Alojzija Hočevar

Is salivary gland ultrasonography a useful tool in Sjögren’s syndrome? A systematic review

OBJECTIVE Ultrasonography (US) is a sensitive tool in the diagnosis of major salivary gland abnormalities in primary Sjögrens syndrome (pSS). The aim of this systematic review was to assess the metric properties of this technique. METHODS PUBMED and EMBASE databases were searched. All publications between January 1988 and January 2013 were considered. Data were extracted from the articles meeting the inclusion criteria according to US definition of salivary gland scoring system and metric properties studied. The type and number of glands tested, study design and metric properties according to OMERACT filter (truth, discrimination, feasibility) were assessed. RESULTS Of 167 publications identified initially with PUBMED and EMBASE, 31 met the inclusion criteria. The number of pSS patients varied among the studies from 16 to 140. The diagnosis of pSS was in line in most of the cases with the American-European Consensus Group (AECG) classification criteria for Sjögrens syndrome. The US examination was performed in suspected pSS only in studies in which the sensitivity ranged from 45.8 to 91.6% and specificity from 73 to 98.1%. There was heterogeneity in regard to the definition of US in B-mode and few studies used US in colour Doppler. Few studies reported reliability of US and sensitivity to change in pSS. CONCLUSION US is a valuable tool for detecting salivary gland abnormalities in pSS. Its reliability has been poorly investigated and there is considerable variation in the definition of US abnormalities. Further studies are required to validate and standardize the US definition of salivary gland in pSS.

Parasympathetic nervous system dysfunction in primary Sjögren’s syndrome

In the past sicca syndromes were attributed to destruction of glandular tissue. It is now thought that cytokines, autoantibodies, and parasympathetic nervous system dysfunction all have an important role in the xerostomia and xerophthalmia in Sjögren’s syndrome.

Salivary gland ultrasound abnormalities in primary Sjögren’s syndrome: consensual US-SG core items definition and reliability

Objectives Ultrasonography (US) is sensitive for detecting echostructural abnormalities of the major salivary glands (SGs) in primary Sjögren’s syndrome (pSS). Our objectives were to define selected US-SG echostructural abnormalities in pSS, set up a preliminary atlas of these definitions and evaluate the consensual definitions reliability in both static and acquisition US-SG images. Methods International experts in SG US in pSS participated in consensus meetings to select and define echostructural abnormalities in pSS. The US reliability of detecting these abnormalities was assessed using a two-step method. First 12 experts used a web-based standardised form to evaluate 60 static US-SG images. Intra observer and interobserver reliabilities were expressed in κ values. Second, five experts, who participated all throughout the study, evaluated US-SG acquisition interobserver reliability in pSS patients. Results Parotid glands (PGs) and submandibular glands (SMGs) intra observer US reliability on static images was substantial (κ > 0.60) for the two main reliable items (echogenicity and homogeneity) and for the advised pSS diagnosis. PG inter observer reliability was substantial for homogeneity. SMGs interobserver reliability was moderate for homogeneity (κ = 0.46) and fair for echogenicity (κ = 0.38). On acquisition images, PGs interobserver reliability was substantial (κ = 0.62) for echogenicity and moderate (κ = 0.52) for homogeneity. The advised pSS diagnosis reliability was substantial (κ = 0.66). SMGs interobserver reliability was fair (0.20< κ ≤ 0.40) for echogenicity and homogeneity and either slight or poor for all other US core items. Conclusion This work identified two most reliable US-SG items (echogenicity and homogeneity) to be used by US-SG trained experts. US-PG interobserver reliability result for echogenicity is in line with diagnosis of pSS.

Subcutaneous infection with Pseudallescheria boydii in an immunocompromised patient

With the broad employment of immunosuppressive therapy, the incidence of Pseudallescheria boydii infections is rising. We report a first case of the localized subcutaneous P. boydii infection in a patient with microscopic polyangiitis. Favorable outcome related to the treatment with voriconazole adds to the growing body of evidence supporting the use of this particular agent in P. boydii infections.

The role of colour doppler ultrasonography of facial and occipital arteries in patients with giant cell arteritis: A prospective study

OBJECTIVE Colour Doppler Sonography (CDS) in giant cell arteritis (GCA) allows the study of involvement of cranial arteries other than the temporal arteries, which are inconvenient to biopsy, such as the facial (FaA), and occipital (OcA) arteries. We aimed to estimate the frequency of the FaA, and OcA involvement in GCA; and to explore the clinical characteristics of these subgroups of patients. METHODS From 1 January 2014 to 31 December 2016 we prospectively performed a CDS of the FaA, and OcA in addition to the temporal (TA), and the extracranial supra-aortic arteries in all newly diagnosed patients suspected of having GCA. All the arteries were evaluated in two planes for the highly specific halo sign. RESULTS During the 36-month observation period we performed a CDS of the cranial and extra-cranial arteries in 93 GCA patients. We observed the halo sign on the FaA, and OcA in 38 (40.9%), and 29 (31.2%) cases, respectively. The FaA, or OcA were affected in 4/22 (18.2%) patients with a negative TA CDS. FaA involvement significantly correlated with jaw claudication and with severe visual manifestations, including permanent visual loss. CONCLUSIONS A fifth of patients with a negative CDS of the TAs had signs of vasculitis on the CDS of the FaA, or OcA. The addition of FaA and OcA CDS to the routine CDS of the TAs could identify 4.3% more patients and thus further improve the sensitivity of the CDS in the suspected GCA.

Do Early Diagnosis and Glucocorticoid Treatment Decrease the Risk of Permanent Visual Loss and Early Relapses in Giant Cell Arteritis: A Prospective Longitudinal Study

AbstractTo determine the incidence of permanent visual loss (PVL) in giant cell arteritis (GCA) and the GCA relapse rate during glucocorticoid (GC) tapering.This prospective, longitudinal single secondary/tertiary rheumatology centre study was conducted between September 2011 and September 2014 in Slovenia. Predetermined clinical and laboratory tests were performed at 12, 24, 48, 96, and 144 weeks after diagnosis.Sixty-eight GCA patients (72.1% female), with a median (IQR) age of 73.2 (67.3–76.1) years and a symptom duration before the diagnosis of a median (IQR) 30 (14–70) days were included. Thirty-nine of 68 patients had symptoms for less than 31 days (14 (10–28) days–early GCA) and 29/68 for 31 days or longer (90 (60–120) days–late GCA). Four (5.9%) patients presented with PVL (1 early GCA). The median (IQR) follow-up was (IQR) 104 (53–126) weeks. GCA relapsed in 17/39 (43.6%) and 14/29 (48.3%) in early and late GCA, respectively. The median (IQR) time to the first relapse was 24.8 (13.6–46.5) weeks (early GCA 14 (13–34) weeks; late GCA 25 (22–48) weeks, P = 0.117), at the methyl-prednisolone dose of 6.0 (4.0–12.0) mg. The patients who relapsed had significantly higher levels of inflammation parameters at the baseline (including ESR, CRP, serum amyloid A, haptoglobin, and fibrinogen).An early GCA diagnosis and prompt GC treatment decreased the PVL rate in comparison to historic controls, but seem to have no impact on the frequency of relapses, which are predicted by the high baseline levels of the biomarkers of inflammation.

AA amyloidosis in a polyarteritis nodosa patient treated with tocilizumab.

Abstract Amyloid A (AA) (secondary) amyloidosis represents a severe complication of chronic inflammatory diseases. Since pathogenic mechanisms point to the central role of interleukin 6 (IL-6) in the process of amyloid AA generation, IL-6 blockade seems an attractive therapeutic option. We report a case of a patient with polyarteritis nodosa complicated by AA amyloidosis treated with tocilizumab.

Definitions and reliability assessment of elementary ultrasound lesions in giant cell arteritis: a study from the OMERACT Large Vessel Vasculitis Ultrasound Working Group

Objectives To define the elementary ultrasound (US) lesions in giant cell arteritis (GCA) and to evaluate the reliability of the assessment of US lesions according to these definitions in a web-based reliability exercise. Methods Potential definitions of normal and abnormal US findings of temporal and extracranial large arteries were retrieved by a systematic literature review. As a subsequent step, a structured Delphi exercise was conducted involving an expert panel of the Outcome Measures in Rheumatology (OMERACT) US Large Vessel Vasculitis Group to agree definitions of normal US appearance and key elementary US lesions of vasculitis of temporal and extracranial large arteries. The reliability of these definitions on normal and abnormal blood vessels was tested on 150 still images and videos in a web-based reliability exercise. Results Twenty-four experts participated in both Delphi rounds. From originally 25 statements, nine definitions were obtained for normal appearance, vasculitis and arteriosclerosis of cranial and extracranial vessels. The ‘halo’ and ‘compression’ signs were the key US lesions in GCA. The reliability of the definitions for normal temporal and axillary arteries, the ‘halo’ sign and the ‘compression’ sign was excellent with inter-rater agreements of 91–99% and mean kappa values of 0.83–0.98 for both inter-rater and intra-rater reliabilities of all 25 experts. Conclusions The ‘halo’ and the ‘compression’ signs are regarded as the most important US abnormalities for GCA. The inter-rater and intra-rater agreement of the new OMERACT definitions for US lesions in GCA was excellent.

A Model Predicting Short Term Severity of IgA Vasculitis in Adults

Background: Predictors of short term severity of adult immunoglobulin A vasculitis (IgAV) are unknown. We aimed to determine clinical features predicting the severity of acute adult IgAV and thus to aid the management of adult IgAV in daily practice. Methods: Medical records of adult, histologically proven IgAV cases, diagnosed between 01.01.2010 and 30.06.2016 at our secondary/tertiary rheumatology centre were reviewed. The disease activity was assessed using Birmingham vasculitis activity score-3. Renal disease was defined severe when nephrotic syndrome or nephritic syndrome with acute renal failure developed. Gastrointestinal (GI) disease was severe in case of bloody diarrhoea, ileus or bowel perforation. Results: During the 78-month observation period, we identified 184 new IgAV cases (57.1% male; 43.5% ever smokers). Skin, GI, renal and joint involvement were present in 184 (generalized purpura above the waist in 47.8%), 63 (severe in 16), 88 (severe in 23), and 81 patients, respectively. Four patients died during acute disease due to vasculitis. Patients with generalized skin lesions had more commonly GI tract, severe GI tract, and kidney involvement. Current smoking was strongly associated with severity of kidney disease. In logistic regression and classification tree model the history of new onset abdominal pain or the presence of generalized purpura in ever smoker emerged as the best predictors of severe GI or renal disease. Conclusions: Based on clinical characteristics only, the presence of abdominal pain, purpura above the waist and smoking history, seem to be good predictors of short term severity of adult IgAV.

A concise review of significantly modified serological biomarkers in giant cell arteritis, as detected by different methods

Giant cell arteritis (GCA) is a primary systemic vasculitis present in subjects older than 50years with involvement of large- and medium-sized arteries. Early diagnosis for GCA is essential to prevent serious complications, such as permanent vision loss and/or cerebrovascular events. Elevated inflammatory cytokines, with acute phase and other proteins dominate large- and medium-sized arteries leading to stenosis or occlusion of arterial lumen. To date, there are no reliable serological markers for monitoring GCA. The review aims to provide concise overview of published GCA studies in order to: a) identify significantly changed serological biomarkers in GCA and compare the influences of techniques for marker evaluation and b) investigate most promising markers in GCA using analyte frequency and meta-analysis.