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1. European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations

Author

Lohr, J. -M., Beuers, U., Vujasinovic, M., Alvaro, D., Frokjaer, J. B., Buttgereit, F., Capurso, G., Culver, E. L., De-Madaria, E., Della-Torre, E., Detlefsen, S., Dominguez-Mu~noz, E., Czubkowski, P., Ewald, N., Frulloni, L., Gubergrits, N., Duman, D. G., Hackert, T., Iglesias-Garcia, J., Kartalis, N., Laghi, A., Lammert, F., Lindgren, F., Okhlobystin, A., Oracz, G., Parniczky, A., Mucelli, R. M. P., Rebours, V., Rosendahl, J., Schleinitz, N., Schneider, A., van Bommel, E. F. H., Verbeke, C. S., Vullierme, M. P., Witt, H., Besselink, M. G., Bruno, M. J., Czako, L., Chiaro, M., Filippova, O., Fukuda, A., Gaujoux, S., Hart, P. A., Hegyi, P., Jonas, E., Kahraman, A., Kleger, A., Kuryata, O., Laukkarinen, J., Lerch, M. M., Marchegiani, G., Marschall, H. -U., Matos, C., Molad, Y., Oguz, D., Pukitis, A., Satoi, S., Stone, J. H., Verheij, J., Vries, N., Lohr, J-Matthias, Beuers, Ulrich, Vujasinovic, Miroslav, Alvaro, Domenico, Frokjaer, Jens Brondum, Buttgereit, Frank, Capurso, Gabriele, Culver, Emma L., De-Madaria, Enrique, Della-Torre, Emanuel, Detlefsen, Sonke, Dominguez-Munoz, Enrique, Czubkowski, Piotr, Ewald, Nils, Frulloni, Luca, Gubergrits, Natalya, Duman, Deniz Guney, Hackert, Thilo, Iglesias-Garcia, Julio, Kartalis, Nikolaos, Laghi, Andrea, Lammert, Frank, Lindgren, Fredrik, Okhlobystin, Alexey, Oracz, Grzegorz, Parniczky, Andrea, Mucelli, Raffaella Maria Pozzi, Rebours, Vinciane, Rosendahl, Jonas, Schleinitz, Nicolas, Schneider, Alexander, van Bommel, Eric F. H., Verbeke, Caroline Sophie, Vullierme, Marie Pierre, Witt, Heiko, Besselink, Marc G., Bruno, Marco J., Czako, Laszlo, del Chiaro, Marco, Filippova, Oleksandra, Fukuda, Akihisa, Gaujoux, Sebastien, Hart, Phil A., Hegyi, Peter, Jonas, Eduard, Kahraman, Alisan, Kleger, Alexander, Kuryata, Olexander, Laukkarinen, Johanna, Lerch, Markus M., Marchegiani, Giovanni, Marschal, Hanns-Ulrich, Matos, Celso, Molad, Yair, Oguz, Dilek, Pukitis, Aldis, Satoi, Sohei, Stone, John H., Verheij, Joanne, de Vries, Niek, KKÜ, Gastroenterology and Hepatology, Tytgat Institute for Liver and Intestinal Research, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, Löhr, Jm, Beuers, U, Vujasinovic, M, Alvaro, D, Frøkjær, Jb, Buttgereit, F, Capurso, G, Culver, El, de-Madaria, E, DELLA TORRE, E, Detlefsen, S, Dominguez-Muñoz, E, Czubkowski, P, Ewald, N, Frulloni, L, Gubergrits, N, Duman, Dg, Hackert, T, Iglesias-Garcia, J, Kartalis, N, Laghi, A, Lammert, F, Lindgren, F, Okhlobystin, A, Oracz, G, Parniczky, A, Mucelli, Rmp, Rebours, V, Rosendahl, J, Schleinitz, N, Schneider, A, van Bommel, Ef, Verbeke, C, Vullierme, Mp, Witt, H, and UEG guideline working, Group.

Subjects
Abdominal pain, IMMUNOGLOBULIN G4-RELATED DISEASE, SERUM IGG4 LEVELS, Medizin, Disease, RC799-869, Severity of Illness Index, immune-related cholangitis, Serology, 0302 clinical medicine, LONG-TERM OUTCOMES, Prednisone, Drug Dosage Calculations, Child, other organ involvement, STEROID-THERAPY, INTERNATIONAL-CONSENSUS, Evidence-Based Medicine, glucocorticoids, Gastroenterology, Induction Chemotherapy, IgG4-related, Diseases of the digestive system. Gastroenterology, PRIMARY SCLEROSING CHOLANGITIS, TYPE-1 AUTOIMMUNE PANCREATITIS, CONSENSUS DIAGNOSTIC-CRITERIA, Europe, Treatment Outcome, Oncology, 030220 oncology & carcinogenesis, diabetes mellitus, 030211 gastroenterology & hepatology, medicine.symptom, digestive, autoimmune pancreatitis type 1, Glucocorticoid, Immunosuppressive Agents, medicine.drug, Adult, medicine.medical_specialty, Digestive System Diseases, biomarkers, cancer, disease, Maintenance Chemotherapy, 03 medical and health sciences, Internal medicine, Diabetes mellitus, medicine, Humans, FINE-NEEDLE-ASPIRATION, Dose-Response Relationship, Drug, business.industry, EMISSION TOMOGRAPHY/COMPUTED TOMOGRAPHY, Body Weight, Editorials, Cancer, Guideline, medicine.disease, business
Abstract

Frulloni, Luca/0000-0001-7417-2655; Hart, Phil/0000-0003-4346-6196; Capurso, Gabriele/0000-0002-0019-8753; de-Madaria, Enrique/0000-0002-2412-9541; Lohr, Matthias/0000-0002-7647-198X; Frokjaer, Jens Brondum/0000-0001-8722-0070 WOS:000542363500001 PubMed: 32552502 The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra-pancreatic digestive organs, including IgG4-related cholangitis. Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6-0.8 mg/kg body weight/day orally (typical starting dose 30-40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2-4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi-organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added. National Societies Committee of the United European Gastroenterology (UEG) We gratefully acknowledge the support from the National Societies Committee of the United European Gastroenterology (UEG) for the conduct of these guidelines independent from other sources. No other funding was received.

Published
2020

2. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phil A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Kubota, Kensuke, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Sekiguchi, Hiroshi, Schleinitz, Nicolas, Stone, James R, Takahashi, Naoki, Umehara, Hisanori, Webster, George, Zen, Yoh, Stone, John H, Akamizu, T, Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chari, S, Chiba, T, Choi, H, Cornell, L, Culver, E, Darabian, S, Torre, Ed, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, L, Hart, Pa, Hernandez-Molina, G, Inoue, D, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kawano, M, Khosroshahi, A, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Lanzillotta, M, Leng, H, Lerch, M, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Naden, Rp, Nakamura, S, Nordeide, J, Notohara, K, Okazaki, K, Paira, S, Perugino, Ca, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Saeki, T, Sato, Y, Schleinitz, N, Sekiguchi, H, Sharma, A, Sokol, Ev, Stone, Jr, Stone, Jh, Sun, W, Takahashi, H, Takahashi, N, Takahira, M, Tanaka, Y, Umehara, H, Vaglio, A, Villamil, A, Wada, Y, Wallace, Zs, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zen, Y, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, H, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Kubota, K, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Sekiguchi, H, Schleinitz, N, Stone, Jr, Takahashi, N, Umehara, H, Webster, G, Zen, Y, Stone, Jh, and American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working, Group.

Subjects
Adult, Male, medicine.medical_specialty, Consensus, Aged, Diagnosis, Differential, Europe, Female, Humans, Immunoglobulin G4-Related Disease, Middle Aged, Reproducibility of Results, Rheumatology, Societies, Medical, United States, Immunology, MEDLINE, Disease, Internal medicine, Medical, parasitic diseases, Diagnosis, Immunology and Allergy, Medicine, business.industry, medicine.disease, Confidence interval, Test (assessment), Differential, IgG4-related disease, business, Societies, Rheumatism, Decision analysis
Abstract

Objective IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises, existing literature, derivation and validation cohorts of 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2-99.8%) and a sensitivity of 85.5% (95% CI 81.9-88.5%). In the second, the specificity was 97.8% (95% CI 93.7-99.2%) and the sensitivity was 82.0% (95% CI 77.0-86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiologic, and basic science investigations.

Published
2018

3. Demographic and clinical data in acquired hemophilia A

Author

Knoebl, P, Marco, P, Baudo, F, Collins, P, Huth Kühne, A, Nemes, L, Pellegrini, F, Tengborn, L, Lévesque, H, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, D'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, POGLIANI, ENRICO MARIA, Carpenedo M, Remiddi C, Santagostino E, Santoro R, Papaleo G, Schinco P, Borchiellini A, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Sartori R, Tagariello G, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolini B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Pascual M, Mingot ME, Perez Garrido R, Perez Gonzale Nz, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, Wilde J., Knoebl, P, Marco, P, Baudo, F, Collins, P, Huth Kühne, A, Nemes, L, Pellegrini, F, Tengborn, L, Lévesque, H, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, DI MINNO, Giovanni, Cerbone, Am, DI MINNO, matteo nicola dario, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Sartori, R, Tagariello, G, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Pascual, M, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., Aspoeck, G, Borg, JY, Huth-Kühne, A, Loreth, RM, Di Minno, G, Cerbone, AM, Di Minno, D, D'incà, M, Mazzucconi, MG, Scortechini, AR, Tagliaferri, AR, Rivolta, GF, Laros-van Gorkom, B, Leebeek, FW, van der Linden, PW, Gutiérrez Pimentel, MJ Jiménez-Yuste, V, Jose-Felix, L, Pascual, M, Mingot, ME, Rodriguez-Huerta, AM, Wilde, J, Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), Huth Kühne, A, Lévesque, H, Borg, J, Loreth, R, Cerbone, A, Mazzucconi, M, Scortechini, A, Tagliaferri, A, Rivolta, G, Brandolini, B, Leebeek, F, van der Linden, P, Gutiérrez Pimentel, M, Mingot, M, Perez Gonzale, N, Rodriguez Huerta, A, and Other departments

Subjects
Registrie, Male, Pediatrics, diagnosis, medicine.medical_treatment, Hemostatic Technique, Kaplan-Meier Estimate, registry, THERAPY, Settore MED/15 - Malattie Del Sangue, Immunosuppressive Agent, IMMUNOADSORPTION, Risk Factors, Pregnancy, 80 and over, Prospective Studies, Registries, Prospective cohort study, health care economics and organizations, Aged, 80 and over, treatment, Immunosuppression, Hematology, Middle Aged, FACTOR-VIII INHIBITOR, Acquired hemophilia, Demographics, Diagnosis, Outcome, Registry, Treatment, Aged, Autoantibodies, Chi-Square Distribution, Europe, Factor VIII, Female, Hemostatic Techniques, Humans, Immunosuppressive Agents, Risk Assessment, Treatment Outcome, Hemophilia A, Hemorrhage, Autoantibodie, acquired hemophilia, demographics, outcome, INTRAVENOUS GAMMA-GLOBULIN, Human, medicine.medical_specialty, Malignancy, hemophilia, registry, medicine, METAANALYSIS, Autoimmune disease, business.industry, Risk Factor, Autoantibody, medicine.disease, Surgery, Prospective Studie, Hemostasis, business, Chi-squared distribution
Abstract

Summary. Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: Five hundred and one (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003 and 2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. Fifty-seven per cent of the non-pregnancy-related cases were male. Four hundred and seventy-four bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. Four hundred and seventy-seven patients underwent immunosuppression, and 72.6% achieved complete remission. Conclusions: Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA.

Published
2012

4. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)

Author

Collins, Peter, Baudo, Francesco, Knoebl, Paul, Lévesque, Hervé, Nemes, László, Pellegrini, Fabio, Marco, Pascual, Tengborn, Lilian, Huth Kühne, Angela, Group Author: Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lévesque H, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Huth Kühne A, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Nemes L, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Baudo F, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Cerbone AM, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, Pogliani E, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Brandolin B, Hamulyák K, Kamphuisen P, Laros van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez Yuste V, Jose Felix L, Marco P, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Tengborn L, Boehlen F, Korte W, Chowdary P, Collins P, Evans G, Pavord S, Rangarajan S, Wilde J., DI MINNO, GIOVANNI, DI MINNO, MATTEO, Interne Geneeskunde, Foundations and methods of Law, RS: CARIM School for Cardiovascular Diseases, Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), Collins, Peter, Baudo, Francesco, Knoebl, Paul, Lévesque, Hervé, Nemes, László, Pellegrini, Fabio, Marco, Pascual, Tengborn, Lilian, Huth Kühne, Angela, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lévesque, H, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Huth Kühne, A, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Nemes, L, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Baudo, F, Caimi, T, Contino, L, D'Angelo Armando, Cl, Fattorini, A, DI MINNO, Giovanni, Cerbone, Am, DI MINNO, Matteo, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, Me, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Marco, P, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Tengborn, L, Boehlen, F, Korte, W, Chowdary, P, Collins, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., and Other departments

Subjects
Registrie, Male, Clinical Trials and Observations, medicine.medical_treatment, THERAPY, Biochemistry, Gastroenterology, Immunosuppressive Agent, Antibodies, Monoclonal, Murine-Derived, Adult, Aged, Aged, 80 and over, Autoantibodies, Cyclophosphamide, Cyclosporine, Europe, Factor VIII, Female, Follow-Up Studies, Hemophilia A, Humans, Immunosuppressive Agents, Middle Aged, Registries, Rituximab, Secondary Prevention, Steroids, Treatment Outcome, FACTOR-VIII INHIBITORS, Monoclonal, 80 and over, UNITED-KINGDOM, Hematology, RECLASSIFICATION, Immunosuppression, Autoantibodie, Human, medicine.drug, Murine-Derived, medicine.medical_specialty, Immunology, DIAGNOSIS, Antibodies, Follow-Up Studie, Internal medicine, MANAGEMENT, medicine, Steroid, Autoimmune disease, business.industry, Settore MED/09 - MEDICINA INTERNA, Autoantibody, Cell Biology, Odds ratio, CENTER DOCTORS ORGANIZATION, medicine.disease, Surgery, DISCRIMINATION, Etiology, business
Abstract

Acquired hemophilia A (AHA) is an autoimmune disease caused by an autoantibody to factor VIII. Patients are at risk of severe and fatal hemorrhage until the inhibitor is eradicated, and guidelines recommend immunosuppression as soon as the diagnosis has been made. The optimal immunosuppressive regimen is unclear; therefore, data from 331 patients entered into the prospective EACH2 registry were analyzed. Steroids combined with cyclophosphamide resulted in more stable complete remission (70%), defined as inhibitor undetectable, factor VIII more than 70 IU/dL and immunosuppression stopped, than steroids alone (48%) or rituximab-based regimens (59%). Propensity score-matched analysis controlling for age, sex, factor VIII level, inhibitor titer, and underlying etiology confirmed that stable remission was more likely with steroids and cyclophosphamide than steroids alone (odds ratio = 3.25; 95% CI, 1.51-6.96; P < .003). The median time to complete remission was approximately 5 weeks for steroids with or without cyclophosphamide; rituximab-based regimens required approximately twice as long. Immunoglobulin administration did not improve outcome. Second-line therapy was successful in approximately 60% of cases that failed first-line therapy. Outcome was not affected by the choice of first-line therapy. The likelihood of achieving stable remission was not affected by underlying etiology but was influenced by the presenting inhibitor titer and FVIII level.

Published
2012

5. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

Author

Baudo, Francesco, Collins, Peter, Huth Kühne, Angela, Lévesque, Hervé, Marco, Pascual, Nemes, László, Pellegrini, Fabio, Tengborn, Lilian, Knoebl, Paul, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lévesque, H, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Huth Kühne, A, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Nemes, L, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Baudo, F, Caimi, T, Contino, L, D'Angelo Armando, Cl, Fattorini, A, Cerbone, Am, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, Me, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Marco, P, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Tengborn, L, Boehlen, F, Korte, W, Chowdary, P, Collins, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., DI MINNO, GIOVANNI, DI MINNO, MATTEO, Other departments, Baudo, Francesco, Collins, Peter, Huth Kühne, Angela, Lévesque, Hervé, Marco, Pascual, Nemes, László, Pellegrini, Fabio, Tengborn, Lilian, Knoebl, Paul, Group Author: Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, Jy, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lévesque, H, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Huth Kühne, A, Loreth, Rm, Steigerwald, U, Tiede, A, Theodossiades, G, Nemes, L, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Baudo, F, Caimi, T, Contino, L, D'Angelo Armando, Cl, Fattorini, A, DI MINNO, Giovanni, Cerbone, Am, DI MINNO, Matteo, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, Mg, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, Me, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, Ar, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, Ar, Di Perna, C, Rivolta, Gf, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Brandolin, B, Hamulyák, K, Kamphuisen, P, Laros van Gorkom, B, Leebeek, Fw, Marten, N, Novakova, I, Schutgens, R, van der Linden, Pw, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, Mj, Jiménez Yuste, V, Jose Felix, L, Marco, P, Mingot, Me, Perez Garrido, R, Perez Gonzale, Nz, Prieto Garcia, M, Rodriguez Huerta, Am, Sedano, C, Tolosa Munoz, A, Baghaei, F, Tengborn, L, Boehlen, F, Korte, W, Chowdary, P, Collins, P, Evans, G, Pavord, S, Rangarajan, S, Wilde, J., Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), Huth Kühne, A, Lévesque, H, Pellegrini, F, Knoebl, P, Aspoeck, G, Borg, J, Loreth, R, D'Angelo Armando, C, Di Minno, G, Cerbone, A, Di Minno, D, Mazzucconi, M, Mancuso, M, Scortechini, A, Tagliaferri, A, Rivolta, G, Leebeek, F, van der Linden, P, Gutiérrez Pimentel, M, Mingot, M, Perez Gonzale, N, Rodriguez Huerta, A, and Wilde, J

Subjects
Registrie, Male, SURGERY, Biochemistry, THERAPY, Hemostatics, Hemostatic, FACTOR-VIII INHIBITORS, 80 and over, Deamino Arginine Vasopressin, Registries, Desmopressin, UNITED-KINGDOM, Factor IX, Aged, 80 and over, Hematology, biology, Incidence, FEIBA, Recombinant Protein, Middle Aged, Blood Coagulation Factors, Recombinant Proteins, Europe, Treatment Outcome, Coagulation, Female, medicine.drug, Blood Coagulation Factor, Human, Adult, medicine.medical_specialty, Adolescent, Immunology, Aged, Factor VIII, Factor VIIa, Hemophilia A, Hemorrhage, Humans, Young Adult, DIAGNOSIS, Internal medicine, BYPASSING ACTIVITY, medicine, business.industry, Settore MED/09 - MEDICINA INTERNA, RECOMBINANT FACTOR VIIA, Retrospective cohort study, Cell Biology, FACTOR-IX, CENTER DOCTORS ORGANIZATION, Surgery, Recombinant factor VIIa, Propensity score matching, biology.protein, business
Abstract

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).

Published
2012

6. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry

Author

Tengborn L, Baudo F, Huth-Kühne A, Knoebl P, Lévesque H, Marco P, Pellegrini F, Nemes L, Collins P, EACH2 registry contributors, Aspoeck G, Heistinger M, Knöbl P, Makipernaa A, André H, Aouba A, Bellucci S, Beurrier P, Borg JY, Darnige L, Devignes J, d'Oiron R, Gautier P, Gay V, Girault S, Gruel Y, Guerin V, Hézard N, Khellaf M, Koenig M, Lifermann F, Marlu R, Ninet J, Peynet J, Quéméneur T, Rothschild C, Schleinitz N, Sigaud M, Trouillier S, Voisin S, Giebl A, Holstein K, Loreth RM, Steigerwald U, Tiede A, Theodossiades G, Radvanyi G, Schlammadinger A, Barillari G, Pasca S, Caimi T, Contino L, D'Angelo Armando CL, Fattorini A, Di Minno G, Cerbone AM, Di Minno D, D'incà M, Falanga A, Maggioni A, Lerede T, Franchini M, Gaidano G, De Paoli L, Gamba G, Ghirardi R, Girotto M, Tasca D, Grandone E, Tiscia G, Imberti D, Iorio A, Landolfi R, Di Gennaro L, Novarese L, Mariani G, Lapecorella M, Marietta M, Pedrazzi P, Mazzucconi MG, Santoro C, Morfini M, Linari S, Moratelli S, Paolini R, Piseddu G, Poggio R, Pogliani E, Carpenedo M, Remiddi C, Santagostino E, Mancuso ME, Santoro R, Papaleo G, Schinco P, Borchiellini A, Valeri F, Scortechini AR, Siragusa S, Sottilotta G, Squizzato A, Tagariello G, Sartori R, Tagliaferri AR, Di Perna C, Rivolta GF, Testa S, Paoletti O, Toschi V, Zanon E, Hamulyák K, Kamphuisen P, Laros-van Gorkom B, Leebeek FW, Marten N, Novakova I, Schutgens R, van der Linden PW, van Esser J, van der Meer J, Ypma P, Campos M, Aguilar C, Altisent C, Bermejo N, Del Campo R, Ferreiro Argüelles M, González Boullosa R, Gutiérrez Pimentel MJ, Jiménez-Yuste V, Jose-Felix L, Mingot ME, Perez Garrido R, Perez Gonzale NZ, Prieto Garcia M, Rodriguez-Huerta AM, Sedano C, Tolosa Munoz A, Baghaei F, Boehlen F, Korte W, Chowdary P, Evans G, Pavord S, Rangarajan S, Wilde J, Tengborn, L, Baudo, F, Huth-Kühne, A, Knoebl, P, Lévesque, H, Marco, P, Pellegrini, F, Nemes, L, Collins, P, EACH2 registry, C, Aspoeck, G, Heistinger, M, Knöbl, P, Makipernaa, A, André, H, Aouba, A, Bellucci, S, Beurrier, P, Borg, J, Darnige, L, Devignes, J, D'Oiron, R, Gautier, P, Gay, V, Girault, S, Gruel, Y, Guerin, V, Hézard, N, Khellaf, M, Koenig, M, Lifermann, F, Marlu, R, Ninet, J, Peynet, J, Quéméneur, T, Rothschild, C, Schleinitz, N, Sigaud, M, Trouillier, S, Voisin, S, Giebl, A, Holstein, K, Loreth, R, Steigerwald, U, Tiede, A, Theodossiades, G, Radvanyi, G, Schlammadinger, A, Barillari, G, Pasca, S, Caimi, T, Contino, L, D'Angelo Armando, C, Fattorini, A, Di Minno, G, Cerbone, A, Di Minno, D, D'Incà, M, Falanga, A, Maggioni, A, Lerede, T, Franchini, M, Gaidano, G, De Paoli, L, Gamba, G, Ghirardi, R, Girotto, M, Tasca, D, Grandone, E, Tiscia, G, Imberti, D, Iorio, A, Landolfi, R, Di Gennaro, L, Novarese, L, Mariani, G, Lapecorella, M, Marietta, M, Pedrazzi, P, Mazzucconi, M, Santoro, C, Morfini, M, Linari, S, Moratelli, S, Paolini, R, Piseddu, G, Poggio, R, Pogliani, E, Carpenedo, M, Remiddi, C, Santagostino, E, Mancuso, M, Santoro, R, Papaleo, G, Schinco, P, Borchiellini, A, Valeri, F, Scortechini, A, Siragusa, S, Sottilotta, G, Squizzato, A, Tagariello, G, Sartori, R, Tagliaferri, A, Di Perna, C, Rivolta, G, Testa, S, Paoletti, O, Toschi, V, Zanon, E, Hamulyák, K, Kamphuisen, P, Laros-van Gorkom, B, Leebeek, F, Marten, N, Novakova, I, Schutgens, R, van der Linden, P, van Esser, J, van der Meer, J, Ypma, P, Campos, M, Aguilar, C, Altisent, C, Bermejo, N, Del Campo, R, Ferreiro Argüelles, M, González Boullosa, R, Gutiérrez Pimentel, M, Jiménez-Yuste, V, Jose-Felix, L, Mingot, M, Perez Garrido, R, Perez Gonzale, N, Prieto Garcia, M, Rodriguez-Huerta, A, Sedano, C, Tolosa Munoz, A, Baghaei, F, Boehlen, F, Korte, W, Chowdary, P, Evans, G, Pavord, S, Rangarajan, S, and Wilde, J

Subjects
Adult, Acquired haemophilia A, diagnosis, pregnancy, treatment, Antifibrinolytic Agents, Blood Coagulation Factors, Drug Therapy, Combination, Europe, Factor VIIa, Female, Follow-Up Studies, Hemostatics, Humans, Immunosuppressive Agents, Kaplan-Meier Estimate, Pregnancy, Prospective Studies, Recombinant Proteins, Registries, Treatment Outcome, Hemophilia A, Pregnancy Complications, Hematologic, Drug Therapy, Hematologic, Acquired Haemophilia, Pregnancy, Pregnancy Complications, Combination
Abstract

Objective The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII. Design Prospective, multi-centre, large-scale, pan-European registry. Setting A total of 117 haemophilia centres in 13 European countries. Population Pregnancy-associated AHA. Methods Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay. Main outcome measures Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome. Results The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up. Conclusions Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.

Published
2012

7. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease

Author

Khosroshahi, A, Wallace, Z. S, Crowe, J. L, Akamizu, T, Azumi, A, Carruthers, M. N, Chari, S. T, Della Torre, E, Frulloni, Luca, Goto, H, Hart, P. A, Kamisawa, T, Kawa, S, Kawano, M, Kim, M. H, Kodama, Y, Kubota, K, Lerch, M. M, Löhr, M, Masaki, Y, Matsui, S, Mimori, T, Nakamura, S, Nakazawa, T, Ohara, H, Okazaki, K, Ryu, J. H, Saeki, T, Schleinitz, N, Shimatsu, A, Shimosegawa, T, Takahashi, H, Takahira, M, Tanaka, A, Topazian, M, Umehara, H, Webster, G. J, Witzig, T. E, Yamamoto, M, Zhang, W, Chiba, T, and Stone, J. H.

Subjects
Biological Products, Treatment Outcome, Immunoglobulin G, Humans, Autoimmune Diseases, Glucocorticoids, Immunosuppressive Agents
Published
2014

8. [Thymic disease associated with nephrotic syndrome: a new case with membranous nephropathy and literature review]

Author

Seguier J, Guillaume-Jugnot P, Ebbo M, Daniel L, Jourde-Chiche N, stephane burtey, Bernit E, Thomas P, Jr, Harlé, and Schleinitz N

Subjects
Nephrotic Syndrome, Thymoma, Humans, Female, Thymus Neoplasms, Kidney, Thymectomy, Glomerulonephritis, Membranous, Aged
Abstract

Malignant thymoma or thymic hyperplasia is associated with various autoimmune diseases. Renal disease has rarely been reported in this condition. We report a new case with improvement of renal disease after thymectomy.A 77-year-old-women with nephritic syndrome was found to have associated thymic mass. Renal pathology showed membranous nephropathy. The thymic mass pathology showed a B2 type thymoma. After thymectomy the nephrotic syndrome improved.Glomerulopathy can be secondary to an acquired thymic disease. Membranous nephropathy but also other glomerular diseases can be observed often presenting with nephritic syndrome. Despite the rarity of this association this clinical observation underlines that a thymoma should be searched in the presence of a glomerulopathy. The glomerulopathy can be improved by the treatment of the thymoma.

Published
2014

9. [Genetic control of hypereosinophilias]

Author

Granel B, Disdier P, Schleinitz N, CHRISTOPHE CHEVILLARD, Jr, Harle, Dessein A, and Pj, Weiller

Subjects
Phenotype, Hypereosinophilic Syndrome, Helminthiasis, Hypersensitivity, Chromosomes, Human, Pair 5, Granulocyte-Macrophage Colony-Stimulating Factor, Humans, Genetic Predisposition to Disease, Interleukin-3, Genetic Testing, Interleukin-4, Interleukin-5
Abstract

Polynuclear eosinophils play a major role in host defense against infectious diseases and especially helminthiasis. Onset of hypereosinophilia can be attributed to two mechanisms which can occur separately or in combination. The first mechanism involves enhancement of medullary production and differentiation of eosinophils. The second is prolonged life span of eosinophils. Response to eosinophils depends on various cytokines including IL-5, IL-4, IL-3 and GM-CSF. Since it contains the genes coding for these cytokines, the 5q31-q33 region of chromosome 5 is the focus of study on genetic control of human hypereosinophilia. The goal of these studies is to allow screening of subjects predisposed to helminthic infection and to deregulation of immune responses that may lead to atopy and various types systemic inflammatory diseases.

10. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon K, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phillip A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Schleinitz, Nicolas, Takahashi, Naoki, Umehara, Hisanori, Zen, Yoh, Stone, Collaborators: Akamizu T, John H., Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chiba, T, Cornell, L, Culver, E, Darabian, S, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, Luca, Hernandez-Molina, G, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Leng, H, Lerch, Mm, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Nakamura, S, Nordeide, J, Notohara, K, Paira, S, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Sato, Y, Sekiguchi, H, Sokol, Ev, Stone, Jr, Sun, W, Takahashi, H, Takahira, M, Tanaka, Y, Vaglio, A, Villamil, A, Wada, Y, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, Hk, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Schleinitz, N, Takahashi, N, Umehara, H, Zen, Y, Stone, Jh, and Members of the ACR/EULAR IgG4-RD Classification Criteria Working, Group.

Subjects
rheumatoid arthritis, Adult, Male, 0301 basic medicine, medicine.medical_specialty, Immunology, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, parasitic diseases, Epidemiology, Humans, Immunology and Allergy, Medicine, Pathological, Sjøgren's syndrome, Aged, 030203 arthritis & rheumatology, business.industry, Middle Aged, inflammation, medicine.disease, Test (assessment), 030104 developmental biology, Rheumatoid arthritis, Radiological weapon, Female, Immunoglobulin G4-Related Disease, business, Rheumatism, Decision analysis
Abstract

IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.

Published
2019

11. An International, Multi-Specialty Validation Study of the IgG4-Related Disease Responder Index

Author

Frank B. Cortazar, Emanuel Della-Torre, Takako Saeki, Arezou Khosroshahi, Marco Lanzillotta, Mollie D. Carruthers, John H. Stone, Hisanori Umehara, Cory A. Perugino, Wen Zhang, Mitsuhiro Kawano, Hyon K. Choi, Paula Tanasa, Nicolas Schleinitz, Omer Karadag, Luca Frulloni, George Webster, Shoko Matsui, Jay H. Ryu, Emma L. Culver, Myung-Hwan Kim, Ana Paula Fernandes, Kazuichi Okazaki, Shigeyuki Kawa, Phil A. Hart, Corrado Campochiaro, Zachary S. Wallace, Mikael Ebbo, Wallace, Z, Khosroshahi, A, Carruthers, Md, Perugino, Ca, Choi, H, Campochiaro, C, Culver, El, Cortazar, F, DELLA TORRE, E, Ebbo, M, Fernandes, A, Frulloni, L, Hart, P, Karadag, O, Kawa, S, Kawano, M, Kim, Mh, Lanzillotta, M, Matsui, S, Okazaki, K, Ryu, Jh, Saeki, T, Schleinitz, N, Tanasa, P, Umehara, H, Webster, G, Zhang, W, and Stone, Jh.

Subjects
0301 basic medicine, medicine.medical_specialty, MEDLINE, Disease, Severity of Illness Index, Article, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, Severity of illness, Humans, Medicine, IgG4-related disease, 030203 arthritis & rheumatology, therapy, business.industry, Organ dysfunction, Discriminant validity, Score, Reproducibility of Results, Construct validity, Intra-rater reliability, Confidence interval, 030104 developmental biology, Immunoglobulin G4-Related Disease, medicine.symptom, business
Abstract

OBJECTIVE:IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ, leading to organ dysfunction and failure. The IgG4-RD Responder Index (RI) was developed to help investigators assess the efficacy of treatment in a structured manner. The aim of this study was to validate the RI in a multinational investigation. METHODS:The RI guides investigators through assessments of disease activity and damage in 25 domains, incorporating higher weights for disease manifestations that require urgent treatment or that worsen despite treatment. After a training exercise, investigators reviewed 12 written IgG4-RD vignettes based on real patients. Investigators calculated both an RI score as well as a physician's global assessment (PhGA) score for each vignette. In a longitudinal assessment, 3 investigators used the RI in 15 patients with newly active disease who were followed up over serial visits after treatment. We assessed interrater and intrarater reliability, precision, validity, and responsiveness. RESULTS:The 26 physician investigators included representatives from 6 specialties and 9 countries. The interrater and intrarater reliability of the RI was strong (0.89 and 0.69, respectively). Correlations (construct validity) between the RI and PhGA were high (Spearman's r = 0.9, P < 0.0001). The RI was sensitive to change (discriminant validity). Following treatment, there was significant improvement in the RI score (mean change 10.5 [95% confidence interval (95% CI) 5.4-12], P < 0.001), which correlated with the change in the PhGA. Urgent disease and damage were captured effectively. DISCUSSION:In this international, multispecialty study, we observed that the RI is a valid and reliable disease activity assessment tool that can be used to measure response to therapy.

Published
2018

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