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Journal of the American Academy of... May 2018Bullous pemphigoid is an autoimmune disease that typically presents with tense bullae and severe pruritus. However, bullae can be lacking, a subtype termed nonbullous... (Review)
Review
BACKGROUND
Bullous pemphigoid is an autoimmune disease that typically presents with tense bullae and severe pruritus. However, bullae can be lacking, a subtype termed nonbullous pemphigoid.
OBJECTIVE
To summarize the reported characteristics of nonbullous pemphigoid.
METHODS
The EMBASE and MEDLINE databases were searched using "nonbullous pemphigoid" and various synonyms. Case reports and series describing nonbullous pemphigoid were included.
RESULTS
The search identified 133 articles. After selection, 39 articles were included, presenting 132 cases. Erythematous, urticarial plaques (52.3%) and papules/nodules (20.5%) were the most reported clinical features. The mean age at presentation was 74.9 years. Histopathology was commonly nonspecific. Linear depositions of IgG and/or C3 along the basement membrane zone were found by direct immunofluorescence microscopy in 93.2%. Indirect immunofluorescence on salt-split skin was positive in 90.2%. The mean diagnostic delay was 22.6 months. A minority of patients (9.8%) developed bullae during the reported follow-up.
LIMITATIONS
Results are mainly based on case reports and small case series.
CONCLUSION
Nonbullous pemphigoid is an underdiagnosed variant of pemphigoid that most often does not evolve to bullous lesions and mimics other pruritic skin diseases. Greater awareness among physicians is needed to avoid delay in diagnosis.
Topics: Age Factors; Aged; Aged, 80 and over; Autoantigens; Biopsy, Needle; Diagnosis, Differential; Enzyme-Linked Immunosorbent Assay; Female; Fluorescent Antibody Technique, Indirect; Humans; Immunoglobulin G; Immunohistochemistry; Incidence; Male; Pemphigoid, Bullous; Prognosis; Pruritus; Severity of Illness Index; Sex Factors
PubMed: 29102490
DOI: 10.1016/j.jaad.2017.10.035 -
Multiple Sclerosis and Related Disorders Jul 2023Aquaporin-4 antibody positive (AQP4+) neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are rare... (Review)
Review
A systematic literature review to examine the considerations around pregnancy in women of child-bearing age with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) or aquaporin 4 neuromyelitis optica spectrum disorder (AQP4+ NMOSD).
BACKGROUND
Aquaporin-4 antibody positive (AQP4+) neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are rare autoimmune diseases with overlapping phenotypes. Understanding their clinical manifestation prior to, during and after pregnancy may influence the management of women of child-bearing age (WOCBA) with these diseases.
METHODS
This systematic review identified relevant MEDLINE-indexed publications dated between 01 January 2011 and 01 November 2021, and congress materials from key conferences between 01 January 2019 and 01 November 2021. These were manually assessed for relevance to AQP4+ NMOSD and/or MOGAD in WOCBA, with selected data extracted and considered.
RESULTS
In total, 107 articles were retrieved and reviewed for relevancy, including 65 clinical studies. Limited evidence was found regarding a conclusive impact of either disease on female fertility, sexual function or menarche, and impact on maternal outcomes requires further investigation in both conditions to establish risk for pre-eclampsia, gestational diabetes and other complications relative to the general population. Collated data for pregnancy outcomes show clear risks in AQP4+ NMOSD to healthy delivery and a rise in annualised relapse rate postpartum that may require adaptation of treatment regimens. Disease activity appears to be attenuated during pregnancy in MOGAD patients with an increased risk of relapse during the postpartum months, but strong conclusions cannot be made due to a paucity of available data.
CONCLUSIONS
This review brings together the literature on AQP4+ NMOSD and MOGAD in WOCBA. The potential impact of pregnancy and the postpartum period on disease activity suggest a proactive management strategy early on may improve maternal and infant outcomes, but more clinical data are needed, particularly for MOGAD.
Topics: Female; Humans; Pregnancy; Aquaporin 4; Neuromyelitis Optica; Myelin-Oligodendrocyte Glycoprotein; Autoantibodies; Autoimmune Diseases
PubMed: 37224631
DOI: 10.1016/j.msard.2023.104760 -
Frontiers in Immunology 2022Anti--methyl-d-aspartate receptor encephalitis (NMDARe), a common autoimmune encephalitis, can be accompanied by demyelinating disorders, including multiple sclerosis... (Review)
Review
Anti--methyl-d-aspartate receptor encephalitis (NMDARe), a common autoimmune encephalitis, can be accompanied by demyelinating disorders, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). To compare the clinical characteristics of patients with different overlapping syndromes, we searched the PubMed database and performed a systematic review. Of the 79 patients with overlapping syndromes, 15 had MS, 18 had aquaporin-4-antibody-positive NMOSD (AQP4-Ab-positive NMOSD), and 46 had MOGAD. Compared with classical NMDARe, overlapping syndromes showed atypical symptoms, such as limb weakness, sensory disturbance, and visual impairments in addition to the main symptoms of NMDARe and a lower ratio of ovarian teratoma. Patients with MOGAD overlap were the youngest, while patients with MS and AQP4-Ab-positive NMOSD overlap tended to be older than patients with classical NMDARe. A majority of patients with NMDARe who overlapped with MS or AQP4-Ab-positive NMOSD were female, but this was not the case for patients overlapped with MOGAD. When NMDARe and demyelinating diseases occurred sequentially, the interval was the longest in patients with NMDARe overlapped with MS. A favorable outcome was observed in patients overlapping with MOGAD, but no robust comparison can be drawn with the patients overlapping with AQP4-Ab-positive NMOSD and MS regarding the small number of available data. The long-term prognosis of overlapping syndromes needs further investigation.
Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Autoantibodies; Female; Humans; Male; Multiple Sclerosis; Myelin-Oligodendrocyte Glycoprotein; Neuromyelitis Optica; Receptors, N-Methyl-D-Aspartate
PubMed: 35837405
DOI: 10.3389/fimmu.2022.857443 -
Association between the XRCC6 polymorphisms and cancer risks: a systematic review and meta-analysis.Medicine Jan 2015A number of studies have been carried out to investigate the association of X-ray repair complementing defective repair in Chinese hamster cells 6 (XRCC6) polymorphisms... (Meta-Analysis)
Meta-Analysis Review
A number of studies have been carried out to investigate the association of X-ray repair complementing defective repair in Chinese hamster cells 6 (XRCC6) polymorphisms and cancer risks, and the results remained inconsistent and inconclusive.To assess the effect of XRCC6 polymorphisms on cancer susceptibility, we conducted a meta-analysis, up to May 23rd 2014, 6267 cases with different types of tumor and 7536 controls from 20 published case-control studies. Summary odds ratios and corresponding 95% confidence intervals for XRCC6 polymorphism and cancer risk were estimated using fixed- or random-effects models when appropriate. Heterogeneity was assessed by chi-squared-based Q-statistic test, and the sources of heterogeneity were explored by subgroup analyses, logistic meta-regression analyses and Galbraith plot. Publication bias was evaluated by Begg funnel plot and Egger test. Sensitivity analyses were also performed.The rs2267437 polymorphism was associated with a significant increase in risks of overall cancers, breast cancer, renal cell carcinoma and hepatocellular carcinoma, and it could increase the cancer risk in Asian population; the rs5751129 polymorphism could increase the cancer risk in overall cancers; the rs132770 polymorphism was associated with the increased renal cell carcinoma risk; furthermore, the rs132793 polymorphism could decrease breast cancer risk and increase risks in "other cancers".Overall, the results provided evidences that the single nucleotide polymorphisms in XRCC6 promoter region might play different roles in various cancers, indicating different cancers have different tumorigenesis mechanisms. Our studies may perhaps supplement for the disease monitoring of cancers in the future, and additional studies to determine the exact molecular mechanism might provide us with interventions to protect the susceptible subgroups.
Topics: Animals; Antigens, Nuclear; Case-Control Studies; DNA-Binding Proteins; Genetic Heterogeneity; Genetic Predisposition to Disease; Humans; Ku Autoantigen; Neoplasms
PubMed: 25569644
DOI: 10.1097/MD.0000000000000283 -
Seminars in Arthritis and Rheumatism Apr 2020ImmunoglobulinG4-related disease (IgG4-RD) is a recently recognized disease and, as such, there is a pressing need to identify biomarkers for diagnosis, monitoring...
OBJECTIVE
ImmunoglobulinG4-related disease (IgG4-RD) is a recently recognized disease and, as such, there is a pressing need to identify biomarkers for diagnosis, monitoring disease activity, and predicting prognosis and response to therapy. Here, we review the recent development and identification of biomarkers for IgG4-RD.
METHODS
Through extensive literature review and analysis, we updated the biomarkers for IgG4-RD and further put forward our own viewpoints.
RESULTS
In addition to traditional biomarkers, such as serum IgG4 concentration and typical histological characteristics, several novel indicators, including IgG2, serum soluble IL-2 receptor (sIL2R), and cc-chemokine ligand 18 (CCL18), indicate inflammation and fibrosis and can be used to accurately diagnose and predict treatment response. Studies to identify target autoantigens in IgG4-RD have shed light on the unmet need for biomarkers that can identify this disorder. Additionally, both serological and histopathologic immune cells involved in antigen-induced responses, innate immune cells (macrophages, mast cells, and the I-IFN/ IL-33 pathway), as well as subsequent acquired immune cells (T and B cell subsets), may also serve as new biomarkers for IgG4-RD. Since IgG4-RD often clinically manifests with multiple organs involvement, non-invasive PET-CT can improve diagnosis and antidiastole levels.
CONCLUSION
These novel biomarkers provide information to help diagnose IgG4-RD, monitor disease activity, as well as predict prognosis and response to therapy.
Topics: Biomarkers; Chemokines, CC; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Receptors, Interleukin-2
PubMed: 31280934
DOI: 10.1016/j.semarthrit.2019.06.018 -
Biomedicines Jun 2022Autoimmune pancreatitis (AIP) is a rare etiological type of chronic pancreatitis. The clinical and radiological presentation of AIP often resembles that of pancreatic... (Review)
Review
Autoimmune pancreatitis (AIP) is a rare etiological type of chronic pancreatitis. The clinical and radiological presentation of AIP often resembles that of pancreatic cancer. Identifying non-invasive markers for their early distinction is of utmost importance to avoid unnecessary surgery or a delay in steroid therapy. Thus, this systematic review was conducted to revisit all current evidence on the clinical utility of different serum biomarkers in diagnosing AIP, distinguishing AIP from pancreatic cancer, and predicting disease course, steroid therapy response, and relapse. A systematic review was performed for articles published up to August 2021 by searching electronic databases such as MEDLINE, Web of Science, and EMBASE. Among 5123 identified records, 92 studies were included in the qualitative synthesis. Apart from immunoglobulin (Ig) G4, which was by far the most studied biomarker, we identified autoantibodies against the following: lactoferrin, carboanhydrase II, plasminogen-binding protein, amylase-α2A, cationic (PRSS1) and anionic (PRSS2) trypsinogens, pancreatic secretory trypsin inhibitor (PSTI/SPINK1), and type IV collagen. The identified novel autoantigens were laminin 511, annexin A11, HSP-10, and prohibitin. Other biomarkers included cytokines, decreased complement levels, circulating immune complexes, -glycan profile changes, aberrant miRNAs expression, decreased IgA and IgM levels, increased IgE levels and/or peripheral eosinophil count, and changes in apolipoprotein isoforms levels. To our knowledge, this is the first systematic review that addresses biomarkers in AIP. Evolving research has recognized numerous biomarkers that could help elucidate the pathophysiological mechanisms of AIP, bringing us closer to AIP diagnosis and its preoperative distinction from pancreatic cancer.
PubMed: 35884816
DOI: 10.3390/biomedicines10071511 -
Frontiers in Immunology 2022NETosis is a form of neutrophil cell death during which extracellular fibrillary structures composed of cytosolic and granule proteins assembled on scaffolds of...
NETosis is a form of neutrophil cell death during which extracellular fibrillary structures composed of cytosolic and granule proteins assembled on scaffolds of decondensed chromatin, called neutrophil extracellular traps (NETs), are released. NETs normally contribute to host immune defense. Accumulating evidence implicates aberrant NET production and/or reduced NET clearance, along with alterations of molecules involved in NETosis pathway, in humans and animals with lupus. The extruded nuclear antigens released by NET are a source of autoantigens, which can contribute to the breakdown of self-tolerance in lupus. Excessive NET can also promote the production of pro-inflammatory cytokine interferon-α, elicit direct cytotoxic effect on various renal cells, and cause capillary necrosis and podocyte loss. Additionally, NET can induce endothelial-to-mesenchymal transdifferentiation, which can promote activated myofibroblasts leading to extracellular matrix production. Thus, aberrant NETosis can play diverse roles, including autoantibody production, inflammation, and tissue damage, at different stages of lupus pathogenesis. Evidence suggests that treatments currently used in lupus may reduce NETosis, suggesting a potential utility of targeting NETosis to treat lupus. In fact, several approaches are being experimented to therapeutically target pathways of NETosis. Future studies should precisely delineate distinct roles of NETosis at different stages of lupus pathogenesis in humans, which would offer a rational basis for NETosis-targeting treatments in the clinic.
Topics: Animals; Autoantigens; Cell Death; Extracellular Traps; Inflammation; Neutrophils
PubMed: 35686129
DOI: 10.3389/fimmu.2022.895216 -
BMC Pregnancy and Childbirth Jul 2020Thyroid dysfunction during pregnancy is associated with adverse outcomes for both mother and fetus. The present meta-analysis was conducted to evaluate thyroid... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Thyroid dysfunction during pregnancy is associated with adverse outcomes for both mother and fetus. The present meta-analysis was conducted to evaluate thyroid dysfunction in Iranian pregnant women.
METHODS
We registered this review at PROSPERO (registration number: CRD42020166655). The research steps in this systematic review and meta-analysis were performed according to the MOOSE protocol, and finally, reports were provided based on the PRISMA guidelines. The literature search was performed in October 2019 using the international online databases, including Web of Science, Ovid, Science Direct, Scopus, EMBASE, PubMed/Medline, Cochrane Library, EBSCO, CINAHL, Google Scholar as well as national databases were reviewed. Data were extracted after applying the inclusion and exclusion criteria and qualitative evaluation of the studies. I index and Q test were used to assess differences in studies. All analyses were performed using Comprehensive Meta-Analysis Software. P-value less than 0.05 was considered statistically significant. We identified 1261 potential articles from the databases, and 426 articles remained after removing the duplicate and unrelated studies. After evaluating the full text, 52 articles were removed.
RESULTS
Finally, 19 eligible studies including 17,670 pregnant women included for meta-analysis. The prevalence of thyroid dysfunction in Iranian pregnant women was 18.10% (95%CI: 13.89-23.25). The prevalence of hypothyroidism, clinical hypothyroidism, and subclinical hypothyroidism in Iranian pregnant women was respectively estimated to be 13.01% (95%CI: 9.15-18.17), 1.35% (95%CI: 0.97-1.86) and 11.90% (95%CI: 7.40-18.57). The prevalence of hyperthyroidism, clinical hyperthyroidism, and subclinical hyperthyroidism in Iranian pregnant women was respectively estimated to be 3.31% (95%CI: 1.62-6.61), 1.06% (95%CI: 0.61-1.84) and 2.56% (95%CI: 0.90-7.05). The prevalence of anti-thyroperoxidase antibody was estimated to be 11.68% (95%CI: 7.92-16.89).
CONCLUSION
The results of this meta-analysis showed a high prevalence of thyroid disorders, especially hypothyroidism. The decision to recommend thyroid screening during pregnancy for all women is still under debate, because the positive effects of treatment on pregnancy outcomes must be ensured. On the other hand, evidence about the effect of thyroid screening and treatment of thyroid disorders on pregnancy outcomes is still insufficient. Nevertheless, a large percentage of general practitioners, obstetricians and gynecologists perform screening procedures in Iran.
Topics: Adult; Autoantigens; Female; Humans; Hypothyroidism; Iodide Peroxidase; Iran; Iron-Binding Proteins; Pregnancy; Pregnancy Complications; Thyroid Diseases
PubMed: 32664874
DOI: 10.1186/s12884-020-03040-5 -
Archives of Dermatological Research Jan 2018Bullous pemphigoid (BP) is the most common autoimmune skin disease of blistering character. The underlying pathophysiological mechanism involves an immune attack,... (Review)
Review
Bullous pemphigoid (BP) is the most common autoimmune skin disease of blistering character. The underlying pathophysiological mechanism involves an immune attack, usually by IgG class autoantibodies, on the autoantigen BP 180/BPAg2, which is a type XVII collagen (COL17) protein acting as the adhesion molecule between the epidermis and the basement membrane of the dermis. About 40 years ago, following consistent findings of elevated total serum IgE levels in BP patients, it was hypothesized that IgE may be involved in the pathophysiology of BP. Our objective was to determine whether there is strong evidence for an association between IgE class autoantibodies and the clinical severity or phenotype of BP. Three databases were searched for relevant studies and appropriate exclusion and inclusion criteria were applied. Data was extracted and assessed in relation to the study questions concerning the clinical significance of IgE autoantibodies in BP. Nine studies found that anti-BP180 autoantibodies of IgE class are associated with increased severity of BP, whereas two studies did not find such an association. The number of studies which found an association between higher IgE autoantibody levels and the erythematous urticarial phenotype of BP (5) was equal in number to the studies which found no such association (5). In conclusion, higher serum IgE autoantibody levels are associated with more severe clinical manifestations of BP. There is insufficient evidence to support higher IgE autoantibody levels being associated with specific clinical phenotypes of BP.
Topics: Autoantibodies; Humans; Immunoglobulin E; Pemphigoid, Bullous; Phenotype; Severity of Illness Index
PubMed: 29071428
DOI: 10.1007/s00403-017-1789-1 -
Cureus May 2024Autoimmune hepatitis (AIH) is a hepatocellular disorder thought to be caused by an immune system that cannot tolerate autoantigens specific to hepatocytes. This study... (Review)
Review
Autoimmune hepatitis (AIH) is a hepatocellular disorder thought to be caused by an immune system that cannot tolerate autoantigens specific to hepatocytes. This study aims to evaluate the efficacy of using corticosteroids (prednisolone and azathioprine) as a combination therapy in treating AIH. This study aims to synthesize and analyze existing evidence to inform clinical practices concerning the overall clinical efficacy of this treatment approach in managing AIH. A comprehensive search was conducted across multiple online databases and search engines, including PubMed, Google Scholar, ScienceDirect, Medline, and Embase. RevMan 5.4 software was used for meta-analysis, with forest plots created for each outcome. Thirteen studies were included in this systematic review and meta-analysis. The results indicate that the combination of prednisolone and azathioprine for treating AIH leads to less recurrence and better disease control.
PubMed: 38854256
DOI: 10.7759/cureus.60049