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  • 1
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    Online Resource
    A Case of Budd-Chiari Syndrome Associated With Antiphospholipid Syndrome Treated Successfully by Transjugular Intrahepatic Portosystemic Shunt
    SAGE Publications ; 2022
    In:  Clinical Medicine Insights: Case Reports Vol. 15 ( 2022-01), p. 117954762211005-
    Details
    In: Clinical Medicine Insights: Case Reports, SAGE Publications, Vol. 15 ( 2022-01), p. 117954762211005-
    Abstract: Budd Chiari syndrome (BCS) is defined as obstruction of hepatic venous outflow that can be located anywhere from small hepatic venules up to the entrance of inferior vena cava (IVC) into right atrium. Etiologies of BCS include myeloproliferative disorders, congenital, and acquired hypercoagulable states. Anticoagulation is the mainstay of treatment for all cases of BCS with a demonstrable hypercoagulable state. Interventional radiology procedures such as transjugular intrahepatic portosystemic shunting (TIPS) can be utilized to reduce portal hypertension and to improve complications related to portal hypertension. We present a patient with systemic lupus erythematosus who first presented with fever, weight loss, malar rash, alopecia, livedo reticularis, symmetric polyarthritis, pancytopenia, and class IV lupus nephritis when she was 23 years old. After receiving an induction treatment of cyclophosphamide and glucocorticoids, she received a maintenance treatment of azathioprine. She presented with ascites and abdominal pain when she was 36 and the abdominal imaging revealed reduced calibration of hepatic venules and intrahepatic segment of inferior vena cava. Lupus anticoagulant was positive and anti cardiolipin IgM and IgG were positive. Work up for hereditary hypercoagulable states was negative. Thus, the diagnosis was secondary antiphospholipid syndrome where BCS was the first clinical manifestation of the antiphospholipid syndrome. Patient was anticoagulated with warfarin and received diuretics for ascites. After the ascites became refractory to diuretics and the patient had multiple vertebral compression fractures due to volume overload secondary to ascites, she was successfully treated with TIPS. When control imaging was performed, 50% of stenosis was observed in the stent. Balloon dilation of the stent was performed. Interventional radiology techniques like TIPS can be used in BCS patients secondary to APS, in cases when medical treatment is insufficient to control complications of portal hypertension. In BCS patients secondary to APS, TIPS enables clinical improvement but due to the presence of endothelial dysfunction in APS patients, there is a risk of shunt dysfunction secondary to thrombosis or stenosis secondary to intimal hyperplasia. Therefore, strict anticoagulation and regular follow up of patients after TIPS is recommended. In cases with stent stenosis, reintervention may be necessary.
    Type of Medium: Online Resource
    ISSN: 1179-5476 , 1179-5476
    URL: Article
    DOI: 10.1177/11795476221100595
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2022
    detail.hit.zdb_id: 2580498-4
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  • 2
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    Description of damage in different clusters of patients with antiphospholipid syndrome
    SAGE Publications ; 2022
    In:  Lupus Vol. 31, No. 4 ( 2022-04), p. 433-442
    Details
    In: Lupus, SAGE Publications, Vol. 31, No. 4 ( 2022-04), p. 433-442
    Abstract: To identify the different clinical phenotypes of antiphospholipid syndrome (APS) by using cluster analysis and describe cumulative damage of disease clusters. Methods This retrospective study includes patients with APS (±systemic lupus erythematosus (SLE)). Two-step cluster analysis was applied by considering clinical data. Damage was calculated for all patients by applying damage index for APS (DIAPS). Results A total of 237 patients (198 females; median age of 43 years; median follow-up of 9.5 years) were classified into four clusters. Cluster 1 ( n = 74) consisted of older patients with arterial-predominant thrombosis, livedo reticularis, and increased cardiovascular risk; cluster 2 ( n = 70) of SLE+APS patients with thrombocytopenia and heart valve disease; cluster 3 ( n = 59) of patients with venous-predominant thrombosis, less extra-criteria manifestations; and cluster 4 ( n = 34) of patients with only pregnancy morbidity with lower frequency of extra-criteria features and cardiovascular risk. Patients with SLE+APS ( n = 123) had the highest mean DIAPS. Regarding clusters, 1 and 2 had high cumulative damage. While cumulative survival rates of clusters did not differ, cluster 2 and 3 had lower survival rates at further years. There was no correlation between DIAPS and mortality. Conclusion SLE+APS patients with extra-criteria manifestations and older APS patients with arterial thrombosis and increased cardiovascular risk have higher cumulative damage. Effective treatment of SLE disease activity and control of cardiovascular risk may help to reduce cumulative damage in these patients.
    Type of Medium: Online Resource
    ISSN: 0961-2033 , 1477-0962
    URL: Article
    DOI: 10.1177/09612033221079781
    RVK:
    XA 10000
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2022
    detail.hit.zdb_id: 2008035-9
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  • 3
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    Immunosuppression use in primary antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”)
    SAGE Publications ; 2022
    In:  Lupus Vol. 31, No. 14 ( 2022-12), p. 1770-1776
    Details
    In: Lupus, SAGE Publications, Vol. 31, No. 14 ( 2022-12), p. 1770-1776
    Abstract: APS ACTION Registry was created to study the outcomes of patients with persistently positive antiphospholipid antibodies (aPL) with or without other systemic autoimmune disease (SAIDx). Given that immunosuppression (IS) is used for certain aPL manifestations, for example, thrombocytopenia (TP), our primary objective was to describe the indications for IS in aPL-positive patients without other SAIDx. Secondly, we report the type of IS used in patients with selected microvascular or non-thrombotic aPL manifestations. Methods An online database is used to collect clinical data. The inclusion criteria are positive aPL based on the laboratory section of the APS Classification Criteria, tested at least twice within one year prior to enrollment. Patients are followed every 12 ± 3 months. For this descriptive retrospective and prospective analysis, we included aPL-positive patients without other SAIDx and excluded those with new SAIDx classification during follow-up. For each patient, we retrieved clinical data at baseline and follow-up including selected aPL manifestations (diffuse alveolar hemorrhage [DAH], antiphospholipid-nephropathy [aPL-N] , livedoid vasculopathy [LV]-related skin ulcers, TP, autoimmune hemolytic anemia [AIHA] , cardiac valve disease [VD]), and IS medications. Results Of 899 patients enrolled, 537 were included in this analysis (mean age 45 ± 13 years, female 377 [70%], APS Classification in 438 [82%] , and at least one selected microvascular or non-thrombotic aPL manifestation in 141 (26%)). Of 537 patients, 76 (14%) were reported to use IS (ever), and 41/76 (54%) received IS primarily for selected aPL manifestation. In six of 8 (75%) DAH patients, 6/19 (32%) aPL-N, 4/28 (14%) LV, 25/88 (28%) TP, 6/11 (55%) AIHA, and 1/43 (2%) VD, the IS (excluding corticosteroids/hydroxychloroquine) indication was specific for selected aPL manifestation. Conclusion In our international cohort, 14% of aPL-positive patients without other SAIDx were reported to receive IS; the indication was at least one of the selected microvascular and/or non-thrombotic aPL-related manifestations in half. Thrombocytopenia was the most frequent among those selected aPL-related manifestations; however, approximately one-third received IS specifically for that indication. Diffuse alveolar hemorrhage was frequently treated with IS followed by AIHA and aPL-N. Systematic controlled studies are urgently needed to better define the role of IS in APS.
    Type of Medium: Online Resource
    ISSN: 0961-2033 , 1477-0962
    URL: Article
    DOI: 10.1177/09612033221128742
    RVK:
    XA 10000
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2022
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  • 4
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    Symptomatic osteonecrosis of the hip and knee in patients with systemic lupus erythematosus: Prevalence, pattern, and comparison of natural course
    SAGE Publications ; 2021
    In:  Lupus Vol. 30, No. 10 ( 2021-09), p. 1603-1608
    Details
    In: Lupus, SAGE Publications, Vol. 30, No. 10 ( 2021-09), p. 1603-1608
    Abstract: Osteonecrosis (ON), also known as avascular necrosis, is characterized by the collapse of the architectural bone structure secondary to the death of the bone marrow and trabecular bone. Osteonecrosis may accompany many conditions, especially rheumatic diseases. Among rheumatic diseases, osteonecrosis is most commonly associated with systemic lupus erythematosus (SLE). We assessed prevalence and distribution pattern of symptomatic ON in patients with SLE and compare the natural courses of hip and knee ON. Methods 912 SLE patients admitted between 1981 and 2012 were reviewed. SLE patients with symptomatic ON were retrospectively identified both from the existing SLE/APS database. The prevalence of symptomatic ON was calculated; with ON, the joint involvement pattern was determined by examining the distribution of the joints involved, and then the data about the hip and knee joints were entered in the Kaplan-Meier analysis. Kaplan-Meier methods were used to calculate 5- and 10-year rates of ON-related hip (the hip group) and knee survival (the knee group). Results Symptomatic ON developed in various joints in 97 of 912 patients with SLE, and the overall prevalence of ON was detected as 10.6%. The mean age at the time of SLE and ON diagnoses were 27.9 ± 9.9 (14–53) and 34.2 ± 11.3 (16–62) years, respectively. The mean duration from diagnosis of SLE to the first development of ON was 70.7± 60.2 (range = 0–216) months. The most common site for symptomatic ON was the hips (68%, n=66), followed by the knees (38%, n = 37). According to Kaplan-Meier analysis, hip and knee joint survival rates associated with 5-year ON were 51% and 88%, and 10-year survival rates were 43% and 84%, respectively. Conclusion We observed that the prevalence of symptomatic ON in patients with SLE was 10.6%. With the estimated 10-year survival rates of 40% versus 84% for the hip and knee joints, respectively, hip involvement may demonstrate a more aggressive course to end-stage osteoarthritis than the knee involvement.
    Type of Medium: Online Resource
    ISSN: 0961-2033 , 1477-0962
    URL: Article
    DOI: 10.1177/09612033211031007
    RVK:
    XA 10000
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2021
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  • 5
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    Genetic screening of early-onset patients with systemic lupus erythematosus by a targeted next-generation sequencing gene panel
    SAGE Publications ; 2022
    In:  Lupus Vol. 31, No. 3 ( 2022-03), p. 330-337
    Details
    In: Lupus, SAGE Publications, Vol. 31, No. 3 ( 2022-03), p. 330-337
    Abstract: In this study, we aimed to screen 31 genes (C1QA, C1QB, C1QC, C1R, C1S, C2, C3, TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR, DNASE1, DNASE1L3, PRKCD, ACP5, SLC7A7, IFIH1, TMEM173, ISG15, CYBB, FAS, FASLG, KRAS, NRAS, MAN2B1, PEPD, PTPN11, RAG2, and SHOC2), that we have categorized under the umbrella term “monogenic lupus” using a targeted next-generation sequencing (NGS) panel in 24 individuals with early-onset (≤10 years of age) systemic lupus erythematosus (SLE) and in 24 patients with late-onset ( 〉 10 years of age) disease. Methods A total of 48 SLE patients (24 with disease onset ≤10 years of age and 24 with disease onset 〉 10 years of age) were included. Patients with late-onset disease have been used as patient controls. Sequencing was carried out using 400 bp kit on the Ion S5 system. Results Among the 48 patients, three had one pathogenic variant and 45 patients had at least one rare variant classified as benign, likely benign or variant of unknown significance (VUS). In all three patients with a pathogenic variant, the onset of disease was before 10 years of age. Two patients (they were siblings) carried C1QA homozygote pathogenic allele (p.Gln208Ter, rs121909581), and one patient carried PEPD heterozygote pathogenic allele (p.Arg184Gln, rs121917722). Conclusion We demonstrated a pathogenic variant in our target gene panel with a frequency of 9.52% in patients with a disease onset ≤10 years of age. All patients with early-onset SLE phenotype, irrespective of a positive family history for SLE or parental consanguinity, should be scanned for a single-gene defect by a targeted gene panel sequencing. With the discovery of many single-gene defects and ongoing efforts to identify novel genes in SLE, similar gene panels including even more genes will possibly become more necessary and practical in the future.
    Type of Medium: Online Resource
    ISSN: 0961-2033 , 1477-0962
    URL: Article
    DOI: 10.1177/09612033221076733
    RVK:
    XA 10000
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2022
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  • 6
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    Performances of different classification criteria for systemic lupus erythematosus in a single-center cohort from Turkey
    SAGE Publications ; 2022
    In:  Lupus Vol. 31, No. 12 ( 2022-10), p. 1536-1543
    Details
    In: Lupus, SAGE Publications, Vol. 31, No. 12 ( 2022-10), p. 1536-1543
    Abstract: Sensitivity and specificity of SLE classification criteria may vary in different populations and clinical settings. In this study, we aimed to compare the performances of three criteria sets/rules (1997, 2012, and 2019) in a large cohort of patients and relevant diseased controls. Methods The medical records of consecutive SLE patients and diseased controls were reviewed for clinical and laboratory features relevant to all sets of criteria. Criteria sets/rules were analyzed based on sensitivity, positive predictive value, specificity, and negative predictive value, using clinical diagnosis with at least 6 months of follow-up as the gold standard. A subgroup analysis was performed in ANA positive patients. Results A total of 393 SLE patients and 308 non-SLE diseased controls were included. Sensitivity was 78.4% for 1997 criteria and was more than 90% for both 2012 (91.9%) and 2019 (94.4%) criteria. Specificity was the highest (95.1%) for 1997 ACR criteria, 91.5% for 2012 SLICC criteria and 91.2% for 2019 EULAR/ACR criteria. When only ANA positive patients were analyzed, sensitivity of each criteria increased by 1%, 0.8%, and 2.2%, respectively. Specificity of 1997 criteria decreased by 2% and specificity of 2012 and 2019 criteria both decreased to less than 90%. Conclusion EULAR/ACR criteria were more sensitive than 1997 criteria and had a comparable performance with SLICC criteria. When only ANA positive patients were analyzed, the presence of false positive results (originated from patients with Sjögren’s disease and antiphospholipid syndrome mainly) decreased the specificity of both SLICC and EULAR/ACR criteria.
    Type of Medium: Online Resource
    ISSN: 0961-2033 , 1477-0962
    URL: Article
    DOI: 10.1177/09612033221126866
    RVK:
    XA 10000
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2022
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  • 7
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    Online Resource
    The relationship between serum A proliferation-inducing ligand and B-cell activating factor levels with disease activity and organ involvement in systemic lupus erythematosus
    SAGE Publications ; 2022
    In:  Lupus Vol. 31, No. 5 ( 2022-04), p. 555-564
    Details
    In: Lupus, SAGE Publications, Vol. 31, No. 5 ( 2022-04), p. 555-564
    Abstract: We aim to investigate the association between serum B-cell activating factor (BAFF) and A proliferation-inducing ligand (APRIL) levels with disease activity and clinical findings in SLE patients. Methods Seventy-nine patients with SLE and 27 healthy controls were included into the study. Serum BAFF and APRIL levels were measured by using ELISA. In 19 patients with active disease at the time of the assessment, BAFF/APRIL levels were reassessed after 6 months of follow-up and disease activity was evaluated by using SLEDAI-2K. The relationship between renal histopathology index scores and lupus nephritis (LN) classes with serum BAFF/APRIL levels was examined in 16 patients who had recent renal involvement and underwent biopsy during the study. Results Although both BAFF/APRIL levels were higher in patients with SLE compared to the control group ( p 〈 0.001), no correlation was found between BAFF/APRIL levels and SLEDAI scores. Serum BAFF levels were higher in patients with renal disease activity ( p = 0.01), and there was a significant correlation between APRIL levels and proteinuria (r = 0.42, p = 0.02). A weak inverse correlation was observed between BAFF and C3 levels (r = 0.25, p = 0.02). No correlation was found between BAFF/APRIL levels and renal SLEDAI scores, renal histopathology, activity, and chronicity index scores. In the active disease group after treatment, there was no significant change in serum BAFF levels, but a significant increase in serum APRIL levels was observed. Conclusion These results suggest that both cytokines are involved in the pathogenesis of SLE and that serum BAFF can be valuable as a biomarker in SLE especially in patients with renal activity.
    Type of Medium: Online Resource
    ISSN: 0961-2033 , 1477-0962
    URL: Article
    DOI: 10.1177/09612033221086123
    RVK:
    XA 10000
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2022
    detail.hit.zdb_id: 2008035-9
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