated dilatation (FMD) test in a brachial artery was performed by the ultrasound system. The blood investigations included serum lipid profile, glucose, and high-sensitivity CRP (hsCRP) measurements. As compared to controls, SSc patients had significantly higher medians of the AIx (p = 0.002) and the PWV (p = 0.04) and the median of the FMD was significantly lower (p = 0.001). Stepwise linear regression including comorbid factors showed that SSc was a significant independent predictor of all arterial wall parameters measures. SSc patients have increased AIx and PWV and lower FMD as compared to control subjects. The relationship between SSc and measures of arterial wall parameters still remains unclear. Though replication of the results presented here is required, we conclude that SSc has a great impact on large and conduit arteries damage.
MS duration has a moderate correlation with RA disease activity. Assessment of MS duration may be clinically helpful in patients with low RAPID3 scores.
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