scholarly journals Obinutuzumab in connective tissue diseases after former rituximab-non-response: a case series

2022 ◽  
pp. annrheumdis-2021-221756
Author(s):  
Peter Kvacskay ◽  
Wolfgang Merkt ◽  
Janine Günther ◽  
Norbert Blank ◽  
Hanns-Martin Lorenz
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Case Series

Ulnar artery vasculopathy: a common but nonspecific feature of systemic sclerosis

2017 ◽  
Vol 2 (3) ◽  
pp. 221-224
Author(s):  
Tae-Jong Kim ◽  
Soon-Young Song ◽  
Hee Chang Ahn ◽  
Yoon-Kyoung Sung ◽  
Sang-Cheol Bae ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Case Series ◽  
Ulnar Artery ◽  
Common Finding ◽  
Clinical Factors ◽  
Vessel Disease ◽  
Large Vessel Disease

Introduction Systemic sclerosis (SSc) is characterized by microvascular abnormalities and fibrosis. Several studies have reported that large vessel disease is also common in SSc. The aim of this case series was to investigate whether ulnar artery involvement in patients with SSc is a disease-specific phenomenon, as compared to other connective tissue diseases (CTD). Methods A total of 28 patients, including 7 with SSc and 12 with systemic lupus erythematosus (SLE), underwent brachial arteriography due to severe Raynaud's phenomenon and/or digital ulcerations and were enrolled in the study. They were divided into two groups: an SSc/SSc-overlap group and a non-SSc group. The collection of the clinical parameters was conducted to investigate the associations between clinical factors and the ulnar artery vasculopathy. Results The SSc/SSc-overlap group (n = 10) consisted of 7 patients with SSc and 3 with features overlapping SSc. In the non-SSc group (n = 18), 12 cases of SLE, 2 of mixed connective tissue disease, 1 of dermatomyositis + SLE, 1 of rheumatoid arthritis, 1 of Sjogren's syndrome, and 1 case of skin vasculitis, were included. The relative frequencies of ulnar artery involvement were not significantly different between the SSc/SSc-overlap and non-SSc groups, respectively (n = 6, 60% vs. n = 9, 50%, p = 0.611). Conclusions Although ulnar artery involvement was frequently detected in patients with SSc/SSc-overlap, it was also a common finding in other CTDs; therefore, it is not specific to SSc or SSc-overlap diseases.

scholarly journals Patients with ANCA-Associated Glomerulonephritis and Connective Tissue Diseases: A Comparative Study from the Maine-Anjou AAV Registry

2019 ◽  
Vol 8 (8) ◽  
pp. 1218
Author(s):  
Fanny Guibert ◽  
Anne-Sophie Garnier ◽  
Samuel Wacrenier ◽  
Giorgina Piccoli ◽  
Assia Djema ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Thrombotic Event ◽  
Connective Tissue Diseases ◽  
Case Series ◽  
Rare Condition ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Control Group ◽  
Renal Relapse ◽  
Patients Experience

Background and objectives: The overlap between antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis (ANCA-GN) and connective tissue diseases (CTD) has been reported mainly as case series in the literature. Frequency of this association, as well as presentation and outcomes are unknown. Materials and Methods: Patients from the Maine-Anjou ANCA-associated vasculitides (AAV) registry with ANCA-GN diagnosed between 01/01/2000 and 01/01/2018, ANCA positivity, and at least six months of follow-up, were included. Results: 106 out of 142 patients fulfilled the inclusion criteria and were analyzed. CTD was present at ANCA-GN diagnosis in 16 (15.1%) patients. The most common CTD were rheumatoid arthritis, Sjogren syndrome and systemic sclerosis. Compared to the control group, females were more represented in the CTD group (75%, p = 0.001). Renal presentation was comparable between groups, including the pathological analysis of renal biopsies. Patients of CTD group presented a higher rate of non-renal relapse (25% versus 7.7%, p = 0.037), and experienced more frequently a venous thrombotic event (31.2% versus 10%, p = 0.021). No difference between groups was observed according to major outcomes. Conclusion: Association between CTD and ANCA-GN is not a rare condition and predominantly affects females. While AAV presentation is not significantly different, CTD patients experience more frequently non-renal relapse and venous thrombotic events.

scholarly journals Myelodysplastic Syndrome and Autoimmunity: A Case Report of an Unusual Presentation of Myelodysplastic Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Andrea L. Merrill ◽  
Hedy Smith
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Myelodysplastic Syndrome ◽  
Lacrimal Gland ◽  
Case Reports ◽  
Connective Tissue Diseases ◽  
Case Series ◽  
Organizing Pneumonia ◽  
Trisomy 8 ◽  
Autoimmune Phenomena

Myelodysplastic syndrome (MDS) commonly presents asymptomatically or with symptomatic cytopenias. However, autoimmune phenomena in association with MDS have been well described in several case reports and case series. Typically, these autoimmune phenomena take the form of vasculitides, arthritis, connective tissue diseases, pulmonary infiltrates, or polymyalgia rheumatica. We present the case of a woman with MDS (karyotype 46,XX,+1,der(1;7)(q10;p10)[20], that evolved with an additional trisomy 8 clone) and a novel spectrum of autoimmune diseases including acute fibrinous and organizing pneumonia (AFOP) and lacrimal gland pseudotumor.

scholarly journals P170 The validity and utility of the SLE-key® rule-out serological test when applied in a selected cohort of patients with SLE and other connective tissue diseases

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Sheilla Achieng ◽  
John A Reynolds ◽  
Ian N Bruce ◽  
Marwan Bukhari
Keyword(s):  
Linear Regression ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Negative Correlation ◽  
Connective Tissue Diseases ◽  
Inflammatory Myositis ◽  
The Mean ◽  
Negative Rule ◽  
Spearman’S Correlation ◽  
Rule Out

Abstract Background/Aims  We aimed to establish the validity of the SLE-key® rule-out test and analyse its utility in distinguishing systemic lupus erythematosus (SLE) from other autoimmune rheumatic connective tissue diseases. Methods  We used data from the Lupus Extended Autoimmune Phenotype (LEAP) study, which included a representative cross-sectional sample of patients with a variety of rheumatic connective tissue diseases, including SLE, mixed connective tissue disease (MCTD), inflammatory myositis, systemic sclerosis, primary Sjögren’s syndrome and undifferentiated connective tissue disease (UCTD). The modified 1997 ACR criteria were used to classify patients with SLE. Banked serum samples were sent to Immune-Array’s CLIA- certified laboratory Veracis (Richmond, VA) for testing. Patients were assigned test scores between 0 and 1 where a score of 0 was considered a negative rule-out test (i.e. SLE cannot be excluded) whilst a score of 1 was assigned for a positive rule-out test (i.e. SLE excluded). Performance measures were used to assess the test’s validity and measures of association determined using linear regression and Spearman’s correlation. Results  Our study included a total of 155 patients of whom 66 had SLE. The mean age in the SLE group was 44.2 years (SD 13.04). 146 patients (94.1%) were female. 84 (54.2%) patients from the entire cohort had ACR SLE scores of ≤ 3 whilst 71 (45.8%) had ACR SLE scores ≥ 4. The mean ACR SLE total score for the SLE patients was 4.85 (SD 1.67), ranging from 2 to 8, with mean disease duration of 12.9 years. The Sensitivity of the SLE-Key® Rule-Out test in diagnosing SLE from other connective tissue diseases was 54.5%, specificity was 44.9%, PPV 42.4% and NPV 57.1 %. 45% of the SLE patients had a positive rule-out test. SLE could not be ruled out in 73% of the MCTD patients whilst 51% of the UCTD patients had a positive Rule-Out test and >85% of the inflammatory myositis patients had a negative rule-out test. ROC analysis generated an AUC of 0.525 illustrating weak class separation capacity. Linear regression established a negative correlation between the SLE-key Rule-Out score and ACR SLE total scores. Spearman’s correlation was run to determine the relationship between ACR SLE total scores and SLE-key rule-out score and showed very weak negative correlation (rs = -0.0815, n = 155, p = 0.313). Conclusion  Our findings demonstrate that when applied in clinical practice in a rheumatology CTD clinic setting, the SLE-key® rule-out test does not accurately distinguish SLE from other CTDs. The development of a robust test that could achieve this would be pivotal. It is however important to highlight that the test was designed to distinguish healthy subjects from SLE patients and not for the purpose of differentiating SLE from other connective tissue diseases. Disclosure  S. Achieng: None. J.A. Reynolds: None. I.N. Bruce: Other; I.N.B is a National Institute for Health Research (NIHR) Senior Investigator and is funded by the NIHR Manchester Biomedical Research Centre. M. Bukhari: None.

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