Jolanta Dadoniene

Arterial wall dysfunction in systemic lupus erythematosus

Carotid-radial pulse wave velocity (PWV), aortic augmentation index (AIx) and endothelium-dependent flow-mediated dilatation (FMD) have been repeatedly showed to be related to premature atherosclerosis and cardiovascular diseases in different settings of population. The increased arterial stiffness and endothelium dysfunction may add to premature aging of the arteries in systemic lupus erythematosus (SLE) patients. Still data about arterial stiffness and endothelium function in inflammatory rheumatic diseases are not well described. The aim of this study was to determine the PWV, its derivate marker AIx and FMD and factors possibly influencing them in young SLE women without significant organ damage. Thirty women between 23 and 55 years with an established SLE diagnosis and 66 healthy women were consequently included in the study and both groups were comparable according to age, body mass index (BMI), serum lipid profile and creatinine. PWV was determined by measuring carotid-radial pulse wave transit time with the help of applanation tonometry and AIx, its derivate marker, was calculated as a difference between two waveform peaks expressed as a percentage of the pulse pressure. The FMD was performed by obtaining the repeated scans of the brachial artery at rest and during reactive hyperemia. In SLE women, PWV and AIx were significantly higher and FMD was not different from controls. In linear multiple stepwise regression analysis if patients and controls were both considered, PWV was weakly related to mean blood pressure (MBP), AIx was mostly predicted by age and MBP and FMD was predicted by the diameter of blood vessel, BMI, high density lipoproteins. If the sole SLE setting was analyzed, PWV was not related to any of the pending parameters, AIx turned out to be related to organ damage measured by Systemic Lupus International collaborative Clinics (SLICC) index and age, and FMD obtained strong and significant relation with vessel diameter, and BMI, and disease duration. Regardless of the small number of study group patients, we can state that controlling for MBP and taking measures towards organ damage prevention can partially slow down the process of early atherosclerosis in SLE patients.

Tumour-induced osteomalacia: a literature review and a case report.

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.

The Prevalence of Systemic Lupus Erythematosus in Lithuania: The Lowest Rate in Northern Europe

The aim of this study was to explore the prevalence of systemic lupus erythematosus (SLE) in Lithuania (Vilnius). Two different studies were designed for SLE cases identification: registry-based SLE study and population-based SLE study. For the registry-based study patients were enrolled during the period of 1999-2004 and from two sources, including out-patient clinics of Vilnius and tertiary rheumatology center with interview during the year 2004. Only Vilnius residents who fulfilled the ACR 1982 revised criteria for the classification of SLE were counted in this study. Seventy-six living adult patients with SLE were interviewed and accounted for the prevalence of 16.2/100 000 (0.016%) using the Vilnius adult population in January 2004 (a population of 470 451). The population study of randomly selected 10 000 Vilnius inhabitants with beforehand validation of the survey was performed in the same year. The population-based study revealed two cases for 4017 respondents, but the low response rate may be important. Extrapolating the results to population of 10 000 inhabitants, the point prevalence of SLE in the entire sample was at least 0.02%. Therefore, the prevalence of SLE in Lithuania is the lowest if compared to Northern European countries.

Roadmap to vasculitis: a rheumatological treasure hunt

Abstract Vasculitis is characterized by inflammation of the wall of blood vessels. It involves immunologically mediated responses to usually unknown antigens, which result in vessel wall damage. Weakening of the vessel wall can lead to aneurysms, dissections or bleeding and narrowing of the lumen (caused by vasculitis per se and complicating thrombosis and embolization) resulting in ischemic damage and necrosis of the affected end organs and tissues. The first part of this four-part review describes the red flags and stop signs, which could help the busy doctor to stop and to start to think of the possibility of vasculitis. This is particularly important as many of these syndromes are life-threatening and hence their diagnostics can be compared to “a rheumatologic treasure hunt” as the treasured life of the patient is often at stake. Everything starts with simple measures, namely taking the patient history and conducting a complete physical examination. This is often enough for the identification of triggering factors as causes as well as targets of therapy in secondary vasculitides. They are often also enough for the right diagnosis, which only needs to be confirmed, perhaps by specialists, with more elaborate and expensive methodology.

Roadmap to vasculitis: a rheumatological treasure hunt: Part II. Classification, features of individual vasculitides and differential diagnosis against pseudovasculitis

Abstract Since the triggering factors causing primary vasculitides are by definition not (yet) known, we have to classify them to clinical syndromes based on the size, site, type and effect of the blood vessel involvement. ACR classification criteria and Chapel Hill nomenclature are useful tools to familiarize with the primary vasculitides, although a lot of criticism has been voiced in the literature indicating that they only represent the best available consensus. The present text takes advantage of the recent developments such as introduction of the anti-neutrophilic cytoplasmic auto (ANCA) antibodies, and divides the vasculitides to those affecting typically the large, medium and small arteries or only small blood vessels. In addition, some vasculitides, which are still difficult to place to the vasculitis map, like Burgers disease, Goodpastures syndrome, primary angiitin of the central nervous system (PACNS) and panniculitis, are dealt with. As it is a long and winding road, attention has to be paid to the clinical details to follow the road sign to “pseudovasculitis”, when that is the right way to go. They represent a bunch of non-vasculitic conditions, which lead to structural or vasospastic impairment of the blood flow, bleeding or thromboembolism and hyperviscosity. These imitators have to some extent, similar clinical symptoms and signs as well as laboratory and radiological findings to those found in true systemic vasculitides. This also emphasizes the importance of internal medicine as the intellectual (albeit not necessarily organizational) home of rheumatology and rheumatologists as we deal with conditions like atherosclerosis, antiphospholipid antibody syndrome, infectious endocarditic, myxoma of the heart and cholesterol embolism.

Vitamin D, cardiovascular and bone healthin postmenopausal women with metabolic syndrome

BACKGROUND The evidence highlights the importance of improving vitamin D levels in the general population for the prevention of adverse long-term health risks, including cardiovascular events, metabolic syndrome, cancer, anxiety and depression, and overall mortality, although controversies in the research are common. OBJECTIVES The purpose of this study was to investigate the relationship between vitamin D and vascular and bone health among postmenopausal metabolic women, controlling for traditional cardiovascular factors, and thus seeking to explore their plausible relation. The secondary aim was to look specifically for the relation between artery stiffness and bone health. MATERIAL AND METHODS This is a cross-sectional study designed to evaluate the relation between vitamin D level and vascular and bone health among women with metabolic syndrome. Two hundred and ten women visiting a cardiologist were recruited consecutively into the study. The study variables included clinical examination, laboratory findings, measurements of vascular stiffness, and bone turnover markers. RESULTS We found 126 (60%) metabolic women with a vitamin D deficiency (50 nmol/L) among the study group. We discovered no statistically significant correlation between vitamin D and vascular stiffness. Vitamin D was not associated neither with femoral neck bone mineral density (BMD) and T score, nor with lumbar spine BMD and T score. Nevertheless, there was an indirect weak correlation between vascular stiffness, in particular the augmentation index (AIx), and all bone health markers, including BMD and T score in both the femur head and lumbar spine. CONCLUSIONS We showed a high proportion of postmenopausal metabolic women with a vitamin D deficiency, but there was no relation between vitamin D and vascular health or vitamin D and bone health. Nevertheless, the relation between vascular health and bone health exists, although the role of vitamin D in this link has not yet been established.

Roadmap to vasculitis: a rheumatological treasure hunt: Part III. Laboratory evaluation and imaging

Abstract In the third part of this four part review, we already have the stop sign and our three road signs pointing to secondary vasculitides, pseudovasculitides and primary vasculitides behind our back and we have also passed the first milestone, where “patient history and physical examination” was written with large black block letters. GP can get far with simple blood, urine and stool tests and routine X-rays (second milestone). Almost all vasculitides of clinical significance are characterized by increased ESR and raised C-reactive protein levels and often also by normocytic normochromic anaemia, leucocytosis, eosinophilia and thrombocytosis. Urine test may demonstrate haematuria, proteinuria and cylindruria, X-ray of the paranasal cavities chronic sinusitis and chest X-ray shadowing and cavitations. Serological tests may disclose an unexpected hepatitis B or C or perhaps ANCA. The possibilities described form such a cornucopia that we need to have our patient history and physical examination right for the right picks. This is even more pertinent when we take to the sledgehammer in the referral centres (third milestone) and deal with the histopathology of vasculitides as hopefully seen in biopsies rather than autopsies or perform invasive radiology. High resolution colour Doppler ultrasound offers a useful, non-invasive method for the diagnosis and guidance of an eventual biopsy site in temporal arteritis and is helpful in the diagnosis of Takayasus arteritis and Kawasaki disease. Aortic arch, mesenteric, splanchnic or renal angiographies, MRI, contrast-enhanced CT, gadolinium-enhanced magnetic resonance angiography and positron emission tomography are dealt with but require the right patient and the right “doctor decision maker” not to cause harm and to avoid waste of scant resources.

Roadmap to vasculitis: a rheumatological treasure hunt: Part IV. Management of vasculitis

Abstract At the stop sign we read the “red flags” and made up our mind and followed one of the road signs pointing to secondary, primary or fake vasculitis. Since then we have steadily followed the road map and passed the first (patient history and physical exam), second and third milestones (laboratory, imaging and pathology studies in the primary care and specialized centres) and have finally reached our destination at the fourth milestone (Part IV) on the road map review to vasculitis. In the management of these syndromes, Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI) are not widely used in the routine clinical work, but they are introduced as the idea behind them is really valid. The backbone of the medical therapy is the use of immunosuppressive doses of prednisone (1 mg/kg/day). In some life-threatening and non-responsive vasculitides this is combined with cyclophosphamide 2–4 mg/kg/day or 0.5–1.0 g/m 2 i.v. every 2–4 weeks (European Vasculitis group uses 15 mg/kg every 2–3 weeks), often at 3–6 months substituted either with methotrexate or azathioprine. In contrast, i.v. immunoglobulins are to be used in Kawasakis syndrome; cyclosporine, dapsone or colchicine in Behcets disease; calcium channel blockers in BACNS; and NSAID in small vessel disease; whereas plasmapheresis or immunoadsorption are added to the therapy in Goodpastures syndrome. Particular attention is drawn to the treatment of the triggers, use of biologicals and new cytostatic drugs and anti-metabolites, prevention of thromboembolic complications with anti-platelet drugs as well as to odd and orphan entities. A short travelogue ends our odyssey as the last sign on our roadmap.