Correlation between salivary gland ultrasonography and scintigraphy in primary Sjögren's syndrome.

OBJECTIVE: To compare findings on salivary gland ultrasonography (SGUS) and salivary gland scintigraphy (SGS) in patients with primary SS (pSS). METHODS: The study cohort included patients newly diagnosed with pSS who underwent SGUS and SGS at the same time at our tertiary care hospital. Baseline demographics, laboratory data, clinical data and SGUS and SGS findings were collected. An SGUS cut-off score ≥14 defined positive SGUS findings and was used to classify patients in SGUS+ and SGUS- groups. SGS findings were quantified by the parotid:submandibular uptake ratio (PU:SU) and percentage parotid/submandibular excretion (%PE/%SE). The correlation between SGUS and SGS findings was evaluated. RESULTS: For analysis, 18 patients with SGUS+ findings and 18 with SGUS- findings were recruited, for a total study cohort of 36 patients. There were no between-group differences in baseline demographics and clinical and laboratory data. The PU, %PE, SU and %SE were significantly lower in the SGUS+vs SGUS- group. The SGUS score for the parotid gland was negatively correlated to the PU (r = -0.36, P = 0.03) and %PE (r = -0.35, P = 0.04). The SGUS score of the submandibular gland was negatively correlated to the SU (r = -0.42, P = 0.01) and %SE (r = -0.39, P = 0.02). CONCLUSIONS: Patients with a higher SGUS score had lower salivary gland function. The SGUS score showed a significant correlation with PU, %PE, SU and %SE. These findings are indicative of a possible predictive role of SGUS to diagnose salivary gland dysfunction.

Ultrasonographic evaluation of lacrimal glands in patients with primary Sjögren's syndrome.

OBJECTIVES: This study focused on distinguishing the characteristic ultrasonographic findings of lacrimal glands in primary Sjögren's syndrome (pSS) from those in idiopathic sicca syndrome. We aimed to set up a semi-quantitative scoring system of lacrimal gland ultrasonography (LGUS) for patients with pSS. METHODS: Fifty-six patients with pSS and 40 patients with idiopathic sicca syndrome were evaluated. Lacrimal glands were examined with ultrasonography using area, major/minor axis length, and five components (presence of intraglandular branch of lacrimal artery, inhomogeneity, hyperechoic bands, hypoechoic areas, and delineation). Except for the area and maximal/minimal length of lacrimal glands, other components were classified as dichotomous variables (present or absent). Using the receiver operating characteristics curve, we inferred the most appropriate combination of LGUS scoring for pSS diagnosis. RESULTS: Patients with pSS had a higher proportion of intraglandular branch of lacrimal artery (70.5% vs. 42.5%, p<0.001), inhomogeneity (72.3% vs. 46.3%, p<0.001), and hyperechoic bands (56.2% vs. 37.5%, p=0.016) than patients with idiopathic sicca syndrome. LGUS A, which represents the summation of one point assigned for the presence of intraglandular branch of lacrimal artery and one for inhomogeneity, was the most suitable diagnostic criterion (area under curve = 0.724, 95% confidence interval 0.620-0.828). If both sides have a score of 2, it results in a total of 4 points. With a cut-off value of 3 out of 4 points, LGUS A had 60.7% sensitivity, 71.1% specificity, 60.7% positive predictive value, and 72.5% negative predictive value. CONCLUSIONS: Semi-quantitative scoring of LGUS was useful when distinguishing patients with pSS from those with idiopathic sicca syndrome. The combination of intraglandular branch of lacrimal artery and inhomogeneity on both sides was most suitable for classifying pSS using LGUS.

Pregnancy-associated risk factors and incidence of systemic sclerosis in primiparous women: A nationwide population-based cohort study.

OBJECTIVES: To evaluate the pregnancy-related risk factors and incidence rate (IR) of systemic sclerosis (SSc) in primipara using the Health Insurance Review and Assessment database, covering all medical claims in South Korea. METHODS: From the database, 2,260,952 primipara aged 18-49 years from 2008 to 2018 were identified. The patients were followed up after their index delivery until December 2019. A Cox proportional hazard analysis was performed to identify the association of pregnancy-related factors with SSc development. RESULTS: The SSc IR was 0.62 cases per 100,000 patient-years. Primipara had a higher risk of developing SSc after 3 years postpartum than in the first 3 years of delivery (OR = 1.98, 95% CI: 1.36-2.78, p < .001). A multivariate analysis showed that older age (35-49 years) (HR = 2.14, 95% CI: 1.05-4.35, p = .037) and a Caesarean section (CS) (HR = 1.86, 95% CI: 1.10-3.15, p = .021) are risk factors for SSc. At 3 years postpartum, CS (HR = 2.97, 95% CI: 1.39-6.32, p = .005) and a female infant (HR = 2.28, 95% CI: 1.11-4.71, p = .026) were associated with SSc development. CONCLUSION: Having a CS, late childbirth, and a female infant are the risk factors for SSc in primipara. This study establishes the IR of SSc in primipara.

Reciprocal interaction between macrophage migration inhibitory factor and interleukin-8 in gout.

OBJECTIVES: Macrophage migration inhibitory factor (MIF) is a proinflammatory, chemotactic, and tissue destructive cytokine. This study determined monosodium urate crystal-induced MIF production and its interaction with interleukin (IL)-8 in gout. METHODS: Peripheral blood (PB), synovial fluid (SF), and clinical data were obtained from 98 patients with gout. SF and serum concentrations of MIF and IL-8 were measured using ELISA. SF monocytes and neutrophils were cultured with monosodium urate (MSU) crystals and the cytokine production was determined. The signalling pathways involved were determined using signal inhibitors. The interaction between MIF and IL-8 was investigated. RESULTS: SF MIF was higher in acute gout and that in serum was higher in patients with intercritical gout compared with controls. SF MIF was positively correlated with SF leukocyte and neutrophil counts and IL-8. The expression of MIF was similar in SF neutrophils and monocytes, while IL-8 was higher in monocytes. MSU crystals induced MIF production in monocytes and IL-8 production in neutrophils. This effect was decreased by inhibiting Fc-gamma receptor 1 and toll-like receptor 4. IL-8 increased MIF production in monocytes while MIF increased interleukin-8 production in neutrophils. CONCLUSIONS: MIF and IL-8 are highly produced in acute gout. MSU crystals induced MIF production in monocytes and IL-8 production in neutrophils with a reciprocal interaction between the two cytokines.

Clinical and diagnostic significance of serum immunoglobulin A rheumatoid factor in primary Sjogren's syndrome.

OBJECTIVES: The aim of this study was to investigate the diagnostic accuracy of rheumatoid factor (RF) isotype for the detection of primary Sjogren's syndrome (pSS) and evaluate the clinical and serological associations of immunoglobulin (Ig) A RF in patients with pSS. MATERIALS AND METHODS: RF levels were measured in 77 and 37 patients with pSS and idiopathic sicca symptoms, respectively, using ELISA and analysed with respect to clinical and laboratory disease characteristics. Receiver operating characteristic curves were used to determine and compare the diagnostic accuracy of IgA RF with other diagnostic tests. RESULTS: Serum levels of IgA RF were significantly higher in patients with pSS than in those with idiopathic sicca symptoms. IgA RF showed sensitivity, specificity, positive, and negative predictive values of 83.1, 78.4, 88.9, and 69.0%, respectively, for pSS diagnosis. IgA RF was associated with xerostomia, severe sialoscintigraphic grade, low unstimulated salivary flow rate (USFR), antinuclear antibody, high IgG and IgM/G RF levels, and low C3 levels in patients with pSS. IgA RF titres had positive correlations with sialoscintigraphic grade and IgG and IgG/M RF levels and had negative correlations with USFR and C3 levels. CONCLUSION: Our findings confirmed the potential of IgA RF to distinguish pSS from idiopathic sicca symptoms. The presence of IgA RF in patients with pSS was associated with significantly worse exocrine function and active serologic profile. No association between IgA RF and extra-glandular manifestations was noted. CLINICAL RELEVANCE: IgA RF should be the predictive and diagnostic marker in patients with pSS.

Diagnostic and predictive evaluation using salivary gland ultrasonography in primary Sjögren's syndrome.

OBJECTIVES: We aimed to assess the diagnostic accuracy of salivary gland ultrasonography (SGUS) as a single test for the detection of primary Sjögren's syndrome (pSS) and examine the prognostic factors for severe structural damage of the salivary glands based on SGUS score. METHODS: Patients with pSS (n=94) and idiopathic sicca syndrome (n=44) were evaluated using the SGUS 0-48 scoring system, which comprises five parameters: parenchymal echogenicity, homogeneity, hypoechoic areas, hyperechogenic reflections, and clearness of posterior borders. The salivary gland volume and intraglandular power Doppler signal (PDS) were also assessed. A multivariate linear regression analysis was performed to determine the factors associated with SGUS score. RESULTS: Patients with pSS showed a significantly higher SGUS score than controls [median (IQR): 24.5 (13.0) vs 6 (3.75), p<0.001]. An SGUS cut-off of ≥14 had a sensitivity of 80.9% and a specificity of 95.5% for the diagnosis of pSS. There were no significant differences in the measured volumes and PDS between pSS patients and controls. The SGUS score correlated with unstimulated salivary flow rate (USFR), serum rheumatoid factor and IgG. Double seropositivity with anti-Ro/SS-A and anti-La/SS-B (ß=6.060, p=0.001) and USFR (ß=-1.913, p<0.001) were independently associated with the SGUS score. CONCLUSIONS: The SGUS scoring system is a valuable diagnostic method for pSS. Double seropositivity of anti-Ro/SS-A and La/SS-B along with USFR were independent predictive factors for structural damage of the salivary glands.

The use of hand perfusion scintigraphy to assess Raynaud's phenomenon associated with hand-arm vibration syndrome.

OBJECTIVES: This study aimed to evaluate the hand perfusion scintigraphic features of hand-arm vibration syndrome (HAVS) and to compare these with the features of primary and secondary Raynaud's phenomenon (RP) associated with systemic sclerosis (SSc). METHODS: Hand perfusion scintigraphy was performed in 57 patients with primary RP, 71 patients with HAVS-related RP, and 15 patients with SSc-related RP. We calculated 6 ratios: chilled to ambient hand and wrist ratios of the first peak height, initial slope, and blood pool uptake. We analysed 3 morphologic characteristics: slow progress pattern, paradoxically increased uptake pattern, and inhomogeneous radioactivity uptake. RESULTS: All of the 71 patients with HAVS-related RP were mine workers. The chilled to ambient hand ratios of the first peak height, the initial slope, and the blood pool uptake were significantly lower in patients with HAVS-related occupational RP than in patients with primary RP. The presence of a paradoxically increased uptake pattern was significantly lower in HAVS than in primary RP. CONCLUSIONS: There were significant differences in hand perfusion scintigraphic features between primary RP and HAVS. These results suggest that the underlying pathophysiology of the two diseases differs.

Comparison of predictive value of FRAX, trabecular bone score, and bone mineral density for vertebral fractures in systemic sclerosis: A cross-sectional study.

Assessing fracture risk is important for managing patients with systemic sclerosis (SSc). Vertebral fracture (VF) is the most common fracture and is associated with future VF and non-VF. We aimed to evaluate the predictive value of FRAX, trabecular bone score (TBS), and bone mineral density (BMD) for VFs, compared to rheumatoid arthritis (RA) patients and postmenopausal women, and to identify risk factors for VFs in SSc. In this cross-sectional study, prevalent VFs, 10-year probability of major osteoporotic fracture by FRAX (FRAX-MOF), TBS, and BMD were assessed in women with SSc (n = 69) and RA (n = 58), and postmenopausal women (n = 38). Risk factors for osteoporosis, modified Rodnan total skin score (mRSS), organ involvement, and patterns of nailfold capillaroscopy (NFC) were also evaluated. The accuracy of BMD (T-score ≤ -2.5), TBS and FRAX-MOF, with and without TBS adjustment, to detect prevalent VF was assessed by determining the area under the receiver operating characteristic (ROC) curve. Patients with SSc (14.5%) and RA (17.2%) had significantly more VFs than postmenopausal women (0%) (P = .031). Non-significant differences were observed in TBS and BMD of all groups. The FRAX-MOF were higher in RA (9.2%) than SSc group (6.1%) and postmenopausal women (5.5%) (P < .001). Based on the ROC curve, TBS-adjusted FRAX-MOF (0.803) showed largest area under curve (AUC) to detect the prevalent VFs, followed by FRAX-MOF (0.796), TBS (0.765), and BMD (0.588) in the SSc group. In the RA group, FRAX-MOF had the largest AUC (0.896), followed by TBS-adjusted FRAX-MOF (0.863), TBS (0.736), and BMD (0.686). The cutoffs for FRAX-MOF and TBS-adjusted FRAX-MOF for detecting VFs were 8.95% and 9.7% for SSc, and 14.5% and 14% for RA. No association between VFs and SSc subtypes, organ involvement, mRSS or NFC patterns was found. FRAX-MOF, with or without TBS, had better predictive value for VFs than BMD and TBS in SSc. However, FRAX-MOF underestimated the probability of VFs in SSc compared with RA.

Clinical course and risk factors for development and progression of interstitial lung disease in primary Sjögren's syndrome.

This single-center, retrospective study aimed to investigate the course and prognostic factors of patients with primary Sjögren syndrome-associated interstitial lung disease (pSS-ILD). We included 120 pSS patients who underwent at least two high-resolution computed tomography (HRCT) scans between 2013 and 2021. Clinical symptoms, laboratory data, HRCT findings, and pulmonary function test results were collected. Two thoracic radiologists reviewed the HRCT findings. In patients with pSS without ILD at baseline (n = 81), no development of ILD was found on follow-up (median, 2.8 years). In patients with pSS-ILD (n = 39), total disease extent, extent of coarse reticulation, and traction bronchiectasis increased on HRCT, whereas the extent of ground glass opacity (GGO) decreased at follow-up (median, 3.2 years) (each p < 0.001). In progressive group of pSS-ILD (48.7%), the extent of coarse reticulation and coarseness score of fibrosis were increased at follow-up (p < 0.05). Usual interstitial pneumonia pattern on CT (OR, 15.237) and follow-up duration (OR, 1.403) were independent risk factors for disease progression in patients with pSS-ILD. In both progressive and non-progressive pSS-ILD, GGO decreased, whereas the extent of fibrosis increased even after treatment with glucocorticoid and/or immunosuppressants. In conclusion, progression occurred in approximately half of the pSS-ILD patients with slow gradual deterioration. Our study identified a definite group of progressive pSS-ILD who did not respond to current anti-inflammatory treatment.

Visual Scoring of Sacroiliac Joint/Sacrum Ratios of Single-Photon Emission Computed Tomography/Computed Tomography Images Affords High Sensitivity and Negative Predictive Value in Axial Spondyloarthritis.

Spondyloarthritis (SpA) is characterized by inflammatory back pain. Magnetic resonance imaging (MRI) was the earlier gold standard technique for detecting early inflammatory change. We reassessed the diagnostic utility of sacroiliac joint/sacrum (SIS) ratios of single-photon emission computed tomography/computed tomography (SPECT/CT) for identifying sacroiliitis. We aimed to investigate of SPECT/CT in diagnosing SpA using a rheumatologist's visual scoring of SIS ratios assessment. We conducted a single-center, medical records review study of patients with lower back pain who underwent bone SPECT/CT from August 2016 to April 2020. We employed semiquantitative visual bone scoring methods of SIS ratio. The uptake of each sacroiliac joint was compared to that of the sacrum (0-2). A score of 2 for the sacroiliac joint of either side was considered diagnostic of sacroiliitis. Of the 443 patients assessed, 40 had axial SpA (axSpA), 24 being radiographic axSpA and 16 being nonradiographic axSpA. The sensitivity, specificity, and positive and negative predictive values of SIS ratio of SPECT/CT for axSpA were 87.5%, 56.5%, 16.6%, and 97.8%, respectively. In receiver operating curve analysis, MRI better diagnosed axSpA than did SIS ratio of SPECT/CT. Although the diagnostic utility of SIS ratio of SPECT/CT was inferior to MRI, visual scoring of SPECT/CT affords high sensitivity and negative predictive value in axSpA. When MRI is inappropriate for certain patients, SIS ratio of SPECT/CT is an alternative tool for identifying axSpA in real practice.

Corrigendum: Computed tomography-based assessment of radiographic progression in spine and sacroiliac joints after pregnancy in women with radiographic axial spondyloarthritis.

[This corrects the article DOI: 10.3389/fmed.2022.970546.].

Clinical significance of quantitative bone SPECT/CT in the evaluation of hand and wrist pain in patients with rheumatic disease.

We investigated the diagnostic value of the maximum standardized uptake value (SUV) at hand and wrist joints for differentiating rheumatic diseases via bone single-photon emission computed tomography (SPECT)/computed tomography (CT). A total of 84 patients manifesting hand and wrist pain (58 women; age, 49.8 ± 15.4 years) were finally diagnosed with rheumatoid arthritis (RA, n = 42), osteoarthritis (OA, n = 16), fibromyalgia (FM, n = 2), and other rheumatic diseases (n = 24). The SUV of each patient was measured in 32 joints including the distal interphalangeal (DIP), proximal interphalangeal (PIP), metacarpophalangeal (MCP), and wrist joints bilaterally. Differences in pain and SUVs between specific rheumatic diseases were assessed using the chi-squared test or one-way analysis of variance. Using the highest SUV (hSUV) in each patient, the diagnostic performance in differentiating specific diseases was evaluated by receiver operating characteristic (ROC) curve analysis. Pain symptoms were present in 886 (33.0%) sites in a total of 2688 joints. In four joint groups (DIP, PIP, MCP, and wrist), the SUVs of joints with pain were significantly higher than those of pain-free joints (all P < 0.001). Active joint sites with higher SUVs than the median value of each joint group were the most common in RA (55.1%). RA showed the greatest hSUV in the PIP (3.0 ± 2.4), MCP (3.5 ± 3.4), and wrist (3.3 ± 1.9) joint groups. FM was characterized by the lowest hSUV of all joint groups. In ROC curve analysis, the cumulative hSUV of the PIP, MCP, and wrist joint groups showed good performance for evaluating RA (area under the curve (AUC), 0.668; P = 0.005). The summation of the hSUVs at all joint groups had an excellent predictive performance for FM (AUC, 0.878; P < 0.001). Consequently, the arthritic activity of the hand and wrist joints based on SUV differed according to specific rheumatic diseases. Quantitative SPECT/CT may provide objective information related to arthritic activity for differentiating specific rheumatic diseases.

Impact of age on the diagnostic performance of unstimulated salivary flow rates and salivary gland ultrasound for primary Sjögren's syndrome.

Background: Age-related changes and different patterns of salivary gland abnormalities according to age may affect the diagnostic performance of unstimulated salivary flow rate (USFR) and salivary gland ultrasound (SGUS) for primary Sjögren's syndrome (pSS). We aimed to evaluate the threshold and diagnostic performance of USFR and whether incorporating SGUS or replacing USFR with SGUS affects the performance of the ACR/EULAR criteria for pSS according to age. Materials and methods: This medical chart review study included patients with suspected pSS who completed evaluations for pSS. Patients were classified based on age at pSS evaluation: elderly (≥65 years), middle-aged (40-64), and young (< 40). The USFR's optimal thresholds were evaluated using the ROC curve. The diagnostic performances of the USFR and modified ACR/EULAR criteria were compared. Results: In total, 239 pSS patients and 92 patients with idiopathic sicca syndrome were included. The cut-off of USFR ≤ 0.1 mL/min was irrelevant to age, demonstrating the best sensitivity (44.3-53.0%) and specificity (74.1-90.9%). SGUS had a significantly better AUC than USFR in the young (p < 0.01) and middle-aged groups (p < 0.01). The middle-aged group demonstrated better diagnostic performance of the ACR/EULAR criteria incorporating SGUS (AUC 0.957) (p < 0.01) and criteria replacing USFR with SGUS (AUC 0.957) (p < 0.001) compared to the original criteria (AUC 0.916). In the young and elderly groups, adding SGUS to the ACR/EULAR criteria or replacing USFR with SGUS did not significantly increase the AUC. Conclusions: The thresholds of USFR ≤ 0.1 mL/min was optimal, irrespective of age. Using SGUS can improve diagnostic accuracy of ACR/EULAR criteria by supplementing the USFR, especially in middle-aged patients.

Computed tomography-based assessment of radiographic progression in spine and sacroiliac joints after pregnancy in women with radiographic axial spondyloarthritis.

Background: Mechanical stress are one of the pathogenesis of axial spondyloarthritis (axSpA). During pregnancy, the mechanical overload on the spine and pelvis increases due to gravid uterus. We aimed to investigate whether pregnancy affects radiographic progression in patients with radiographic axSpA (r-axSpA) based on computed tomography (CT) evaluations. Materials and methods: This retrospective study included women with r-axSpA aged 19-49 years who underwent at least two CT evaluations of the whole spine and/or sacroiliac joints (SIJs) at intervals of 2-4 years. To compare radiographic progression after delivery, we classified the patients into two groups: delivery group and controls. The delivery group was restricted to women who had the first CT ∼2 years before delivery and the second CT ∼2 years after delivery. The CT Syndesmophyte Score (CTSS) (0-522) and SIJ scores (0-40) were used to evaluate spinal syndesmophytes and erosion, joint space narrowing, and sclerosis of the SIJs. Results: A total of 21 women in the delivery group and 38 women in the control group were included. The median (Q1-Q3) CTSS at baseline in the delivery group and controls was 19 (16-23) and 20 (13.25-27.75), and the median progression was 1 (0-3) and 0 (0-1) during the median 2.9-year follow-up, respectively. The median (Q1-Q3) SIJ score at baseline in the delivery group and controls was 13 (8-22) and 11 (6-22), and the median progression was 1.5 (0-3) and 1 (0-2), respectively. Using cut-off 0.5, 52.9, and 61.9% of r-axSpA patients and 39.3 and 44.4% of controls showed progression of whole spine and SIJs, respectively. However, no difference in proportion of spinal and SIJ progression and absolute score changes per time point was observed between two groups. Moreover, the SIJ score changes were comparable according to the delivery method. Conclusion: Pregnancy and delivery do not affect the radiographic progression of the spine and SIJs in women with r-axSpA assessed by CT.

Characteristic alterations of gut microbiota in uncontrolled gout.

Microbiome research has been on the rise recently for a more in-depth understanding of gout. Meanwhile, there is a need to understand the gut microbiome related to uric acid-lowering drug resistance. In this study, 16S rRNA gene-based microbiota analysis was performed for a total of 65 stool samples from 17 healthy controls and 48 febuxostat-treated gout patients (including 28 controlled subjects with decreased uric acid levels and 20 uncontrolled subjects with non-reduced uric acid levels). Alpha diversity of bacterial community decreased in the healthy control, controlled, and uncontrolled groups. In the case of beta diversity, the bacterial community was significantly different among groups (healthy control, controlled, and uncontrolled groups). Taxonomic biomarker analysis revealed the increased population of g-Bifidobacterium in healthy controls and g-Prevotella in uncontrolled patients. PCR further confirmed this result at the species level. Additionally, functional metagenomics predictions led to the exploration of various functional biomarkers, including purine metabolism. The results of this study can serve as a basis for developing potential new strategies for diagnosing and treating gout from microbiome prospects.

Discriminative ability of trabecular bone score over bone mineral density for vertebral and fragility fracture in patients treated with long-term and low-dose glucocorticoid.

AIM: To evaluate the ability of the trabecular bone score (TBS) to discriminate vertebral fracture (VF) and fragility fracture (FF) in patients with chronic inflammatory rheumatic diseases on long-term and low-dose glucocorticoid (GC) treatment and those without exposure to GC. METHODS: This study assessed TBS and bone mineral density (BMD) in chronic GC users, defined as ≥2.5 mg/d of prednisone for >3 months (n = 89, mean age: 62.5 ± 11 years), and in controls (n = 59, mean age: 60.3 ± 9.6 years). Osteoporosis risk factors, radiographs of the thoracolumbar spine, non-VF history, osteoporosis drugs, and current/cumulative GC doses were collected. Patients were classified as high (TBS <1.23), intermediate (1.23-1.31), or low risk (>1.31), according to the fracture risk based on a recent meta-analysis. RESULTS: The mean current dose and duration of GC treatment were 3.9 ± 1.9 mg/d and 3.9 ± 4.2 years, respectively. The prevalence of VF was significantly higher in chronic GC users than in controls (20.2% vs 5.1%, P = .010), although the prevalence of non-VF was similar (11.2% vs 5.1%). The GC group had significantly lower L1-L4 TBS and femur total BMD than did the controls (all with P < .01) without significantly different lumbar BMD. TBS (<1.31) showed a higher sensitivity for patients with VF and FF (83.3% and 81.8%, respectively) than with densitometric osteoporosis in the GC group (61.1% and 59.1%, respectively). Using the receiver operating characteristic curve, TBS <1.31 showed better diagnostic accuracy than TBS <1.23 and BMD in chronic GC users. CONCLUSION: TBS is more sensitive than BMD in detecting VF and FF in chronic GC users, even at a lower dose.

High prevalence of salivary gland ultrasound abnormalities in systemic sclerosis.

OBJECTIVE: To evaluate salivary gland (SG) involvement in patients with systemic sclerosis (SSc) using SG ultrasound (SGUS). METHODS: Patients with SSc (n=62), primary Sjögren's syndrome (pSS) (n=59), and idiopathic Sicca syndrome (n=43) were evaluated using the outcome measures in rheumatology clinical trial (OMERACT) definitions of the SGUS scoring system. The hyperechogenic bands using the 0-3 scoring system, intraglandular power Doppler signal (PDS), and SG volumes were also assessed. RESULTS: The proportion of patients with OMERACT grades (≥2) among the four SGs was significantly higher in SSc (51.6%) and pSS (62.7%) groups than those in the idiopathic Sicca syndrome group (4.7%). Patients with SSc and pSS had significantly higher total fibrosis grades than controls. No difference in fibrosis grades was observed between SSc and pSS groups. The PDS scores of SGs were higher in the SSc group than in the idiopathic Sicca syndrome group. SG volumes did not differ between the groups. SSc patients with SGUS grades ≥2 had more anti-centromere antibodies (ACA) (65.6% vs. 30.0%) than individuals with grades 0-1. SSc patients with fibrosis grades ≥2 reported more Sicca symptoms than those with grades 0-1. Inhomogeneity and hyperechogenic bands within the SGs were not associated with organ involvement in SSc. CONCLUSIONS: More than half of patients with SSc, specifically with ACA, showed SG involvement. SG fibrosis was more prominent in SSc than in idiopathic Sicca syndrome and was associated with subjective Sicca symptoms. However, hyperechoic bands within the SGs are not features that can differentiate between SSc and pSS.

Immunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a multi-system fibroin-flammatory disorder that can involve any organ, including the salivary glands, pancreas, and biliary tree. Treatment of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is similar to that for IgG4-RD, but progression is irreversible in some cases. We present a case of IgG4-SC in which an immuno-suppressant induced marked clinical and radiologic improvement. CASE SUMMARY: A 63-year-old male presented with a prominent itching sensation and wholebody jaundice. He showed obstructive-pattern jaundice, an elevated IgG4 level, and infiltration of a large number of IgG4-positive cells in the ampulla of Vater. The imaging findings of intrahepatic duct (IHD) and common bile duct dilation, an elevated serum IgG4 level, and characteristic histological findings led to diagnosis of IgG4-SC that compatible with the 2019 ACR/EULAR classification criteria. We planned to treat the patient with high-dose glucocorticoid (GC), followed by cyclophosphamide pulse therapy. After treatment with high-dose GC and an immunosuppressant, imaging studies showed that IHD dilatation had completely resolved. CONCLUSION: Prompt diagnosis and appropriate treatment of IgG4-SC are important. Because there is a risk of relapse of IgG4-SC, the GC dose should be gradually reduced, and a maintenance immunosuppressant should be given.

Reliability and validity of the Korean version of the University of California-Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract instrument in patients with systemic sclerosis.

BACKGROUND/AIMS: Systemic sclerosis (SSc) is associated with a wide range of gastrointestinal (GI) changes. The University of California-Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract (UCLA SCTC GIT 2.0) instrument is a self-administered GI assessment instrument for patients with SSc. We developed a Korean version of the UCLA SCTC GIT 2.0 instrument and evaluated its reliability and internal consistency. METHODS: The participants were 37 Korean patients with SSc. Translation and cross-cultural adaptation of the UCLA SCTC GIT 2.0 were performed according to international standardized guidelines. We evaluated reproducibility by calculating the intraclass correlation coefficients and assessed the internal consistency of the Korean version of the UCLA SCTC GIT 2.0. We assessed its construct validity by evaluating its correlations with the Short Form Health Survey version 2 and EQ-5D scores by means of Spearman correlation analyses. RESULTS: Patients with SSc were mostly women (89.19%) with a mean age of 52.2 years, median disease duration of 24 months, and median modified Rodnan total skin score of 4. The median total GIT score on the UCLA SCTC GIT 2.0 was 0.3. The UCLA SCTC GIT 2.0 Korean version showed excellent internal consistency (Cronbach's α of total GIT score = 0.863). Most domains of the ULCA SCTC GIT 2.0 were correlated with those of the EuroQol (EQ)-5D score. CONCLUSION: The Korean version of the UCLA SCTC GIT 2.0 has acceptable internal consistency, reliability, and validity. Therefore, it can be used to assess GIT involvement in Korean patients with SSc.