The significant IgG4 infiltrate in autoimmune hepatitis is associated with a greater ductular reaction and more advanced liver disease.

BACKGROUND: Sclerosing cholangitis is the typical IgG4-related disease digestive involvement. However, the role of the IgG4 liver expression in autoimmune hepatitis remains unknown. AIMS: to assess whether the expression of IgG4 plasma cells in patients with autoimmune hepatitis (AIH) was associated with different outcomes. METHODS: Retrospective study including patients diagnosed with AIH by biopsy from January-2009 to June-2021. At least mild IgG4 expression (>10 IgG4+-plasma cells per field) was considered as significant. RESULTS: 85 patients with AIH were included. Overall, 58.8% were women, mean age 54 years. Nine (10.6%) presented cirrhosis at diagnosis. Fifteen (17.6%) had significant IgG4 liver expression. Patients with IgG4 infiltrate were older (p = 0.021), presented liver cirrhosis more frequently (33.3% vs. 5.7%, p = 0.007), greater IgG plasma values (p = 0.008) and atypical ANCAs (p = 0.086); ductular reaction was also more common (p = 0.009). Complete remission rate was similar regardless of the IgG4 infiltrate. Time to corticosteroids discontinuation was longer in subjects with IgG4 infiltrate (p = 0.068), but second-line therapy tended to be less frequent (p = 0.187). CONCLUSION: Significant IgG4 liver infiltrate in patients with autoimmune hepatitis is associated with more advanced liver disease. The greater ductular reaction mediated by the IgG4 infiltrate may be the cause for this finding, though this finding should be prospectively assessed.

Development of an algorithm for IgG4-related disease management.

OBJECTIVES: IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory condition affecting multiple organs lacking standardized management. In this article, we review the evidence available to provide European expert-based statements on the management of IgG4-RD which were integrated in a final algorithm. METHODS: A panel of nine European experts in IgG4-RD from different specialties was asked to elaborate a set of consensus statements through a Delphi exercise. Three rounds of survey were taken. Consensus was reached when ≥75% of the responders agreed with a statement. RESULTS: Thirty-one statements on induction treatment, maintenance treatment, non-pharmacological treatment, and general considerations were assessed. Patients should be treated promptly in situations when there is an immediate organ threatened, or when organ damage is anticipated. Glucocorticoids (GC) are considered the first line of treatment and should be progressively tapered. Maintenance treatment is recommended for patients with high disease activity or with risk factors for relapse. Rituximab is effective for induction and maintenance of remission, but its use can be limited by economics. Low dose GC with or without GC-sparing agents can be used for maintenance therapy. Stenting or surgery should be ancillary to pharmacological treatment. Follow up should be based on physical examination, blood works, and imaging studies. Furthermore, it should be tailored on individual patient clinical history. 18-fluorodeoxyglucose positron emission tomography/computerized tomography may provide additional information over other imaging modalities. CONCLUSIONS: These new statements and algorithm reached a high degree of agreement and may help guiding the clinical management of IgG4-RD.

Radiography-based triage for COVID-19 in the Emergency Department in a Spanish cohort of patients.

Background: Strategies to determine who could be safely discharged home from the Emergency Department (ED) in COVID-19 are needed to decongestion healthcare systems. Objectives: To describe the outcomes of an ED triage system for non-severe patients with suspected COVID-19 and possible pneumonia based on chest X-ray (CXR) upon admission. Material and methods: Retrospective, single-center study performed in Barcelona (Spain) during the COVID-19 peak in March-April 2020. Patients with COVID-19 symptoms and potential pneumonia, without respiratory insufficiency, with priority class IV-V (Andorran triage model) had a CXR upon admission. This approach tried to optimize resource use and to facilitate discharges. The results after adopting this organizational approach are reported. Results: We included 834 patients, 53% were female. Most patients were white (66%) or Hispanic (27%). CXR showed pneumonia in 523 (62.7%). Compared to those without pneumonia, patients with pneumonia were older (55 vs 46.6 years old) and had a higher Charlson comorbidity index (1.9 vs 1.3). Patients with pneumonia were at a higher risk for a combined outcome of admission and/or death (91 vs 12%). Death rates tended to be numerically higher in the pneumonia group (10 vs 1). Among patients without pneumonia in the initial CXR, 10% reconsulted (40% of them with new pneumonia). Conclusion: CXR identified pneumonia in a significant number of patients. Those without pneumonia were mostly discharged. Mortality among patients with an initially negative CXR was low. CXR triage for pneumonia in non-severe COVID-19 patients in the ED can be an effective strategy to optimize resource use.

Introducción: La pandemia de COVID-19 conlleva una alta ocupación de los servicios de urgencias (SU). Se requieren nuevas estrategias para optimizar la gestión de estos recursos. Objetivos: Describir los resultados de un sistema de triaje en urgencias para pacientes no graves con sospecha de COVID-19 y posible neumonía, basado en la radiografía de tórax (RT). Material y métodos: Estudio retrospectivo, unicéntrico realizado en Barcelona (España) entre marzo y abril de 2020. Se realizó una RT al ingreso en SU de pacientes con síntomas de COVID-19 y sospecha de neumonía, sin insuficiencia respiratoria, con una prioridad clase IV-V (sistema andorrano de triaje). Esta medida pretende optimizar los recursos y facilitar las altas. Se reportan los resultados tras adoptar esta estrategia. Resultados: Se incluyeron 834 pacientes (53% mujeres, 66% caucásicos, 27% hispánicos). La RT mostró neumonía en 523 (62,7%). Comparados con los pacientes sin neumonía, los que sí la padecían eran mayores (55 vs. 46,6 años), con un índice de comorbilidad de Charlson más elevado (1,9 vs. 1,3) y con mayor riesgo de ingreso y/o muerte (91 vs. 12%). La mortalidad fue numéricamente mayor en el grupo con neumonía (10 vs. 1). El 10% de los pacientes sin neumonía en RT consultaron de nuevo al SU (40% con neumonía). Conclusión: La RT identificó neumonía en múltiples pacientes. Los que no tenían neumonía fueron mayoritariamente dados de alta. La mortalidad entre pacientes con RT negativa fue baja. La RT como triaje para neumonía en pacientes con COVID-19 no grave puede ahorrar recursos.

Radiography-based triage for COVID-19 in the Emergency Department in a Spanish cohort of patients.

BACKGROUND: Strategies to determine who could be safely discharged home from the Emergency Department (ED) in COVID-19 are needed to decongestion healthcare systems. OBJECTIVES: To describe the outcomes of an ED triage system for non-severe patients with suspected COVID-19 and possible pneumonia based on chest X-ray (CXR) upon admission. MATERIAL AND METHODS: Retrospective, single-center study performed in Barcelona (Spain) during the COVID-19 peak in March-April 2020. Patients with COVID-19 symptoms and potential pneumonia, without respiratory insufficiency, with priority class IV-V (Andorran triage model) had a CXR upon admission. This approach tried to optimize resource use and to facilitate discharges. The results after adopting this organizational approach are reported. RESULTS: We included 834 patients, 53% were female. Most patients were white (66%) or Hispanic (27%). CXR showed pneumonia in 523 (62.7%). Compared to those without pneumonia, patients with pneumonia were older (55 vs 46.6 years old) and had a higher Charlson comorbidity index (1.9 vs 1.3). Patients with pneumonia were at a higher risk for a combined outcome of admission and/or death (91 vs 12%). Death rates tended to be numerically higher in the pneumonia group (10 vs 1). Among patients without pneumonia in the initial CXR, 10% reconsulted (40% of them with new pneumonia). CONCLUSION: CXR identified pneumonia in a significant number of patients. Those without pneumonia were mostly discharged. Mortality among patients with an initially negative CXR was low. CXR triage for pneumonia in non-severe COVID-19 patients in the ED can be an effective strategy to optimize resource use.

Response to Treatment in IgG4-Related Disease Assessed by Quantitative PET/CT Scan.

OBJECTIVE: The aim of this study was to assess disease activity by different PET/CT measurements in IgG4-related disease (IgG4-RD) flares and their correlation with the IgG4-RD responder index (IgG4-RI). PATIENTS AND METHODS: Patients were retrospectively recruited from a single center in Barcelona, Spain. They all had IgG4-RD flares with an 18F-FDG PET/CT examination performed within the 2 first weeks of the flare onset and another one after at least 3 months of treatment between 2012 and 2018. Epidemiologic, clinical, laboratory, and therapeutic data were collected at baseline and at follow-up. Semiquantitative and volumetric measurements from PET/CT explorations were recorded. In addition, a 5-point visual scale was (adapted Deauville score) trialed. The IgG4-RI was used as the criterion standard to assess response before and after treatment. RESULTS: Eighteen patients with a total of 23 flares were included. The median time to second PET/CT examination was 7 months. Remission (complete and partial) according to IgG4-RI was observed in 20 flares (87%). All PET/CT measurements (SUVmax and SUVmean, total lesion glycolysis, MTV, and adapted Deauville score) were statistically significantly lower on the follow-up evaluation, except for the size of the lesion. The correlation of all these parameters with the IgG4-RI was positive except for SUVmean and the size of the lesion. CONCLUSIONS: Semiquantitative, volumetric, and visual parameters in PET/CT scans correlated with response to treatment assessed by IgG4-RI. Volumetric and visual items are less subject to variations and could be used to improve activity scores and treatment strategies.

Update in ethiopathogeny, diagnosis and treatment of the IgG4 related disease. / Etiopatogenia, diagnóstico y tratamiento de la enfermedad relacionada con la IgG4.

IgG4 related disease (IgG4-RD) is probably an autoimmune pathology of unknown etiology. Diverse interactions participate in its pathogen between the adaptive and innate immune systems, activating lymphocytes B and T which trigger the inflammatory cascade, which culminates in fibrosis of the organs and their malfunction. It can affect a multitude of organs simultaneously. The diagnosis is based on the correlation of clinical findings with anatomopathological results (lymphoplasmocitary infiltrate, storiform fibrosis, obliterative phlebitis and IgG4+plasmatic cell count) and with the presence of elevated IgG4 in serum, depending on the criteria used. Corticoids and rituximab are among the few validated treatments available. There are multiple biomarkers and treatments in development. In this review, we aim to go over the principal pathogenic and clinical characteristics of IgG4-RD, as well as its handling, in accordance with the available scientific evidence.

IgG4-related disease: Evidence from six recent cohorts.

IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosing disorder. In this review we aim to describe and compare the characteristics of the six largest IgG4-RD cohorts, since the new 2012 consensus diagnostic criteria were released. These observational studies were published between 2012 and 2015. Patients were included using the comprehensive diagnostic criteria or the 2012 consensus criteria. Results were reviewed and summarized. Most patients were middle aged men. Fibro-inflammatory masses developed in virtually all organs except the brain, with an unexplained preference for salivary glands, lymph nodes and pancreas. Corticosteroids were the treatment of choice but up to 40% of patients relapsed within the first year. Standardized response assessment tools, biomarkers and the validation of new treatments are still in development. In conclusion, the features of IgG4-RD are similar across the globe. At the moment, corticosteroids are the only validated treatment but rituximab seems to be promising.