Systemic sclerosis and primary biliary cholangitis: An overlapping entity?

2018 ◽  
Vol 4 (2) ◽  
pp. 111-117 ◽  
Author(s):  
Gemma Lepri ◽  
Silvia Bellando Randone ◽  
Marco Matucci Cerinic ◽  
Yannick Allanore
Keyword(s):  
Systemic Sclerosis ◽  
T Lymphocytes ◽  
Early Stage ◽  
Primary Biliary Cholangitis ◽  
Specific Antibodies ◽  
American College Of Rheumatology ◽  
Regulatory T Lymphocytes ◽  
Fibrotic Disorders ◽  
New Research ◽  
And Function

Systemic sclerosis (SSc) is a complex autoimmune disease that may lead to skin and internal organ fibrosis. Based on skin involvement, two subsets of the disease are recognized (limited cutaneous SSc and diffuse cutaneous SSc). The new 2013 American College of Rheumatology/European League against Rheumatism classification criteria allow to identify SSc patients at the early stage of the disease that allows new research avenues. The aetiology of the disease is still unknown, but it has an important autoimmune basis and its association with other autoimmune diseases has been reproducibly reported. Among them, primary biliary cholangitis is considered the most common liver disease in SSc. The aim of this review is to provide an overview on recent findings about SSc associated to primary biliary cholangitis. Although the aetiology of the two diseases is still unknown, data suggest that these two disorders share the expression of fibrogenic cytokines, involved both in generation and function of T lymphocytes subpopulation (Th17 cells) and regulatory T lymphocytes. In addition, the relationships between SSc and primary biliary cholangitis may be closer as suggested by the presence of primary biliary cholangitis–specific antibodies in SSc patients and vice versa. Recent findings confirm a prevalence of overt primary biliary cholangitis in about 2% of SSc population, in particular in patients with limited cutaneous SSc and positive anticentromere antibodies. The prevalence increases if also patients with only primary biliary cholangitis–specific antibodies are considered. Data regarding SSc prevalence in primary biliary cholangitis patients have also been recently clarified. Altogether, stimulating results are moving the field forward regarding the relationships of these two autoimmune and fibrotic disorders that may belong to an overlapping entity.

Prevalence of Systemic Sclerosis in Primary Biliary Cholangitis Using the New ACR/EULAR Classification Criteria

2016 ◽  
Vol 44 (1) ◽  
pp. 33-39 ◽  
Author(s):  
Boyang Zheng ◽  
Catherine Vincent ◽  
Marvin J. Fritzler ◽  
Jean-Luc Senécal ◽  
Martial Koenig ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Tertiary Care ◽  
Classification Criteria ◽  
Primary Biliary Cholangitis ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
European League Against Rheumatism ◽  
High Prevalence ◽  
Established Disease ◽  
Secondary Objective

Objective.Systemic sclerosis (SSc) is a well-established disease associated with primary biliary cholangitis (PBC). However, the original 1980 American College of Rheumatology (ACR) criteria have poor sensitivity, especially for the detection of earlier SSc in previous studies. The objective was to evaluate the prevalence of SSc in patients with PBC using more sensitive 2001 LeRoy and Medsger criteria and the 2013 ACR/European League Against Rheumatism (EULAR) classification criteria. The secondary objective was to evaluate the frequency of individual clinical features.Methods.One hundred consecutive patients with PBC without previously diagnosed SSc were recruited between 2005 and 2007 from a tertiary care gastroenterology clinic. All patients underwent a complete clinical examination, determination of SSc-specific antibodies, and a nailfold capillary microscopy. Fulfillment of the 3 different criteria sets was analyzed, along with individual disease features.Results.Of 100 patients with PBC, 1% met the ACR 1980 criteria, 22% met the 2001 LeRoy and Medsger criteria for early SSc, and 17% the 2013 ACR/EULAR criteria. Raynaud phenomenon, SSc-related antibodies, and SSc capillaroscopic patterns were the most prevalent findings, with the highest sensitivities to help guide future screening.Conclusion.Our data show a high prevalence of SSc in patients with PBC with probable underestimation by previous studies using the original ACR criteria. Comorbid SSc should be actively searched for based on newly described criteria to improve detection and increase benefits of earlier treatment.

Dynamics and functions of CD4+CD25high regulatory T lymphocytes in Chinese rhesus macaques during the early stage of infection with SIVmac239

2012 ◽  
Vol 157 (5) ◽  
pp. 961-967 ◽  
Author(s):  
Shao-You Li ◽  
Hou-Jun Xia ◽  
Zheng-Xi Dai ◽  
Gao-Hong Zhang ◽  
Bo Fan ◽  
...  
Keyword(s):  
T Lymphocytes ◽  
Early Stage ◽  
Rhesus Macaques ◽  
Regulatory T Lymphocytes ◽  
Chinese Rhesus Macaques

Th17 and regulatory T lymphocytes in primary biliary cirrhosis and systemic sclerosis as models of autoimmune fibrotic diseases

2012 ◽  
Vol 12 (2) ◽  
pp. 300-304 ◽  
Author(s):  
Daniela Fenoglio ◽  
Francesca Bernuzzi ◽  
Florinda Battaglia ◽  
Alessia Parodi ◽  
Francesca Kalli ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
T Lymphocytes ◽  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis ◽  
Regulatory T Lymphocytes ◽  
Fibrotic Diseases

Evaluation of the primary biliary cholangitis-related serologic profile in a large cohort of Belgian systemic sclerosis patients

2020 ◽  
Vol 58 (3) ◽  
pp. 416-423 ◽  
Author(s):  
Lisa Florin ◽  
Kaat Rubben ◽  
Amber Vanhaecke ◽  
Katrien Devreese ◽  
Filip De Keyser ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Liver Enzymes ◽  
Primary Biliary Cholangitis ◽  
Antimitochondrial Antibodies ◽  
Specific Antibodies ◽  
Tissue Sections ◽  
Wide Range ◽  
Glutamyl Transferase ◽  
Disease Specific

AbstractBackgroundSystemic sclerosis (SSc) and primary biliary cholangitis (PBC) are autoimmune diseases that may occur concomitantly and are both strongly associated with disease-specific autoantibodies. This study investigated the prevalence and fine specificity of PBC-specific serology (PBC-Ab) and associations with the SSc-subtypes and SSc-specific antibodies as well as the association with cholestatic liver enzymes. Furthermore, three different techniques for the detection of PBC-Ab were compared.MethodsSerum of 184 Belgian SSc patients with a known SSc-antibody profile, was analyzed for PBC-Ab (antimitochondrial antibodies [AMA], anti-Gp210, anti-Sp100 and anti-PML) using indirect immunofluorescence (IIF) analysis on human epithelioma-2000 (HEp-2000) cells (ANA-IIF, Immunoconcepts) and liver-kidney-stomach tissue sections (IIF-LKS) (Menarini), and a line immunoblot (LB) (EuroImmun). Alkaline phosphatase/γ-glutamyl transferase (ALP/GGT) were evaluated at time of first sampling (t0) and after 3 years of follow-up (t3).ResultsPBC-Ab were present in 13% of patients and significantly correlated with centromere antibodies (anti-CENP-B), but not correlated with the limited cutaneous SSc subgroup (lcSSc). The most frequent reactivities were AMA (11%, with 9% AMA-M2) and Sp-100 antibodies (5%), showing a major overlap. There was no relevant association between the presence of PBC-Ab and ALP or GGT elevation at t0 nor at t3. Detection of AMA with IIF-LKS is comparable to LB. ANA-IIF screening was less sensitive compared to LB.ConclusionsA wide range of PBC-Ab is detectable in SSc in the absence of cholestatic liver enzyme elevations, even after 3 years of follow-up. However, as these antibodies may precede PBC-disease up to 10 years further prospective follow-up of our cohort will be necessary.

scholarly journals Dynamics of T Lymphocyte Phenotypes Between the Periphery and the Brain From the Acute to the Chronic Phase Following Ischemic Stroke in Mice

2020 ◽  
Author(s):  
Myung-Shin Jeon ◽  
Minha Kim ◽  
So-Dam Kim ◽  
Kyoung-In Kim ◽  
Eun Hae Jeon ◽  
...  
Keyword(s):  
T Cells ◽  
T Lymphocytes ◽  
T Lymphocyte ◽  
Early Stage ◽  
Chronic Phase ◽  
Artery Occlusion ◽  
Immune Organs ◽  
Regulatory T Lymphocytes ◽  
Fundamental Information ◽  
The Brain

Abstract BackgroundT lymphocytes are involved in infarct size at the early stage of stroke. However, the phenotypes of T lymphocytes and their functions in peripheral immune organs and the brain have not been well-analyzed from the acute to the chronic phase of stroke.MethodsA 45 min transient middle cerebral artery occlusion mouse model was used. The phenotypes of T lymphocytes in the thymus, spleen, blood, and brain were determined using the neurological severity score (NSS) and body weights during the 6-month follow-up. ResultsImpairment of thymocyte numbers, development, proliferation, and apoptosis was observed for up to 2 weeks. The number of mature T cells in the spleen and blood decreased and showed less interferon- production for up to 2 weeks. Increased numbers of CD44+CD62L- effector T cells and CD4-CD8-CD3+ double negative T cells were observed in mouse brains in the early phase of stroke, while interleukin (IL)-10+Foxp3+ regulatory T cell levels increased for 1 week during the chronic phase. These phenotypes were correlated with body weight and the NSS. ConclusionsThe recovery of T lymphocyte numbers and increased IL-10+Foxp3+ regulatory T lymphocytes may be important for the improvement of long-term neurological outcomes. Dynamic changes in T lymphocytes from the acute and chronic phase may play different roles, such as pathological and recovery roles, respectively. This study provides fundamental information regarding the T lymphocyte alterations from the brain to the peripheral immune organs from the acute to the chronic phase of stroke.

The Challenge of Very Early Systemic Sclerosis: A Combination of Mild and Early Disease?

2020 ◽  
Vol 48 (1) ◽  
pp. 82-86 ◽  
Author(s):  
Elisabeth Blaja ◽  
Suzana Jordan ◽  
Carmen-Marina Mihai ◽  
Rucsandra Dobrota ◽  
Mike Oliver Becker ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Disease Duration ◽  
Nailfold Capillaroscopy ◽  
Organ Involvement ◽  
Early Disease ◽  
Specific Antibodies ◽  
Mild Disease ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
Clinical Expert

Objective.To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients.Methods.We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom.Results.One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation.Conclusion.This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.

scholarly journals Difference in patient-reported outcomes of various patellar component designs in total knee arthroplasty: A randomized clinical study

2021 ◽  
Vol 29 (1) ◽  
pp. 230949902199606
Author(s):  
Takeshi Mochizuki ◽  
Koichiro Yano ◽  
Katsunori Ikari ◽  
Ken Okazaki
Keyword(s):  
Knee Osteoarthritis ◽  
Clinical Effects ◽  
Patellar Component ◽  
American College Of Rheumatology ◽  
Randomized Clinical Study ◽  
Component Design ◽  
Patient Reported ◽  
Total Knee ◽  
The Difference ◽  
And Function

Purpose: This study investigated the clinical effects of different patellar components without being affected by the femoral component design in total knee arthritis (TKA) for patients with knee osteoarthritis (OA). Methods: In total, 48 patients with OA who met the criteria of the American College of Rheumatology for OA were enrolled and randomly assigned in a 1:1 ratio to two groups according to the usage of patellar component design for TKA (medialized dome type [dome group] or medialized anatomic type [anatomic group]). To evaluate the clinical outcomes for TKA, knee range of motion (ROM), pain intensity of 0–100 mm visual analog scale (pain VAS), and the Japanese Knee Osteoarthritis Measure (JKOM) score were obtained at baseline and year 1. Results: The difference in knee ROM, pain VAS, or total JKOM score at year 1 was not significant between the dome and anatomic groups ( p = 0.398, 0.733 and 0.536, respectively). Moreover, similar results were obtained for changes in knee ROM, pain VAS, or total JKOM scores from baseline. In both groups, the pain VAS and total JKOM scores were significantly improved at year 1. Conclusion: Both dome and anatomic groups in TKA are significantly effective for pain and function using the JKOM score. However, their efficacy did not differ, according to the JKOM score. Results of this study are rare information focusing on the patellar component design and provide one of the insights into the TKA clinical management.

scholarly journals Advances in the role of miRNAs in the occurrence and development of osteosarcoma

Open Medicine ◽  
2020 ◽  
Vol 15 (1) ◽  
pp. 1003-1011
Author(s):  
Guanyu Zhang ◽  
Yiran Li ◽  
Jiasheng Xu ◽  
Zhenfang Xiong
Keyword(s):  
Target Gene ◽  
Early Stage ◽  
Research Direction ◽  
Research Progress ◽  
Skeletal System ◽  
Translation Process ◽  
Non Coding Rnas ◽  
New Research ◽  
Post Transcriptional Regulation

AbstractOsteosarcoma (OS) is the most common primary malignant tumor of the skeletal system in the clinic. It mainly occurs in adolescent patients and the pathogenesis of the disease is very complicated. The distant metastasis may occur in the early stage, and the prognosis is poor. MicroRNAs (miRNAs) are non-coding RNAs of about 18–25 nt in length that are involved in post-transcriptional regulation of genes. miRNAs can regulate target gene expression by promoting the degradation of target mRNAs or inhibiting the translation process, thereby the proliferation of OS cells can be inhibited and the apoptosis can be promoted; in this way, miRNAs can affect the metabolism of OS cells and can also participate in the occurrence, invasion, metastasis, and recurrence of OS. Some miRNAs have already been found to be closely related to the prognosis of patients with OS. Unlike other reviews, this review summarizes the miRNA molecules closely related to the development, diagnosis, prognosis, and treatment of OS in recent years. The expression and influence of miRNA molecule on OS were discussed in detail, and the related research progress was summarized to provide a new research direction for early diagnosis and treatment of OS.

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