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Experimental Dermatology Jul 2023Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of... (Review)
Review
A systematic review on efficacy, safety and treatment durability of intravenous immunoglobulin in autoimmune bullous dermatoses: Special focus on indication and combination therapy.
Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of intravenous immunoglobulin (IVIg) as an alternative treatment should be explored to systematically review the available literature regarding treatment outcomes with IVIg in AIBD patients. The predefined search strategy was incorporated into the following database, MEDLINE/PubMed, Embase, Scopus and Web of Science on 18 July 2022. Sixty studies were enrolled using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. The use of IVIg alone or combined with rituximab was reported in 500 patients with pemphigus, 82 patients with bullous pemphigoid, 146 patients with mucous membranes pemphigoid and 19 patients with epidermolysis bullosa acquisita. Disease remission with IVIg therapy and RTX + IVIg combination therapy were recorded as 82.8% and 86.7% in pemphigus, 88.0% and 100% in bullous pemphigoid and 91.3% and 75.0% in mucous membrane pemphigoid, respectively. In epidermolysis bullosa acquisita, treatment with IVIg led to 78.6% disease remission; no data were available regarding the treatment with RTX + IVIg in this group of patients. Among all the included patients, 37.5% experienced at least one IVIg-related side effect; the most common ones were headaches, fever/chills and nausea/vomiting. The use of IVIg with or without rituximab had a favourable clinical response in patients with AIBDs. IVIg has no major influence on the normal immune system, which makes its utilization for patients with AIBDs reasonable.
Topics: Humans; Pemphigoid, Bullous; Immunoglobulins, Intravenous; Epidermolysis Bullosa Acquisita; Pemphigus; Rituximab; Autoimmune Diseases; Skin Diseases, Vesiculobullous; Pemphigoid, Benign Mucous Membrane
PubMed: 37150538
DOI: 10.1111/exd.14829 -
Frontiers in Immunology 2021Numerous cases of the coronavirus disease 2019 (COVID-19) with autoimmune and rheumatic manifestations have been reported. Despite the available reviews that summarized...
Numerous cases of the coronavirus disease 2019 (COVID-19) with autoimmune and rheumatic manifestations have been reported. Despite the available reviews that summarized its autoimmune/rheumatic manifestations, a systematic approach is still lacking. Therefore, we conducted a comprehensive systematic review in order to give an overview upon these rare but clinically significant manifestations. We performed a literature search of PubMed and EMBASE as of October 9, 2020. All articles relevant to either systemic or organ-specific autoimmune and rheumatic manifestations potentially associated with COVID-19 were collected. The reviewed literature were limited to adults ≥18 years. Although most of the existing evidence was based on case reports or case series without a long-term follow-up, a variety of autoimmune/rheumatic manifestations were associated with COVID-19. The manifestations that have a consistent association with COVID-19 include autoimmune cytopenia, cutaneous vasculitis, encephalitis, and Guillain-Barre syndrome. Such association is conflicting as regards to antiphospholipid syndrome, hemophagocytic lymphohistiocytosis, and myasthenia gravis. Our systematic review indicated the potential of the COVID-19 virus to trigger a myriad of autoimmune and rheumatic manifestations, which should be considered amid global efforts to combat COVID-19.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Autoimmune Diseases; Autoimmunity; COVID-19; Child; Child, Preschool; Female; Host-Pathogen Interactions; Humans; Male; Middle Aged; Rheumatic Diseases; SARS-CoV-2; Young Adult
PubMed: 33777042
DOI: 10.3389/fimmu.2021.645013 -
Frontiers in Immunology 2022To evaluate the safety and efficacy of fecal microbiota transplantation for autoimmune diseases and autoinflammatory diseases. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To evaluate the safety and efficacy of fecal microbiota transplantation for autoimmune diseases and autoinflammatory diseases.
METHODS
Relevant literature was retrieved from the PubMed database, Embase database, Cochrane Library database, etc. The search period is from the establishment of the database to January 2022. The outcomes include clinical symptoms, improvement in biochemistry, improvement in intestinal microbiota, improvement in the immune system, and adverse events. Literature screening and data extraction were independently carried out by two researchers according to the inclusion and exclusion criteria, and RevMan 5.3 software was used for statistics and analysis.
RESULTS
Overall, a total of 14 randomized controlled trials (RCTs) involving six types of autoimmune diseases were included. The results showed the following. 1) Type 1 diabetes mellitus (T1DM): compared with the autologous fecal microbiota transplantation (FMT) group (control group), the fasting plasma C peptide in the allogenic FMT group at 12 months was lower. 2) Systemic sclerosis: at week 4, compared with one of two placebo controls, three patients in the experimental group reported a major improvement in fecal incontinence. 3) Ulcerative colitis, pediatric ulcerative colitis, and Crohn's disease: FMT may increase clinical remission, clinical response, and endoscopic remission for patients with ulcerative colitis and increase clinical remission for patients with Crohn's disease. 4) Psoriatic arthritis: there was no difference in the ratio of ACR20 between the two groups.
CONCLUSION
Based on current evidence, the application of FMT in the treatment of autoimmune diseases is effective and relatively safe, and it is expected to be used as a method to induce remission of active autoimmune diseases.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021235055, identifier CRD42021235055.
Topics: Autoimmune Diseases; C-Peptide; Child; Colitis, Ulcerative; Crohn Disease; Fecal Microbiota Transplantation; Hereditary Autoinflammatory Diseases; Humans
PubMed: 36248877
DOI: 10.3389/fimmu.2022.944387 -
European Radiology Dec 2023To determine informational CT findings for distinguishing autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to review their diagnostic... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To determine informational CT findings for distinguishing autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to review their diagnostic accuracy.
METHODS
A systematic and detailed literature review was performed through PubMed, EMBASE, and the Cochrane library. Similar descriptors to embody the identical image finding were labeled as a single CT characteristic. We calculated the pooled diagnostic odds ratios (DORs) of each CT characteristic using a bivariate random-effects model.
RESULTS
A total of 145 various descriptors from 15 studies (including 562 AIP and 869 PDAC patients) were categorized into 16 CT characteristics. According to the pooled DOR, 16 CT characteristics were classified into three groups (suggesting AIP, suggesting PDAC, and not informational). Seven characteristics suggesting AIP were diffuse pancreatic enlargement (DOR, 48), delayed homogeneous enhancement (DOR, 46), capsule-like rim (DOR, 34), multiple pancreatic masses (DOR, 16), renal involvement (DOR, 15), retroperitoneal fibrosis (DOR, 13), and bile duct involvement (DOR, 8). Delayed homogeneous enhancement showed a pooled sensitivity of 83% and specificity of 85%. The other six characteristics showed relatively low sensitivity (12-63%) but high specificity (93-99%). Four characteristics suggesting PDAC were discrete pancreatic mass (DOR, 23), pancreatic duct cutoff (DOR, 16), upstream main pancreatic duct dilatation (DOR, 8), and upstream parenchymal atrophy (DOR, 7).
CONCLUSION
Eleven CT characteristics were informational to distinguish AIP from PDAC. Diffuse pancreatic enlargement, delayed homogeneous enhancement, and capsule-like rim suggested AIP with the highest DORs, whereas discrete pancreatic mass suggested PDAC. However, pooled sensitivities of informational CT characteristics were moderate.
CLINICAL RELEVANCE STATEMENT
This meta-analysis underscores eleven distinctive CT characteristics that aid in differentiating autoimmune pancreatitis from pancreatic adenocarcinoma, potentially preventing misdiagnoses in patients presenting with focal/diffuse pancreatic enlargement.
KEY POINTS
• Diffuse pancreatic enlargement (pooled diagnostic odds ratio [DOR], 48), delayed homogeneous enhancement (46), and capsule-like rim (34) were CT characteristics suggesting autoimmune pancreatitis. • The CT characteristics suggesting autoimmune pancreatitis, except delayed homogeneous enhancement, had a general tendency to show relatively low sensitivity (12-63%) but high specificity (93-99%). • Discrete pancreatic mass (pooled diagnostic odds ratio, 23) was the CT characteristic suggesting pancreatic ductal adenocarcinoma with the highest pooled DORs.
Topics: Humans; Pancreatic Neoplasms; Autoimmune Pancreatitis; Pancreatitis; Adenocarcinoma; Tomography, X-Ray Computed; Autoimmune Diseases; Carcinoma, Pancreatic Ductal; Diagnosis, Differential
PubMed: 37466708
DOI: 10.1007/s00330-023-09959-5 -
Liver International : Official Journal... Nov 2023Patients with some chronic liver diseases have increased risk of diabetes. Whether this is also the case for patients with autoimmune liver diseases is unknown. The... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND AIMS
Patients with some chronic liver diseases have increased risk of diabetes. Whether this is also the case for patients with autoimmune liver diseases is unknown. The study aimed to calculate risk and worldwide prevalence of diabetes in patients with autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC).
METHODS
We performed a case-control study using data from the United Kingdom Biobank (UKB) and compared frequency of type 1 diabetes (T1D) and type 2 diabetes (T2D) in AIH and PBC with age-, sex-, BMI- and ethnicity-matched controls. Next, we performed a systematic review and proportional meta-analysis searching PubMed, Embase, Cochrane Library and Web of Science (inception to 1 May 2022 [AIH]; 20 August 2022 [PBC]; 11 November 2022 [PSC]). The pooled prevalence of diabetes was calculated using an inverse method random effects model.
RESULTS
Three hundred twenty-eight AIH patients and 345 PBC patients were identified in UKB and risk of T1D and T2D significantly increased compared with matched controls. Our systematic search identified 6914 records including the UKB study. Of these, 77 studies were eligible for inclusion comprising 36 467, 39 924 and 4877 individuals with AIH, PBC and PSC, respectively. The pooled prevalence of T1D was 3.8% (2.6%-5.7%), 1.7% (0.9%-3.1%), 3.1% (1.9%-4.8%) and of T2D 14.8% (11.1%-19.5%), 18.1% (14.6%-22.2%), 6.3% (2.8%-13.3%) in patients with AIH, PBC and PSC, respectively.
CONCLUSIONS
Patients with autoimmune liver diseases have increased risk of diabetes. Increased awareness of diabetes risk in patients with autoimmune liver diseases is warranted.
Topics: Humans; Liver Cirrhosis, Biliary; Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Case-Control Studies; Propensity Score; Liver Diseases; Autoimmune Diseases; Hepatitis, Autoimmune; Cholangitis, Sclerosing
PubMed: 37752719
DOI: 10.1111/liv.15720 -
Annals of the Rheumatic Diseases Jan 2023Recent insights supporting the safety of live-attenuated vaccines and novel studies on the immunogenicity of vaccinations in the era of biological disease-modifying...
OBJECTIVES
Recent insights supporting the safety of live-attenuated vaccines and novel studies on the immunogenicity of vaccinations in the era of biological disease-modifying antirheumatic drugs in paediatric patients with autoimmune/inflammatory rheumatic diseases (pedAIIRD) necessitated updating the EULAR recommendations.
METHODS
Recommendations were developed using the EULAR standard operating procedures. Two international expert committees were formed to update the vaccination recommendations for both paediatric and adult patients with AIIRD. After a systematic literature review, separate recommendations were formulated for paediatric and adult patients. For pedAIIRD, six overarching principles and seven recommendations were formulated and provided with the level of evidence, strength of recommendation and Task Force level of agreement.
RESULTS
In general, the National Immunisation Programmes (NIP) should be followed and assessed yearly by the treating specialist. If possible, vaccinations should be administered prior to immunosuppressive drugs, but necessary treatment should never be postponed. Non-live vaccines can be safely given to immunosuppressed pedAIIRD patients. Mainly, seroprotection is preserved in patients receiving vaccinations on immunosuppression, except for high-dose glucocorticoids and B-cell depleting therapies. Live-attenuated vaccines should be avoided in immunosuppressed patients. However, it is safe to administer the measles-mumps-rubella booster and varicella zoster virus vaccine to immunosuppressed patients under specific conditions. In addition to the NIP, the non-live seasonal influenza vaccination should be strongly considered for immunosuppressed pedAIIRD patients.
CONCLUSIONS
These recommendations are intended for paediatricians, paediatric rheumatologists, national immunisation agencies, general practitioners, patients and national rheumatology societies to attain safe and effective vaccination and optimal infection prevention in immunocompromised pedAIIRD patients.
Topics: Adult; Humans; Child; Vaccines, Attenuated; Rheumatic Diseases; Vaccination; Immunosuppressive Agents; Antirheumatic Agents; Autoimmune Diseases
PubMed: 35725297
DOI: 10.1136/annrheumdis-2022-222574 -
Clinical Rheumatology Oct 2023Secondary autoimmune inner ear disease (AIED) is often bilateral and asymmetric in patients presenting with audiovestibular symptoms due to a systemic autoimmune... (Meta-Analysis)
Meta-Analysis Review
Secondary autoimmune inner ear disease (AIED) is often bilateral and asymmetric in patients presenting with audiovestibular symptoms due to a systemic autoimmune disease. This systematic review and meta-analysis are aimed at identifying and highlighting patterns in prevalence of vestibular dysfunction, symptom presentation, and diagnostic methods in extant literature by combining clinical context from case reports with quantitative analyses from cohort studies. Screening of articles by title, abstract, and full text was completed by four reviewers (K.Z., A.L., S.C., and S.J.). In this study, we grouped secondary AIED and systemic autoimmune diseases by pathophysiologic mechanism: (1) connective tissue disease (CTD), (2) vasculitides (VAS), (3) systemic inflammatory disorders (SID), and (4) other immune-mediated disorders (OIMD). The search for AIED disease identified 120 articles (cohorts and case reports) that met the final inclusion criteria. All 120 were included in the qualitative review, and 54 articles were included for meta-analysis. Of these 54 articles, 22 included a control group (CwC). Ninety individual cases or patient presentations from 66 articles were included for analysis in addition to the 54 cohort articles. Secondary AIED does not have a diagnostic algorithm for managing vestibular symptoms. The management of audiovestibular symptoms requires close collaboration between otolaryngologists and rheumatologists to preserve end-organ function of the ear. To improve our ability to understand the impact on the vestibular system, vestibular clinicians need to develop a standardized reporting method. Clinical presentation should frequently be paired with vestibular testing to contextually investigate symptom severity and provide higher quality care.
Topics: Humans; Ear Diseases; Autoimmune Diseases
PubMed: 37380912
DOI: 10.1007/s10067-023-06674-w -
European Radiology Oct 2022To identify reliable MRI features for differentiating autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to summarize their diagnostic... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To identify reliable MRI features for differentiating autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to summarize their diagnostic accuracy.
METHODS
We conducted a systematic literature review and meta-analysis using PubMed, EMBASE, and the Cochrane Library to identify original articles published between January 2006 and July 2021. The pooled diagnostic accuracy, including the diagnostic odds ratios (DORs) with 95% confidence intervals (CIs) of the identified features, was calculated using a bivariate random effects model.
RESULTS
Twelve studies were included, and 92 overlapping descriptors were subsumed under 16 MRI features. Ten features favoring AIP were diffuse enlargement (DOR, 75; 95% CI, 9-594), capsule-like rim (DOR, 52; 95% CI, 20-131), multiple main pancreatic duct (MPD) strictures (DOR, 47; 95% CI, 17-129), homogeneous delayed enhancement (DOR, 46; 95% CI, 21-104), low apparent diffusion coefficient value (DOR, 30), speckled enhancement (DOR, 30), multiple pancreatic masses (DOR, 29), tapered narrowing of MPD (DOR, 15), penetrating duct sign (DOR, 14), and delayed enhancement (DOR, 13). Six features favoring PDAC were target type enhancement (DOR, 41; 95% CI, 11-158), discrete pancreatic mass (DOR, 35; 95% CI, 15-80), upstream MPD dilatation (DOR, 13), peripancreatic fat infiltration (DOR, 10), upstream parenchymal atrophy (DOR, 5), and vascular involvement (DOR, 3).
CONCLUSION
This study identified 16 informative MRI features to differentiate AIP from PDAC. Among them, diffuse enlargement, capsule-like rim, multiple MPD strictures, and homogeneous delayed enhancement favored AIP with the highest DORs, whereas discrete mass and target type enhancement favored PDAC.
KEY POINTS
• The MRI features with the highest pooled diagnostic odds ratios (DORs) for autoimmune pancreatitis were diffuse enlargement of the pancreas (75), capsule-like rim (52), multiple strictures of the main pancreatic duct (47), and homogeneous delayed enhancement (46). • The MRI features with the highest pooled DORs for pancreatic ductal adenocarcinoma were target type enhancement (41) and discrete pancreatic mass (35).
Topics: Adenocarcinoma; Autoimmune Diseases; Autoimmune Pancreatitis; Carcinoma, Pancreatic Ductal; Constriction, Pathologic; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Pancreatic Neoplasms; Retrospective Studies
PubMed: 35486167
DOI: 10.1007/s00330-022-08816-1 -
European Journal of Neurology Jul 2024This study was undertaken to provide a comprehensive review of neuroimaging characteristics and corresponding clinical phenotypes of autoimmune glial fibrillary acidic... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
This study was undertaken to provide a comprehensive review of neuroimaging characteristics and corresponding clinical phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A), a rare but severe neuroinflammatory disorder, to facilitate early diagnosis and appropriate treatment.
METHODS
A PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis)-conforming systematic review and meta-analysis was performed on all available data from January 2016 to June 2023. Clinical and neuroimaging phenotypes were extracted for both adult and paediatric forms.
RESULTS
A total of 93 studies with 681 cases (55% males; median age = 46, range = 1-103 years) were included. Of these, 13 studies with a total of 535 cases were eligible for the meta-analysis. Clinically, GFAP-A was often preceded by a viral prodromal state (45% of cases) and manifested as meningitis, encephalitis, and/or myelitis. The most common symptoms were headache, fever, and movement disturbances. Coexisting autoantibodies (45%) and neoplasms (18%) were relatively frequent. Corticosteroid treatment resulted in partial/complete remission in a majority of cases (83%). Neuroimaging often revealed T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities (74%) as well as perivascular (45%) and/or leptomeningeal (30%) enhancement. Spinal cord abnormalities were also frequent (49%), most commonly manifesting as longitudinally extensive myelitis. There were 88 paediatric cases; they had less prominent neuroimaging findings with lower frequencies of both T2/FLAIR hyperintensities (38%) and contrast enhancement (19%).
CONCLUSIONS
This systematic review and meta-analysis provide high-level evidence for clinical and imaging phenotypes of GFAP-A, which will benefit the identification and clinical workup of suspected cases. Differential diagnostic cues to distinguish GFAP-A from common clinical and imaging mimics are provided as well as suitable magnetic resonance imaging protocol recommendations.
Topics: Humans; Astrocytes; Autoantibodies; Autoimmune Diseases of the Nervous System; Glial Fibrillary Acidic Protein; Neuroimaging; Neuroinflammatory Diseases; Phenotype
PubMed: 38506182
DOI: 10.1111/ene.16284 -
RMD Open Dec 2023Refractory autoimmune diseases remain a significant challenge in clinical practice and new therapeutic options are needed. This systematic review evaluates the existing...
OBJECTIVE
Refractory autoimmune diseases remain a significant challenge in clinical practice and new therapeutic options are needed. This systematic review evaluates the existing reported data on the CD38-targeting antibody daratumumab as a new therapeutic approach in autoantibody-mediated autoimmune diseases.
METHODS
A protocolised systematic literature review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed. Two databases (Medline and Embase) were searched for suitable studies. Usage of daratumumab in non-oncological or non-transplantation associated diseases with autoimmune pathophysiology was analysed including patient characteristics, therapeutic regimen, adverse events and patient outcome.
RESULTS
38 publications reporting the clinical course of 83 patients met the inclusion criteria. Daratumumab usage was reported in therapy-refractory cases (median of 5 different previous therapies) in 24 different autoimmune diseases. The median number of applications of daratumumab was 4, mainly via intravenous applications (87%). Concomitant treatment included glucocorticoids in 64% of patients, intravenous immunoglobulins (33%) and rituximab (17%). Remission or improvement of disease was reported in 81% of patients. Autoantibody depletion or reduction was stated in 52% of patients. Death occurred in three patients (3%). Adverse events were reported in 45% of patients including application-associated reaction (20%), infection (19%) and hypogammaglobulinaemia (33%).
CONCLUSION
Targeting CD38 via daratumumab is a new promising therapeutic option in therapy refractory autoimmune diseases. Efficacy as well as optimal therapeutic regimen and management or prevention of adverse events require further investigation. Therefore, systematic clinical trials of this therapeutic approach are needed.
Topics: Humans; Antibodies, Monoclonal; Rituximab; Autoimmune Diseases; Autoantibodies
PubMed: 38101819
DOI: 10.1136/rmdopen-2023-003604