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 Evolving liver inflammation in biochemically normal individuals with anti-mitochondria antibodies

2019 ◽  
Vol 10 (1) ◽  
Author(s):  
Danielle Cristiane Baldo ◽  
Alessandra Dellavance ◽  
Maria Lucia Gomes Ferraz ◽  
Luis Eduardo C. Andrade
Keyword(s):  
Liver Fibrosis ◽  
Rat Kidney ◽  
Surrogate Marker ◽  
Serum Levels ◽  
Autoimmune Response ◽  
Primary Biliary Cholangitis ◽  
Gamma Glutamyl Transferase ◽  
Normal Individuals ◽  
Glutamyl Transferase

Abstract Background Anti-mitochondria autoantibodies (AMA) occur in > 95% primary biliary cholangitis (PBC) patients. Biochemically normal AMA-positive (BN/AMA+) individuals, occasionally noticed by indirect immunofluorescence (IIF) on HEp-2 cells and confirmed in AMA-specific assays, may represent early stages of PBC. The Enhanced Liver Fibrosis (ELF) score is a surrogate marker for liver fibrosis. This prospective study investigated the ELF score in BN/AMA+ individuals and PBC patients, considering autoantibody avidity and serum levels along the years. Methods 327 samples from 35 PBC and 59 BN/AMA+ were prospectively obtained in average 3.83 (range 0.50–7.40) years apart. Samples were tested by IIF on rat-kidney (IIF-AMA), western-blot for AMA (WB-AMA), and ELISA for antibodies against pyruvate-dehydrogenase (PDC-E2), gp210, sp100 and CENP-A/B. Anti-PDC-E2 avidity was determined by 6 M urea-elution ELISA. Alkaline phosphatase (ALP), gamma glutamyl transferase (ɣGT) and ELF score were measured by automated methods. Results Along the follow-up period BN/AMA+ subjects and PBC patients presented significant increase in serum anti-PDC-E2 (mean 10.45% and 8.86% per year; respectively), anti-PDC-E2 avidity (3.02% and 4.94%/year) and ELF score (3.24% and 2.71%/year). IIF-AMA and ɣGT increased in BN/AMA+ (6.59% and 2.36%) and decreased in PBC (− 4.89%/year and − 3.88%/year). In BN/AMA+ individuals there was positive correlation of ELF with IIF-AMA titer (r = 0.465; p < 0.001) and with anti-PDC-E2 levels (r = 0.239; p < 0.001). Expansion of autoantibody targets along time occurred in 39% BN/AMA+ and 49% PBC patients. The frequency of BN/AMA+ with high probability of having established PBC increased from 7 to 14%. Conclusions BN/AMA+ individuals present an orchestrated increase in ELF score and humoral autoimmune response over time, indicating an opportunity for early therapeutic intervention and prevention in autoimmunity.

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.

scholarly journals FRI0241 INFLUENCE OF PATIENT REPORTED ‘’ARTHRITIS ACTIVITY’’ IN DETERMINING SHAQ, HAQ-DI AND COCHIN SCORES IN SYSTEMIC SCLEROSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 704.1-705
Author(s):  
K. Gjeloshi ◽  
F. Danzo ◽  
G. Abignano ◽  
A. M. Dean ◽  
F. Masini ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Health Assessment Questionnaire ◽  
Pulmonary Function Tests ◽  
Health Assessment ◽  
Joint Involvement ◽  
Wide Range ◽  
Patient Reported ◽  
Hand Disability ◽  
Assessment Questionnaire

Background:Arthritic involvement in systemic sclerosis (SSc) is a common manifestation impacting on the quality of life. A wide range of articular involvement is recognised including from arthralgia to inflammatory joint and tendon disease.Objectives:To assess the weight of patient reported ‘’arthritis activity’’ in determining Patient Reported Outcome Measures (PROMs) in an observational cohort of SSc patients.Methods:We conducted a retrospective study of 330 clinic episodes from 121 unselected patients diagnosed with SSc according to EULAR/ACR 2013 classification criteria, in annual follow-up (for a total of 165 patients/year) with Pulmonary Function Tests (PFTs), Health Assessment Questionnaire - Disability Index (HAQ-DI), Scleroderma Health Assessment Questionnaire (sHAQ) and Cochin Hand Function Score (CHFS). Hand disability index was assessed by CHFS and global disability index was assessed by HAQ and sHAQ. Patient reported arthritis activity was assessed by Visual Analogical Scale for Arthritis Activity (VAS3). Based on the median of VAS3, patients were classified in two groups and the evaluation of global and hand disability index was performed for each group. Furthermore, we assessed the correlation between the change of VAS3 and the modification of disability scores (ΔHAQ, ΔSHAQ, ΔCHFS) over 12 months of follow-up. Following analysis of distribution, Spearman or Pearson Test were used to determine correlation coefficients, as appropriate (Prism 7).Results:The median disease duration was 5 years (IQR 3-10). The median of VAS3 was 35 (IQR 2 - 66). In patients with VAS3 <35 and VAS ≥35 the HAQ-DI medians were 0.625 (IQR 0.25 - 1.5) and 1.75 (IQR 1.125-2.25) respectively, (p<0.0001); the sHAQ medians were 0.628 (IQR 0.255 - 1.114) and 1.701 (IQR 1.234-2.059), respectively, (p<0.0001); and the CHFS medians were 4 (IQR 0 – 19) and 28 (IQR 10 – 46) respectively, (p<0.0001). A significant correlation was observed between VAS3 and HAQ (r= 0.463, p<0.0001), SHAQ (r=0.651, p<0.0001), CHFS (r=0.497, p<0.0001); between ΔVAS3 and ΔSHAQ (r=0.493, p<0.0001).Conclusion:This analysis of a monocentric non-selected population supports the key role of joint involvement in determining global patient reported functional and hand disability in SSc. Severity of musculoskeletal involvement should be carefully considered when interpreting PROs in patients with SSc.References:[1]Sandler RD, Matucci-Cerinic M, Hughes M. Musculoskeletal hand involvement in systemic sclerosis. Seminars in Arthritis and Rheumatism (2019)Disclosure of Interests:None declared

scholarly journals Prevalence and time course of elevated serum levels of liver enzymes in otherwise healthy Thai infants with breast milk jaundice: a cohort study

2020 ◽  
Vol 14 (2) ◽  
pp. 67-73
Author(s):  
Pirarat Kotcharit ◽  
Voranush Chongsrisawat ◽  
Susheera Chatproedprai
Keyword(s):  
Breast Milk ◽  
Time Course ◽  
Liver Enzymes ◽  
Elevated Serum ◽  
Serum Levels ◽  
Breast Milk Jaundice ◽  
Glutamyl Transferase ◽  
A Prospective Study ◽  
Watchful Waiting Strategy

AbstractBackgroundNeonatal jaundice and elevated levels of liver enzymes are found in infants with breast milk jaundice (BMJ).ObjectivesTo determine the prevalence and duration of elevated serum levels of liver enzymes in Thai infants with BMJ.MethodsWe conducted a prospective study of Thai infants with BMJ, excluding those with pathological causes of jaundice. We measured the serum levels of total bilirubin (TB), aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), and γ-glutamyl transferase (GGT); followed infants with elevated levels; and estimated the time for levels to become normal using Kaplan–Meier analysis.ResultsWe included 42 infants (median age: 17.5 days) with BMJ, and elevated serum levels of at least 1 enzyme were found in 27 (64%) infants. We excluded 4 (10%) infants because they did not continue to be exclusively breastfed, 17 (40%) were lost to follow-up, and 21 (50%) completed the study. We found that 19 (45%) of the 42 infants had elevated GGT, 11 (26%) had elevated ALT, and 9 (21%) each had elevated AST and ALP levels. The median time for enzyme levels to normalize was 291 days (95% confidence interval [CI], 109.8 to 472.2) for ALT, 240 days (95% CI, 139.0 to 340.9) for AST, 184 days (95% CI, 4.4 to 363.6) for ALP, 120 days (95% CI, 74.6 to 164.5) for TB, and 63 days (95% CI, 61.44 to 64.6) for GGT. Infants were otherwise healthy during the follow-up.ConclusionThe prevalence of elevated serum levels of liver enzymes in Thai infants was unexpectedly high, but the levels became normal spontaneously despite continued breastfeeding, which endorses a “watchful waiting” strategy in managing asymptomatic infants with BMJ.

Long-term exposure to ambient fine particulate matter and liver enzymes in adults: a cross-sectional study in Taiwan

2019 ◽  
Vol 76 (7) ◽  
pp. 488-494 ◽  
Author(s):  
Zilong Zhang ◽  
Cui Guo ◽  
Ly-yun Chang ◽  
Yacong Bo ◽  
Changqing Lin ◽  
...  
Keyword(s):  
Particulate Matter ◽  
Liver Enzymes ◽  
Fine Particles ◽  
Fine Particulate Matter ◽  
Animal Experiments ◽  
Cross Sectional ◽  
Elevated Liver Enzymes ◽  
Wide Range ◽  
Glutamyl Transferase

ObjectivesAnimal experiments indicate that exposure to particulate matter (PM) can induce hepatotoxic effects but epidemiological evidence is scarce. We aimed to investigate the associations between long-term exposure to PM air pollution and liver enzymes, which are biomarkers widely used for liver function assessment.MethodsA cross-sectional analysis was performed among 351 852 adult participants (mean age: 40.1 years) who participated in a standard medical screening programme in Taiwan. Aspartate aminotransferase (AST), alanine aminotransferase (ALT) and γ-glutamyl transferase (GGT) levels were measured. A satellite-based spatio-temporal model was used to estimate the concentrations of ambient fine particles (PM with an aerodynamic diameter ≤2.5 µm, PM2.5) at each participant’s address. Linear and logistic regression models were used to investigate the associations between PM2.5 and the liver enzymes with adjustment for a wide range of potential confounders.ResultsAfter adjustment for confounders, every 10 µg/m3 increment in 2-year average PM2.5 concentration was associated with 0.02%(95% CI: −0.04% to 0.08%), 0.61% (95% CI: 0.51% to 0.70%) and 1.60% (95% CI: 1.50% to 1.70%) increases in AST, ALT and GGT levels, respectively. Consistently, the odds ratios of having elevated liver enzymes (>40 IU/L) per 10 µg/m3 PM2.5 increment were 1.06 (95% CI: 1.04 to 1.09), 1.09 (95% CI: 1.07 to 1.10) and 1.09 (95% CI: 1.07 to 1.11) for AST, ALT and GGT, respectively.ConclusionsLong-term exposure to PM2.5 was associated with increased levels of liver enzymes, especially ALT and GGT. More studies are needed to confirm our findings and to elucidate the underlying mechanisms.

scholarly journals Antinuclear Antibodies and Clinical Associations in a British Cohort with Limited Cutaneous Systemic Sclerosis

2011 ◽  
Vol 38 (4) ◽  
pp. 702-705 ◽  
Author(s):  
ANGELA E. GLIDDON ◽  
CAROLINE J. DORÉ ◽  
JULIET DUNPHY ◽  
ZOË BETTERIDGE ◽  
NEIL J. McHUGH ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Antinuclear Antibody ◽  
Clinical Characteristics ◽  
Antinuclear Antibodies ◽  
Prognostic Significance ◽  
Selective Reduction ◽  
Anticentromere Antibody ◽  
Specific Antibodies ◽  
Clinical Associations ◽  
Disease Specific

Objective.To assess the prevalence of disease-specific autoantibodies in patients with limited cutaneous systemic sclerosis (lcSSc).Methods.Sera from 180 patients with lcSSc were analyzed for antinuclear antibody (ANA). Clinical characteristics were compared in the presence or absence of specific autoantibodies.Results.SSc-specific antibodies were detected in 135 patients (75%). Associations were found between anticentromere antibody and age at lcSSc diagnosis, telangiectasia, reduced creatinine clearance, and selective reduction in DLCO, and between antitopoisomerase-I and pulmonary fibrosis.Conclusion.The majority of patients with lcSSc belong to distinctive serologic subsets, potentially with prognostic significance.

A Follow-up Study of Skin Thickness in a Patient with Systemic Sclerosis Using an Ultrasound Apparatus.

1996 ◽  
Vol 58 (1) ◽  
pp. 29-31
Author(s):  
Hironobu URA ◽  
Hironobu IHN ◽  
Sakae HARADA ◽  
Tsutomu FURUYA ◽  
Michiro SHIMOZUMA ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Skin Thickness ◽  
Follow Up Study

DIAGNOSTIC VALUE OF SPECIFIC ANTIBODY SYNTHESIS IN BRAIN OF PATIENTS WITH NEUROINFECTIONS

2019 ◽  
Vol 72 (8) ◽  
pp. 1437-1441
Author(s):  
Pavel Dyachenko ◽  
Igor Filchakov ◽  
Anatoly Dyachenko ◽  
Victoria Kurhanskaya
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Manifestations ◽  
Diagnostic Value ◽  
Diagnostic Challenge ◽  
Specific Antibodies ◽  
Intrathecal Synthesis ◽  
Varicella Zoster ◽  
Mrz Reaction ◽  
Wide Range

Introduction: Viral encephalitis accounts for 40-70% of all cases worldwide, central nervous system infections pose a diagnostic challenge because clinical manifestations are not typically pathognomonic for specific pathogens, and a wide range of agents can be causative. The aim: To assess the diagnostic value of intrathecal synthesis of specific antibodies in patients with inflammatory lesions of the central nervous system. Materials and methods: Within the framework of the study, two groups of 90 people in each were formed from the patients with neuroinfections admitted to our Center. Intrathecal synthesis (ITS) of total (unspecific) IgG in members of one of group (group of compare) was determined. Brain synthesis of specific antibodies (Ab) to some neurotropic pathogens (herpes simplex virus 1/2, cytomegalovirus, Epstein-Barr virus, varicella zoster virus, rubella virus, Borrelies) was studied in the second group of patients (group of interest). There were no statistically significant differences between groups by gender and age. Encephalitis and encephalomyelitis prevailed among patients of both groups Results: ITS of total IgG was established in 30 (33.3 ± 6.1 %) patients of the first group with IgG index more than 0.6 indicating on inflammatory process in CNS and no marked changes of CSF. ITS of specific Ab was determined in 23 of 90 (25.6 ± 4.6 %) patients included into group of interest. In more than half of cases Ab to several infectious agents were detected simultaneously. ITS of various specificity, in particular, to measles and rubella viruses, and VZV, known as MRZ-reaction, is characteristic of some autoimmune lesions of CNS, multiple sclerosis first of all. In fact, further research of 5 patients with MRZ-reaction confirmed their autoimmune failure of CNS. Detection of ITS in the CSF samples didn’t depend on concentration of specific Ab in serum and CSF and wasn’t followed by HEB dysfunctions which were observed with the same frequency in patients with or without ITS (13.0 % and 13.6 % respectively). Conclusion: Specific Ab synthesis to several neurotropic pathogens in the CSF of significant part of examined patients was established. Thus, diagnostic value of ITS of specific immunoglobulins seems to be limited to cases in which autoimmune damage of the CNS is suspected.

Cerebellotrigeminal Dermal Dysplasia (Gómez-López-Hernández Syndrome)

2018 ◽  
Vol 16 (05) ◽  
pp. 362-368 ◽  
Author(s):  
Federica Sullo ◽  
Agata Polizzi ◽  
Stefano Catanzaro ◽  
Selene Mantegna ◽  
Francesco Lacarrubba ◽  
...  
Keyword(s):  
De Novo ◽  
Chromosomal Rearrangements ◽  
Number Of Patients ◽  
Wide Range ◽  
Visual Problems ◽  
Neurodevelopmental Abnormalities ◽  
Clinical Triad ◽  
High Degree ◽  
Motor Handicap

Cerebellotrigeminal dermal (CTD) dysplasia is a rare neurocutaneous disorder characterized by a triad of symptoms: bilateral parieto-occipital alopecia, facial anesthesia in the trigeminal area, and rhombencephalosynapsis (RES), confirmed by cranial magnetic resonance imaging. CTD dysplasia is also known as Gómez-López-Hernández syndrome. So far, only 35 cases have been described with varying symptomatology. The etiology remains unknown. Either spontaneous dominant mutations or de novo chromosomal rearrangements have been proposed as possible explanations. In addition to its clinical triad of RES, parietal alopecia, and trigeminal anesthesia, CTD dysplasia is associated with a wide range of phenotypic and neurodevelopmental abnormalities.Treatment is symptomatic and includes physical rehabilitation, special education, dental care, and ocular protection against self-induced corneal trauma that causes ulcers and, later, corneal opacification. The prognosis is correlated to the mental development, motor handicap, corneal–facial anesthesia, and visual problems. Follow-up on a large number of patients with CTD dysplasia has never been reported and experience is limited to few cases to date. High degree of suspicion in a child presenting with characteristic alopecia and RES has a great importance in diagnosis of this syndrome.

Sign in / Sign up

Export Citation Format

Share Document