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 Pictorial review: Thoracic involvement in connective tissue diseases: radiological patterns and follow-up

2015 ◽  
Vol 98 (1) ◽  
pp. 3
Author(s):  
G. Serra ◽  
A. L. Brun ◽  
P. Ialongo ◽  
M. L. Chabi ◽  
Philippe A. Grenier
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Pictorial Review ◽  
Radiological Patterns

scholarly journals Serum KL-6 as predictive and prognostic marker of interstitial lung disease in childhood connective tissue diseases: a pilot study

Reumatismo ◽  
2021 ◽  
Vol 73 (3) ◽  
Author(s):  
R. El-Beheidy ◽  
A.M. Domouky ◽  
H. Zidan ◽  
Y.A. Amer
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Control Group ◽  
Connective Tissue Disorders ◽  
Juvenile Systemic Lupus Erythematosus ◽  
Median Serum ◽  
Juvenile Systemic Sclerosis

This study was aimed to evaluate serum KL-6 levels to determine if this marker can be used for diagnosing and assessing severity of interstitial lung disease (ILD) in children with connective tissue disorders. In total, 40 patients [18 patients with juvenile systemic lupus erythematosus (JSLE), 10 patients with juvenile idiopathic arthritis (JIA), 8 patients with juvenile mixed connective tissue disease (JMCTD), 3 patients with juvenile systemic sclerosis (JSSc), and 1 patient with juvenile dermatomyositis (JDM)] and 20 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. 20 of the 40 patients with CTD (50%) had ILD, 12 were mild and 8 were severe as assessed by spirometry. The median serum KL-6 level was 102.7 U/mL (76.1-180.8) in the CTD with severe ILD group, 72.2 U/mL (58.4- 100.5) in the CTD with mild ILD group, 56.7 U/mL (35.8-68.5) in the CTD without ILD group, and 52.3 U/mL (32.8-62.4) in the control group. KL-6 levels were significantly higher in the CTD with ILD (p<0.05), at a cutoff of 63.4 U/ml identified by ROC curve, serum KL-6 showed a sensitivity of 95.2% and specificity of 89.7%. KL-6 is a valuable biomarker for diagnostic purposes and to detect severity in ILD in childhood CTD.

Safety of hydroxychloroquine in pregnant patients with connective tissue diseases: A study of one hundred thirty-three cases compared with a control group

2003 ◽  
Vol 48 (11) ◽  
pp. 3207-3211 ◽  
Author(s):  
Nathalie Costedoat-Chalumeau ◽  
Zahir Amoura ◽  
Pierre Duhaut ◽  
Du Le Thi Huong ◽  
Djamel Sebbough ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Control Group

Connective tissue diseases

2020 ◽  
pp. 127-184
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Granulomatosis With Polyangiitis ◽  
Connective Tissue Diseases ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Assessment Tools ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Key Points ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis

This chapter covers the connective tissue diseases including systemic lupus erythematosus, Sjögren’s syndrome, scleroderma, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (including granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly known as Churg–Strauss syndrome), and microscopic polyangiitis), polyarteritis nodosa, and Behçet’s disease. For each example of a connective tissue disease it provides an overview of the condition and classification criteria, alongside the prognosis. Techniques and tricks for diagnosis, clinical features, assessment tools, and treatment are all covered. Key points of nursing care are described, including the nurse’s role in treatment with thalidomide and cyclophosphamide, and any particular organs that can be affected is detailed.

scholarly journals The Serum Cell-Free microRNA Expression Profile in MCTD, SLE, SSc, and RA Patients

2020 ◽  
Vol 9 (1) ◽  
pp. 161 ◽  
Author(s):  
Barbara Stypinska ◽  
Anna Wajda ◽  
Ewa Walczuk ◽  
Marzena Olesinska ◽  
Aleksandra Lewandowska ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Signaling Pathway ◽  
Expression Profile ◽  
Mirna Expression ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Mirna Expression Profile ◽  
Expression Level ◽  
Control Group ◽  
Mirna Expression Level

Mixed connective tissue disease (MCTD) is a rare disorder characterized by symptoms that overlap two or more Autoimmune Connective Tissue Diseases (ACTDs). The aim of this study was to determine whether miRNAs participating in the TLRs signaling pathway could serve as biomarkers differentiating MCTD or other ACTD entities from a healthy control group and between groups of patients. Although the selected miRNA expression level was not significantly different between MCTD and control, we observed that miR-126 distinguishes MCTD patients from all other ACTD groups. The expression level of miRNAs was significantly higher in the serum of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) patients compared to controls. The miR-145 and -181a levels distinguished RA from other ACDT patients. miR-155 was specific for SLE patients. MiR-132, miR-143, and miR-29a distinguished RA and SLE patients from the systemic sclerosis (SSc) group. Additionally, some clinical parameters were significantly related to the miRNA expression profile in the SLE group. SLE and RA are characterized by a specific serum expression profile of the microRNAs associated with the Toll-like receptors (TLRs) signaling pathway. The analysis showed that their level distinguishes these groups from the control and from other ACTD patients. The present study did not reveal a good biomarker for MCTD patients.

scholarly journals Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Nicola Ciancio ◽  
Mauro Pavone ◽  
Sebastiano Emanuele Torrisi ◽  
Ada Vancheri ◽  
Domenico Sambataro ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Connective Tissue Diseases ◽  
Function Tests

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.

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