| 121. |
- Sjöwall, Christopher, et al.
(author)
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Epidemiology of hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis).
- 2018
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In: Rheumatology. - : oxford academic. - 1462-0324 .- 1462-0332. ; 57:8, s. 1400-1407
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Journal article (peer-reviewed)abstract
- Objectives: The aim was to describe the clinical characteristics and epidemiology of hypocomplementaemic urticarial vasculitis (HUV; anti-C1q vasculitis) in two geographically defined areas of Sweden.Methods: In the health-care districts surrounding Skåne University Hospital (mean population 950 560) and Linköping University Hospital (mean population 428 503), all incident cases of HUV residing within the study areas at the onset of disease were identified during the years 2000-15. The diagnosis of HUV was confirmed by review of medical records. Only patients meeting the proposed diagnostic HUV criteria and/or the 2012 Chapel Hill consensus definitions in combination with an ever-positive anti-C1q antibody test were included.Results: Sixteen patients (14 females) were identified during the study period. The median (interquartile range) age at diagnosis was 51 (40.7-56.7) years. Median (interquartile range) time of follow-up from diagnosis to 31 December 2015, or death, was 94 (46.5-136.2) months. The most frequent manifestations at diagnosis were urticaria (100%), arthritis (88%), followed by biopsy-proven glomerulonephritis (19%), episcleritis/scleritis (19%) and recurrent abdominal pain (13%). The annual incidence rate per million inhabitants was estimated as 0.7 (95% CI: 0.4, 1.1). Sixty-three per cent suffered from pulmonary disease at the last follow-up. Two patients died during the follow-up period. One patient underwent lung transplantation, and two patients proceeded to end-stage renal disease. The point prevalence on 31 December 2015 was 9.5/million (95% CI: 4.5, 14.5).Conclusion: Hypocomplementaemic urticarial vasculitis constitutes a rare, but not always benign condition. Renal and lung manifestations were severe in some cases, highlighting the need for careful screening and monitoring of this potentially serious condition.
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| 122. |
- Sjöwall, Christopher, et al.
(author)
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HUVS [Hypocomplementemic Urticarial Vasculitis Syndrome] : sällsynt men inte ofarlig vaskulit associerad med urtikaria [A Rare but Not Always Benign Condition]]
- 2019
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In: Läkartidningen. - : Läkartidningen Förlag AB. - 0023-7205 .- 1652-7518. ; 116
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Journal article (peer-reviewed)abstract
- Although more than 45 years have passed since hypocomplementemic urticarial vasculitis (HUVS) was first described by McDuffie and colleagues at the Mayo clinic, data on epidemiology, disease outcomes, prognosis and clinical features are scarce. Recently, we published the first epidemiological study of HUVS including data on incidence, prevalence, disease outcomes, prognosis and clinical features using data from two separate Swedish regions during a period of 16 years. The estimation of incidence and prevalence rates indicates that HUVS is rare but not always benign. Renal and lung manifestations were severe in some cases, highlighting the need for careful screening and monitoring of this potentially serious condition. It is reasonable to suspect HUVS in patients with unexplained systemic inflammation combined with >6 months of urticaria. Special attention should be paid to patients with recent-onset dyspnea and proteinuria.
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| 123. |
- Sjöwall, Christopher, 1975-
(author)
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Reumatiska systemsjukdomar
- 2018. - 6
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In: Internmedicin. - Stockholm : Liber. - 9789147113262 ; , s. 920-939
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Book chapter (other academic/artistic)
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| 124. |
- Sjöwall, Christopher, et al.
(author)
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Serum levels of autoantibodies against C-reactive protein correlate with renal disease activity and response to therapy in lupus nephritis
- 2009
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In: Arthritis Research & Therapy. - : Springer Science and Business Media LLC. - 1478-6362 .- 1478-6354. ; 11:6, s. R188-
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Journal article (peer-reviewed)abstract
- Introduction Serum levels of C-reactive protein (CRP) seldom reflect disease activity in systemic lupus erythematosus (SLE). We have previously shown that autoantibodies against neo-epitopes of CRP often occur in SLE, but that this does not explain the modest CRP response seen in flares. However, we have repeatedly found that anti-CRP levels parallel lupus disease activity, with highest levels in patients with renal involvement; thus, we aimed to study anti-CRP in a material of well-characterized lupus nephritis patients. Methods Thirty-eight patients with lupus nephritis were included. Treatment with corticosteroids combined with cyclophosphamide, mycophenolate mofetil or rituximab was started after baseline kidney biopsy. A second biopsy was taken after ≥ 6 months. Serum creatinine, cystatin C, complement, anti-dsDNA, anti-CRP and urinalysis were done on both occasions. Biopsies were evaluated regarding World Health Organisation (WHO) class and indices of activity and chronicity. Renal disease activity was estimated using the British Isles Lupus Assessment Group (BILAG) index. Results At baseline, 34/38 patients had renal BILAG-A; 4/38 had BILAG-B. Baseline biopsies showed WHO class III (n = 8), IV (n = 19), III to IV/V (n = 3) or V (n = 8) nephritis. Seventeen out of 38 patients were anti-CRP-positive at baseline, and six at follow-up. Overall, anti-CRP levels had dropped at follow-up (P < 0.0001) and anti-CRP levels correlated with renal BILAG (r = 0.29, P = 0.012). A positive anti-CRP test at baseline was superior to anti-dsDNA and C1q in predicting poor response to therapy as judged by renal BILAG. Baseline anti-CRP levels correlated with renal biopsy activity (r = 0.33, P = 0.045), but not with chronicity index. Anti-CRP levels were positively correlated with anti-dsDNA (fluorescence-enhanced immunoassay: r = 0.63, P = 0.0003; Crithidia luciliae immunofluorescence microscopy test: r = 0.44, P < 0.0001), and inversely with C3 (r = 0.35, P = 0.007) and C4 (r = 0.29, P = 0.02), but not with C1q (r = 0.14, P = 0.24). No associations with urinary components, creatinine, cystatin C or the glomerular filtration rate were found. Conclusions In the present study, we demonstrate a statistically significant correlation between anti-CRP levels and histopathological activity in lupus nephritis, whereas a baseline positive anti-CRP test predicted poor response to therapy. Our data also confirm previous findings of associations between anti-CRP and disease activity. This indicates that anti-CRP could be helpful to assess disease activity and response to therapy in SLE nephritis, and highlights the hypothesis of a pathogenetic role for anti-CRP antibodies in lupus nephritis.
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| 125. |
- Sjöwall, Christopher, et al.
(author)
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Soluble urokinase plasminogen activator receptor levels are associated with severity of fibrosis in nonalcoholic fatty liver disease
- 2015
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In: Translational Research. - : Elsevier. - 1931-5244 .- 1878-1810. ; 165:6, s. 658-666
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Journal article (peer-reviewed)abstract
- The identification of individuals with severe liver fibrosis among patients with chronic liver disease is of major importance when evaluating prognosis, potential risk for complications, and when deciding treatment strategies. Although percutaneous liver biopsy is still considered a "gold standard" for staging of liver fibrosis, attempts to find reliable noninvasive markers of liver fibrosis are frequent. Inflammation is essential for the progression of fibrosis. The urokinase plasminogen activator and its receptor have been associated with hepatic inflammation and fibrosis in mice. High serum concentrations of soluble urokinase plasminogen activator receptor (suPAR) are suggested to be involved in inflammation, tissue remodeling, and cancer metastasis. Here, we evaluated serum suPAR as a noninvasive test to detect liver fibrosis in 82 well-characterized patients with nonalcoholic fatty liver disease (NAFLD), and in 38 untreated patients with chronic hepatitis C virus (HCV) infection at the time of their first liver biopsy. suPAR levels were increased in chronic liver disease compared with blood donors (P < 0.001). Patients with HCV had higher suPAR concentrations than patients with NAFLD (P < 0.002). suPAR levels were associated with the severity of fibrosis, particularly in NAFLD, but did not correlate with inflammation. Regarding the performance in predicting severity of fibrosis, suPAR was essentially as good as other commonly used noninvasive fibrosis scoring systems. The results in HCV confirm previous observations. However, this is the first study to investigate suPAR as a biomarker in NAFLD, and the results indicate that suPAR may constitute a severity marker related to fibrosis and prognosis rather than reflecting inflammation.
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| 126. |
- Sjöwall, Christopher, et al.
(author)
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Successful treatment of refractory systemic lupus erythematosus using proteasome inhibitor bortezomib followed by belimumab: description of two cases
- 2017
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In: Lupus. - : SAGE PUBLICATIONS LTD. - 0961-2033 .- 1477-0962. ; 26:12, s. 1333-1338
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Journal article (peer-reviewed)abstract
- Although the putative therapeutic options for patients with systemic lupus erythematosus (SLE) are steadily increasing, refractory disease is indeed a major challenge to many clinicians and patients. The proteasome inhibitor bortezomib - approved for the treatment of multiple myeloma since the beginning of this century - was recently reported successful in twelve cases of refractory SLE by German colleagues. Herein, we describe two Swedish SLE cases with refractory renal and pulmonary manifestations that were rescued by bortezomib as induction of remission followed by monthly doses of belimumab. The patients were carefully monitored with regard to disease activity and renal function. Anti-dsDNA and anti-C1q antibodies, complement proteins and lymphocyte subsets were analysed in consecutive samples. In December 2016, the patients had been in clinical remission post bortezomib administration for a period of 28 and 22 months, respectively. Potential benefits of using belimumab as maintenance therapy to prevent regeneration of autoreactive B cell clones are discussed.
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| 127. |
- Sjöwall, Christopher, et al.
(author)
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Two-Parametric Immunological Score Development for Assessing Renal Involvement and Disease Activity in Systemic Lupus Erythematosus
- 2018
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In: Journal of Immunology Research. - : HINDAWI LTD. - 2314-8861 .- 2314-7156.
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Journal article (peer-reviewed)abstract
- Objective. Anti-double-stranded (ds) DNA and anti-C1q autoantibodies are useful tools in the assessment of disease activity and nephritis in systemic lupus erythematosus (SLE) patients. This study aimed to explore the utility of these antibodies along with anti-Ku antibodies in an oligoparametric model approach for the assessment of disease activity and lupus nephritis. Methods. Samples from 261 well-characterized SLE patients were tested using chemiluminescent immunoassays (CIA) for anti-dsDNA and anti-Ku antibodies as well as by anti-C1q antibody ELISA (Inova Diagnostics, USA). Of these SLE patients, 26.4% had lupus nephritis (LN) at the time of blood draw or had a history of LN, and modified SLE disease activity index-2K (SLEDAI) scores were used to assess disease activity. Results. All three antibodies demonstrated higher prevalence and higher antibody levels in active versus inactive SLE patients and in LN versus non-LN patients. When oligoparametric analysis was performed, the likelihood of LN and patients with active disease increased with dual and triple positivity. Conclusions. Anti-dsDNA and anti-C1q antibodies are useful tools to identify disease activity and/or renal involvement in SLE patients. In addition, the combination of those antibodies in a two-parametric score might improve the clinical utility of those markers.
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| 128. |
- Skoglund, Caroline, 1981-, et al.
(author)
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C-reactive protein and C1q regulate platelet adhesion and activation on adsorbed immunoglobulin G and albumin.
- 2008
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In: Immunology and cell biology. - : Wiley. - 0818-9641 .- 1440-1711. ; 86:5, s. 466-74
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Journal article (peer-reviewed)abstract
- Blood platelets and C-reactive protein (CRP) are both used clinically as markers of ongoing inflammation, and both participate actively in inflammatory responses, although the biological effects are still incompletely understood. Rapidly adhering platelets express receptors for complement factor 1q (C1q) and the Fc part of immunoglobulin G (IgG), and CRP is known to activate/regulate complement via C1q binding, and to ligate FcgammaRs. In the present study, we used normal human IgG pre-adsorbed to a well-characterized methylated surface as a model solid-phase immune complex when investigating the effects of CRP and C1q on platelet adhesion and activation. Protein adsorption was characterized using ellipsometry and polyclonal antibodies, and human serum albumin (HSA) and non-coated surfaces were used as reference surfaces. Platelet adhesion to IgG and HSA was inhibited by both C1q and CRP. Furthermore, CRP (moderately) and C1q (markedly) decreased the spreading of adhering platelets. The combination of C1q and CRP was slightly more potent in reducing cell adhesion to IgG, and also impaired the adhesion to HSA and non-coated surfaces. Platelet production of thromboxane B2 (TXB(2)) was also reduced by C1q both in the presence and absence of CRP, whereas CRP alone had no effect on TXB(2) production. We conclude that CRP and C1q regulate the behaviour of platelets, and that this may be an important immunoregulatory mechanism during inflammatory conditions.
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| 129. |
- Skoglund, Oliver, et al.
(author)
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Fighting Fatigue in Systemic Lupus Erythematosus : Experience of Dehydroepiandrosterone on Clinical Parameters and Patient-Reported Outcomes
- 2022
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In: Journal of Clinical Medicine. - : MDPI. - 2077-0383. ; 11:18
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Journal article (peer-reviewed)abstract
- Manifestations related to ongoing inflammation in systemic lupus erythematosus (SLE) are often adequately managed, but patient-reported outcome measures (PROMs) support that fatigue and low quality of life (QoL) in the absence of raised disease activity remain major burdens. The adrenal hormone dehydroepiandrosterone (DHEA) has shown potential as a pharmacological agent for managing fatigue in mild SLE. We retrospectively evaluated data on dosage, disease activity, corticosteroid doses, concomitant antirheumatic drugs, and PROMs regarding pain intensity, fatigue, and well-being (visual analogue scales), QoL (EQ-5D-3L) and functional disability. A total of 15 patients with SLE were exposed to DHEA and 15 sex- and age-matched non-exposed SLE patients served as comparators. At baseline, 83% of the DHEA-exposed patients had subnormal DHEA concentration. The 15 subjects prescribed DHEA were exposed during a median time of 12 months (IQR 16.5) [range 3-81] and used a median daily dose of 50 mg of DHEA (IQR 25.0) [range 25-200]. Neither disease activity, nor damage accrual, changed significantly over time among patients using DHEA, and no severe adverse events were observed. Numerical improvements of all evaluated PROMs were seen in the DHEA-treated group, but none reached statistical significance. For DHEA-exposed patients, a non-significant trend was found regarding fatigue comparing baseline and 36 months (p = 0.068). In relation to SLE controls, the DHEA-exposed group initially reported significantly worse fatigue, pain, and well-being, but the differences diminished over time. In conclusion, DHEA was safe, but evidence for efficacy of DHEA supplementation in relation to PROMs were not found. Still, certain individuals with mild SLE, plagued by fatigue and absence of increased disease activity, appear to benefit from DHEA in terms of improved fatigue and QoL. Testing of DHEA concentration in blood should be performed before initiation, and investigation of other conditions, or reasons responsible for fatigue, must always be considered first.
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| 130. |
- Skoog, Johan, et al.
(author)
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High-frequency ultrasound with superb microvascular imaging: a potential tool for ultrasound assessment in patients with giant cell arteritis
- 2024
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In: Frontiers in Medicine. - : FRONTIERS MEDIA SA. - 2296-858X. ; 11
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Journal article (peer-reviewed)abstract
- Objective The objective of this study was 2-fold: first, to evaluate whether superb microvascular imaging (SMI) could be used to visualize neovascularization in temporal arteries, and, second, to evaluate the diagnostic performance of high frequency ultrasound with SMI using an extended protocol in patients with suspected giant cell arteritis (GCA).Methods This retrospective study comprised 120 patients consecutively examined with an extended CDU protocol (temporal, facial, axillary, subclavian, brachiocephalic, and carotid arteries) between 2020 and 2022. Of all patients, 107 had no previous GCA diagnosis and 13 had a previous GCA diagnosis. SMI was used to evaluate neovascularization in the temporal arteries. Arteritis were characterized as low- or medium-echogenic, homogeneous wall thickening, with or without a positive compression sign in the temporal arteries. The Halo count, i.e., the number of temporal and axillary artery segments with signs of arteritis, was evaluated. The reference was clinically diagnosed GCA confirmed after >= 6-month follow-up.Results Of the eligible 107 patients with new suspected GCA, 33 (31%) received a clinical GCA diagnosis. Neovascularization was detected in 14 patients (43%). Patients with neovascularization displayed a higher halo count [median 6 (25th-75th percentile 4.75-7) vs. 3 (2-4-4), p = 0.005]. CDU of only the temporal arteries showed sensitivity and specificity (95% confidence intervals) of 94% (80-100%) and 100% (95-100%), respectively. The addition of extra-cranial arteries increased the sensitivity to 100%. Of the 13 patients investigated for suspected relapse, three had a clinically confirmed relapse. One of them displayed neovascularization together with other signs of inflammation.Conclusions We show for the first time that inflammatory neovascularization of the temporal arteries can be detected by SMI. Neovascularization is associated with a more-widespread cranial disease. The value of neovascularization should be further investigated, especially for the detection of GCA relapse.
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