Alojzija Hočevar

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Full Papers; Authors: A. Hočevar, A. Grošelj, G. Hawlina, M. Koželj, A. Škoberne, J. Pižem, V. Jurčić

Background: Data on the incidence of IgG4-related disease (IgG4-RD) are scarce. Our aim was to determine the incidence of IgG4-RD in a well-defined region. Methods: This retrospective study covered the Ljubljana region over the period from January 2012 to December 2024. A review of cases diagnosed with IgG4-RD was performed at several departments of the University Medical Centre Ljubljana—an integrated secondary/tertiary university teaching hospital (rheumatology, nephrology, angiology, gastroenterology, abdominal surgery, ENT surgery, ophthalmology). While IgG4-RD cases at the Department of Rheumatology were collected prospectively, potential cases at other departments were retrieved by searching electronic medical database for the keyword “IgG4”. In addition, the Institute of Pathology, Faculty of Medicine, University of Ljubljana, provided a list of patients with histological features consistent with IgG4-RD. Year-specific incidence rates and an average incidence rate over the 13-year period were determined. Clinical features of patients were analysed. Results: During the observation period, 58 cases of IgG4-RD were diagnosed. Of these, 35 patients were residents of the Ljubljana region, which had an average adult population of 541,600. The estimated average annual incidence rate of IgG4-RD was 5.0 per million (95% confidence interval: 3.5; 6.9), with year-specific incidence rates fluctuating between 1.8 and 9.3 per million adults. The cases were stratified into four phenotypic categories: pancreato-hepato-biliary (17%), retroperitoneal fibrosis-aortitis (43%), head and neck-limited (14%), and Mikulicz syndrome with systemic involvement (26%). Conclusions: The average annual incidence rate of IgG4-RD was 5 per million adults, with the retroperitoneal fibrosis-aortitis phenotype predominating in our cohort.

Letters to the Editor; Authors: A. Hočevar, A. Grošelj, G. Hawlina, M. Koželj, A. Škoberne, J. Pižem, V. Jurčić

Objectives: Cancer has been reported as a potential trigger for IgA vasculitis (IgAV) in adults; however, data on this topic are scarce. The aim of our study was to examine the frequency and location of cancer in adults with IgAV.Methods: We included 295 IgAV patients diagnosed between January 2010 and June 2021 and followed at our secondary/tertiary rheumatology centre. Cancer episodes were recorded and classified into three groups according to their temporal relation to IgAV: (1) cancer diagnosed prior to IgAV; (2) cancer diagnosed concurrently with IgAV; and (3) cancer diagnosed during IgAV follow-up. The term IgAV associated with cancer (IgAV-CA) was used for IgAV cases presenting while cancer was active or diagnosed within a three-year period before or after the IgAV diagnosis.Results: Forty-seven IgAV patients (15.9%) developed a total of 56 different cancers. Of these, 43 cancers (76.8%) preceded the IgAV diagnosis, 3 cancers (5.3%) were diagnosed concurrently with IgAV, and 10 cancers (17.9%) were diagnosed during the IgAV follow-up. Twenty-two IgAV patients (7.5%) who developed a total of 24 cancers (42.9%) could be classified as IgAV-CA cases. The three most frequently diagnosed cancers in the IgAV-CA group were prostatic, renal and bladder cancer, together accounting for 50% of cancers. Age and smoking were associated with concurrent cancers, and arthritis represented a risk factor for cancer development during IgAV follow-up.Conclusion: IgAV cases associated with cancer represent 7% of adult IgAV cases. In elderly patients, careful examination of the urogenital tract is warranted when cancer is suspected.

Objective: Ultrasonography is crucial for diagnosing giant cell arteritis (GCA); however, training opportunities are rare. This study tested the reliability of ultrasonography findings and measurements of the intima-media thickness (IMT) among ultrasonography experts by using phantoms of the axillary (AA) and temporal arteries (TA) created with high-resolution 3D printing. Methods: Twenty-eight participants from 12 European countries received eight sets of phantoms of the AA and the superficial TA (including common, frontal and parietal branches), which were examined in a blinded fashion according to a predefined protocol and evaluated based on Outcome Measures in Rheumatology (OMERACT) GCA ultrasound definitions. Due to difficulties with the delineation of the intima-media complex, the parietal branch of the phantoms was modified, and a second round was conducted. The IMT was measured, and phantoms were classified as normal or vasculitic. Results: In both rounds, the phantoms were correctly classified as normal/abnormal in >81% of cases yielding a Fleiss’ kappa of 0.80 (95% CI 0.78 to 0.81) in round 1 and 0.74 (95% CI 0.72 to 0.75) in round 2. IMT measurements revealed an intraclass correlation coefficient (ICC 1.1) of 0.98 (95% CI 0.98 to 0.99) in both rounds. Intrarater reliability was good with a median Cohens Kappa of 0.83 and median ICC of 0.78. Conclusion: The study demonstrated high reliability among ultrasound experts in applying the OMERACT ultrasound definitions for GCA and in measuring the IMT using a 3D-printed phantom of the AA and TA. This phantom could assist clinicians in training to assess the large arteries of patients with suspected or established GCA.

Objective To test the reliability of the Outcome Measures in Rheumatology Giant cell arteritis (GCA) Ultrasonography Score (OGUS) and other composite scores in a patient-based exercise involving experts and non-experts in vascular ultrasonography. Methods Six GCA patients were scanned twice (two rounds separated ≥3 hours) by 12 experts and 12 non-experts. Non-experts received 90 min of theoretical and 240 min of practical training between rounds 1 and 2. Ultrasonography was conducted on temporal arteries (common superficial, frontal and parietal branches) and axillary arteries bilaterally to calculate the OGUS, the Southend score and the Halo count. Inter-reader and intra-reader reliability were assessed by intraclass correlation coefficient (ICC). Results Mean age of GCA patients was 78±5.1 years, 2 (33.3%) were women, and all were in clinical remission. Expert inter-reader ICC of the OGUS was 0.60 in both rounds, 0.40 in round 1 and 0.51 in round 2 for the Southend score and 0.45 and 0.52, respectively, for the Halo count. Median ICCs for intra-reader reliability were 0.86, 0.73 and 0.65 for the OGUS, Southend score and Halo count, respectively. For non-experts, inter-reader ICCs in round 1 were 0.20 for the OGUS, 0.20 for a normalised Southend score (=score divided by available segments) and 0.35 for a normalised Halo count. After training, inter-reader reliability ICCs improved to 0.52, 0.29 and 0.54, respectively. Conclusion Inter-reader reliability was fair to moderate, and intra-reader reliability was good for OGUS, Southend score and Halo count among experts. Inter-reader reliability of non-experts in vascular ultrasonography improved after the training.