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3. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products

Author

Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., Mosca M., Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., and Mosca M.

Abstract

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP

Published
2023

4. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products

Author

Talarico, R.R. Ramirez, G.A. Barreira, S.C. Cardamone, C. Triggianese, P. Aguilera, S. Andersen, J. Avcin, T. Benistan, K. Bertsias, G. Bortoluzzi, A. Bouillot, C. Bulina, I. Burmester, G.R. Callens, S. Carreira, P.E. Cervera, R. Cutolo, M. Damian, L. Torre, E.D. Faria, R. Fonseca, J.E. Galetti, I. Hachulla, E. Iaccarino, L. Jacobsen, S. Khmelinskii, N. Limper, M. Marinello, D. Meyer, A. Moroncini, G. Nagy, G. Olesinska, M. Pamfil, C. Pileckyte, M. Pistello, M. Rednic, S. Richez, C. Romão, V.C. Schneider, M. Sciascia, S. Scirè, C.A. Simonini, G. Smith, V. Sulli, A. Tani, C. Tas, S.W. Tincani, A. Vonk, M.C. Tektonidou, M. Mosca, M. and Talarico, R.R. Ramirez, G.A. Barreira, S.C. Cardamone, C. Triggianese, P. Aguilera, S. Andersen, J. Avcin, T. Benistan, K. Bertsias, G. Bortoluzzi, A. Bouillot, C. Bulina, I. Burmester, G.R. Callens, S. Carreira, P.E. Cervera, R. Cutolo, M. Damian, L. Torre, E.D. Faria, R. Fonseca, J.E. Galetti, I. Hachulla, E. Iaccarino, L. Jacobsen, S. Khmelinskii, N. Limper, M. Marinello, D. Meyer, A. Moroncini, G. Nagy, G. Olesinska, M. Pamfil, C. Pileckyte, M. Pistello, M. Rednic, S. Richez, C. Romão, V.C. Schneider, M. Sciascia, S. Scirè, C.A. Simonini, G. Smith, V. Sulli, A. Tani, C. Tas, S.W. Tincani, A. Vonk, M.C. Tektonidou, M. Mosca, M.

Abstract

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the fina

Published
2023

5. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products.

Author

Talarico, R., Ramirez, G.A., Barreira, S.C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G.R., Callens, S., Carreira, P.E., Cervera, R., Cutolo, M., Damian, L., Della-Torre, E., Faria, R., Fonseca, J.E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A, Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V.C., Schneider, M., Sciascia, S., Scirè, C.A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S.W., Tincani, A., Vonk, M.C., Tektonidou, M., Mosca, M., Talarico, R., Ramirez, G.A., Barreira, S.C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G.R., Callens, S., Carreira, P.E., Cervera, R., Cutolo, M., Damian, L., Della-Torre, E., Faria, R., Fonseca, J.E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A, Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V.C., Schneider, M., Sciascia, S., Scirè, C.A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S.W., Tincani, A., Vonk, M.C., Tektonidou, M., and Mosca, M.

Abstract

Item does not contain fulltext, Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP

Published
2023

6. Hyperuricemia remodels the serum proteome toward a higher inflammatory state.

Author

Cabău, G., Gaal, O., Badii, O.M., Nica, V., Mirea, A.M., Hotea, I., Pamfil, C., Popp, R.A., Netea, M.G., Rednic, S., Crisan, T.O., Joosten, L.A.B., Cabău, G., Gaal, O., Badii, O.M., Nica, V., Mirea, A.M., Hotea, I., Pamfil, C., Popp, R.A., Netea, M.G., Rednic, S., Crisan, T.O., and Joosten, L.A.B.

Abstract

Item does not contain fulltext, Gout is an autoinflammatory disease triggered by a complex innate immune response to MSU crystals and inflammatory triggers. While hyperuricemia is an obligatory risk factor for the development of gout, the majority of individuals with hyperuricemia never develop gout but have an increased risk of developing cardiometabolic disorders. Current management of gout aims at MSU crystal dissolution by lowering serum urate. We apply a targeted proteomic analysis, using Olink inflammation panel, to a large group of individuals with gout, asymptomatic hyperuricemia, and normouricemic controls, and we show a urate-driven inflammatory signature. We add in vivo evidence of persistent immune activation linked to urate exposure and describe immune pathways involved in the pathogenesis of gout. Our results support a pro-inflammatory effect of asymptomatic hyperuricemia and pave the way for new research into targetable mechanisms in gout and cardiometabolic complications of asymptomatic hyperuricemia.

Published
2023

7. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products

Author

Talarico, Rosaria R., Ramirez, G. A., Barreira, S. C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Callens, S., Carreira, P. E., Cervera, R., Cutolo, M., Damian, L., Torre, E. Della, Faria, R., Fonseca, J. E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A., Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V. C., Schneider, M., Sciascia, S., Scirè, C. A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S. W., Tincani, A., Vonk, M. C., Tektonidou, M., Mosca, M., Talarico, Rosaria R., Ramirez, G. A., Barreira, S. C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Callens, S., Carreira, P. E., Cervera, R., Cutolo, M., Damian, L., Torre, E. Della, Faria, R., Fonseca, J. E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A., Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V. C., Schneider, M., Sciascia, S., Scirè, C. A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S. W., Tincani, A., Vonk, M. C., Tektonidou, M., and Mosca, M.

Abstract

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient’s representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP, Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final

Published
2023

8. AB1309 CLINICAL CHARACTERISTICS OF NEW-ONSET IDIOPATHIC INFLAMMATORY MYOPATHIES FOLLOWING SARS-CoV-2 INFECTION OR VACCINATION – ANALYSIS OF A CASE SERIES

Author

Marian, A., primary, Tamas, M. M., additional, Muntean, L., additional, Resteu, O. M., additional, Vaida-Voevod, D. A., additional, Felea, I., additional, Damian, L., additional, Filipescu, I., additional, Pamfil, C., additional, Szabo, I., additional, Simon, S. P., additional, and Rednic, S., additional

Published
2023
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13. EULAR recommendations for womenʼs health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

Author

Andreoli, L, Bertsias, G K, Agmon-Levin, N, Brown, S, Cervera, R, Costedoat-Chalumeau, N, Doria, A, Fischer-Betz, R, Forger, F, Moraes-Fontes, M F, Khamashta, M, King, J, Lojacono, A, Marchiori, F, Meroni, P L, Mosca, M, Motta, M, Ostensen, M, Pamfil, C, Raio, L, Schneider, M, Svenungsson, E, Tektonidou, M, Yavuz, S, Boumpas, D, and Tincani, A

Published
2017
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14. Rare clinical manifestations in systemic lupus erythematosus: A review on frequency and clinical presentation

Author

Tani, C. Elefante, E. Arnaud, L. Barreira, S.C. Bulina, I. Cavagna, L. Costedoat-Chalumeau, N. Doria, A. Fonseca, J.E. Franceschini, F. Fredi, M. Iaccarino, L. Limper, M. Majnik, J. Nagy, G. Pamfil, C. Rednic, S. Reynolds, J.A. Tektonidou, M.G. Troldborg, A. Zanframundo, G. Mosca, M. and Tani, C. Elefante, E. Arnaud, L. Barreira, S.C. Bulina, I. Cavagna, L. Costedoat-Chalumeau, N. Doria, A. Fonseca, J.E. Franceschini, F. Fredi, M. Iaccarino, L. Limper, M. Majnik, J. Nagy, G. Pamfil, C. Rednic, S. Reynolds, J.A. Tektonidou, M.G. Troldborg, A. Zanframundo, G. Mosca, M.

Abstract

Objectives. The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE). Methods. A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a couple of authors to perform a literature search and article review. Results. In total, 149 articles were included in the literature review: 37 for gastrointestinal manifestations, 6 for liver manifestations, 27 for pulmonary manifestations, 50 for cardiac manifestations, 16 for ocular manifestations, 13 for neurological manifestations. Gastrointestinal disorders included several clinical presentations with variable frequency (from 0.5% to 10.7% of the cases); liver involvement included lupusrelated hepatitis (9.3%) and autoimmune hepatitis (2.3%). The rarest pulmonary manifestations identified were shrinking lung syndrome, described in 1.5% of patients, while interstitial lung disease and lupus pneumonia were reported in 4% and 3% of patients, respectively. Myocarditis and pulmonary hypertension were also rarely described in SLE patients although ranging from 0.4-16% and 1-14% respectively, depending on the methodology used for its identification. Ocular manifestations in SLE included some rare manifestations (reported in less than 5% of patients) and lupus retinopathy that is described in 1.2-28.8% of patients depending on methods of ascertainment. Aseptic meningitis and chorea were also confirmed as very rare manifestations being reported in less than 1% and in 0.3-2.4% of cases respectively. Conclusion. The results of this literature review provide the basis for a better understanding of some less-known manifestations of SLE and for stressing the need for a higher awareness in diagnostic and therapeutic protocols regarding these rare disease aspects. © Copyright Clinical and Experimental Rheumatology 2022.

Published
2022

19. AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

Author

Rusu, I., primary, Muntean, L., additional, Tamas, M. M., additional, Felea, I., additional, Damian, L., additional, Filipescu, I., additional, Simon, S. P., additional, Pamfil, C., additional, Marinescu, C. M., additional, Man, M. A., additional, Csutak, C., additional, and Rednic, S., additional

Published
2020
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21. Sjögren's syndrome: State of the art on clinical practice guidelines

Author

Romao, V, Talarico, R, Scire, C, Vieira, A, Alexander, T, Baldini, C, Gottenberg, J, Gruner, H, Hachulla, E, Mouthon, L, Orlandi, M, Pamfil, C, Pineton De Chambrun, M, Taglietti, M, Toplak, N, Van Daele, P, Van Laar, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Mariette, X, Romao V. C., Talarico R., Scire C. A., Vieira A., Alexander T., Baldini C., Gottenberg J. -E., Gruner H., Hachulla E., Mouthon L., Orlandi M., Pamfil C., Pineton De Chambrun M., Taglietti M., Toplak N., Van Daele P., Van Laar J. M., Bombardieri S., Schneider M., Smith V., Cutolo M., Mosca M., Mariette X., Romao, V, Talarico, R, Scire, C, Vieira, A, Alexander, T, Baldini, C, Gottenberg, J, Gruner, H, Hachulla, E, Mouthon, L, Orlandi, M, Pamfil, C, Pineton De Chambrun, M, Taglietti, M, Toplak, N, Van Daele, P, Van Laar, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Mariette, X, Romao V. C., Talarico R., Scire C. A., Vieira A., Alexander T., Baldini C., Gottenberg J. -E., Gruner H., Hachulla E., Mouthon L., Orlandi M., Pamfil C., Pineton De Chambrun M., Taglietti M., Toplak N., Van Daele P., Van Laar J. M., Bombardieri S., Schneider M., Smith V., Cutolo M., Mosca M., and Mariette X.

Abstract

Sjögren's syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. Up to 30% to 50 % of patients with SS may present systemic disease; moreover, there is an increased risk for the development of non-Hodgkin's lymphoma that occurs in a minority of patients. The present work was developed in the framework of the European Reference Network (ERN) dedicated to Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET). In line with its goals of aiming to improve early diagnosis, treatment and care of rare connective and musculoskeletal diseases, ERN-ReCONNET set to review the current state of clinical practice guidelines (CPGs) in the rare and complex connective tissue diseases of interest of the network. Therefore, the present work was aimed at providing a state of the art of CPGs for SS.

Published
2018

22. Systemic lupus erythematosus: State of the art on clinical practice guidelines

Author

Tamirou, F, Arnaud, L, Talarico, R, Scire, C, Alexander, T, Amoura, Z, Avcin, T, Bortoluzzi, A, Cervera, R, Conti, F, Cornet, A, Devilliers, H, Doria, A, Frassi, M, Fredi, M, Govoni, M, Houssiau, F, Llado, A, Macieira, C, Martin, T, Massaro, L, Moraes-Fontes, M, Pamfil, C, Paolino, S, Tani, C, Tas, S, Tektonidou, M, Tincani, A, Van Vollenhoven, R, Bombardieri, S, Burmester, G, Eurico, F, Galetti, I, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Costedoat-Chalumeau, N, Tamirou F., Arnaud L., Talarico R., Scire C. A., Alexander T., Amoura Z., Avcin T., Bortoluzzi A., Cervera R., Conti F., Cornet A., Devilliers H., Doria A., Frassi M., Fredi M., Govoni M., Houssiau F., Llado A., Macieira C., Martin T., Massaro L., Moraes-Fontes M. F., Pamfil C., Paolino S., Tani C., Tas S. W., Tektonidou M., Tincani A., Van Vollenhoven R. F., Bombardieri S., Burmester G., Eurico F. J., Galetti I., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., Costedoat-Chalumeau N., Tamirou, F, Arnaud, L, Talarico, R, Scire, C, Alexander, T, Amoura, Z, Avcin, T, Bortoluzzi, A, Cervera, R, Conti, F, Cornet, A, Devilliers, H, Doria, A, Frassi, M, Fredi, M, Govoni, M, Houssiau, F, Llado, A, Macieira, C, Martin, T, Massaro, L, Moraes-Fontes, M, Pamfil, C, Paolino, S, Tani, C, Tas, S, Tektonidou, M, Tincani, A, Van Vollenhoven, R, Bombardieri, S, Burmester, G, Eurico, F, Galetti, I, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Costedoat-Chalumeau, N, Tamirou F., Arnaud L., Talarico R., Scire C. A., Alexander T., Amoura Z., Avcin T., Bortoluzzi A., Cervera R., Conti F., Cornet A., Devilliers H., Doria A., Frassi M., Fredi M., Govoni M., Houssiau F., Llado A., Macieira C., Martin T., Massaro L., Moraes-Fontes M. F., Pamfil C., Paolino S., Tani C., Tas S. W., Tektonidou M., Tincani A., Van Vollenhoven R. F., Bombardieri S., Burmester G., Eurico F. J., Galetti I., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., and Costedoat-Chalumeau N.

Abstract

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-Three original guidelines published between 2004 and 2017 were identified. Many aspects of disease management are covered, including global disease management, lupus nephritis and neuropsychiatric involvement, management of pregnancies, vaccinations and comorbidities monitoring. Unmet needs relate with disease management of some clinical manifestations and adherence to treatment. Many patient's unmet needs have been identified starting with faster diagnosis, need for more therapeutic options, guidelines on lifestyle issues, attention to quality of life and adequate education.

Published
2018

23. Combined genetic and transcriptome analysis of patients with SLE: distinct, targetable signatures for susceptibility and severity

Author

Panousis, N.I. Bertsias, G.K. Ongen, H. Gergianaki, I. Tektonidou, M.G. Trachana, M. Romano-Palumbo, L. Bielser, D. Howald, C. Pamfil, C. Fanouriakis, A. Kosmara, D. Repa, A. Sidiropoulos, P. Dermitzakis, E.T. Boumpas, D.T.

Abstract

Objectives Systemic lupus erythematosus (SLE) diagnosis and treatment remain empirical and the molecular basis for its heterogeneity elusive. We explored the genomic basis for disease susceptibility and severity. Methods mRNA sequencing and genotyping in blood from 142 patients with SLE and 58 healthy volunteers. Abundances of cell types were assessed by CIBERSORT and cell-specific effects by interaction terms in linear models. Differentially expressed genes (DEGs) were used to train classifiers (linear discriminant analysis) of SLE versus healthy individuals in 80% of the dataset and were validated in the remaining 20% running 1000 iterations. Transcriptome/genotypes were integrated by expression-quantitative trail loci (eQTL) analysis; tissue-specific genetic causality was assessed by regulatory trait concordance (RTC). Results SLE has a 'susceptibility signature' present in patients in clinical remission, an 'activity signature' linked to genes that regulate immune cell metabolism, protein synthesis and proliferation, and a 'severity signature' best illustrated in active nephritis, enriched in druggable granulocyte and plasmablast/plasma-cell pathways. Patients with SLE have also perturbed mRNA splicing enriched in immune system and interferon signalling genes. A novel transcriptome index distinguished active versus inactive disease - but not low disease activity - and correlated with disease severity. DEGs discriminate SLE versus healthy individuals with median sensitivity 86% and specificity 92% suggesting a potential use in diagnostics. Combined eQTL analysis from the Genotype Tissue Expression (GTEx) project and SLE-associated genetic polymorphisms demonstrates that susceptibility variants may regulate gene expression in the blood but also in other tissues. Conclusion Specific gene networks confer susceptibility to SLE, activity and severity, and may facilitate personalised care. © Author(s) (or their employer(s)) 2019.

Published
2019

26. Sjögren's syndrome: State of the art on clinical practice guidelines

Author

Romão, V.C. (Vasco C.), Talarico, R. (Rosaria), Scirè, C.A. (Carlo Alberto), Vieira, A. (Ana), Alexander, T. (Tobias), Baldini, C. (Chiara), Gottenberg, J.-E. (Jacques-Eric), Gruner, H. (Heidi), Hachulla, E. (Eric), Mouthon, L. (Luc), Orlandi, M. (Martina), Pamfil, C. (Cristina), Pineton De Chambrun, M. (Marc), Taglietti, M. (Marco), Toplak, N. (Natasa), Daele, P.L.A. (Paul) van, Van Laar, J.M. (Jacob M), Bombardieri, S. (Stefano), Schneider, M. (Matthias), Smith, V. (Vanessa), Cutolo, M. (Maurizio), Mosca, M. (Marta), Mariette, X. (Xavier), Romão, V.C. (Vasco C.), Talarico, R. (Rosaria), Scirè, C.A. (Carlo Alberto), Vieira, A. (Ana), Alexander, T. (Tobias), Baldini, C. (Chiara), Gottenberg, J.-E. (Jacques-Eric), Gruner, H. (Heidi), Hachulla, E. (Eric), Mouthon, L. (Luc), Orlandi, M. (Martina), Pamfil, C. (Cristina), Pineton De Chambrun, M. (Marc), Taglietti, M. (Marco), Toplak, N. (Natasa), Daele, P.L.A. (Paul) van, Van Laar, J.M. (Jacob M), Bombardieri, S. (Stefano), Schneider, M. (Matthias), Smith, V. (Vanessa), Cutolo, M. (Maurizio), Mosca, M. (Marta), and Mariette, X. (Xavier)

Abstract

Sjögren's syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. Up to 30% to 50 % of patients with SS may present systemic disease; moreover, there is an increased risk for the development of non-Hodgkin's lymphoma that occurs in a minority of patients. The present work was developed in the framework of the European Reference Network (ERN) dedicated to Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET). In line with its goals of aiming to improve early diagnosis, treatment and care of rare connective and musculoskeletal diseases, ERN-ReCONNET set to review the current state of clinical practice guidelines (CPGs) in the rare and complex connective tissue diseases of interest of the network. Therefore, the present work was aimed at providing a state of the art of CPGs for SS.

Published
2018
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27. Sjogren's syndrome: state of the art on clinical practice guidelines

Author

Romao, VC, Talarico, R, Scire, CA, Vieira, A, Alexander, T, Baldini, C, Gottenberg, JE, Gruner, H, Hachulla, E, Mouthon, L, Orlandi, M, Pamfil, C, de Chambrun, MP, Taglietti, M, Toplak, N, van Daele, Paul, van Laar, JM, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Mariette, X, Romao, VC, Talarico, R, Scire, CA, Vieira, A, Alexander, T, Baldini, C, Gottenberg, JE, Gruner, H, Hachulla, E, Mouthon, L, Orlandi, M, Pamfil, C, de Chambrun, MP, Taglietti, M, Toplak, N, van Daele, Paul, van Laar, JM, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, and Mariette, X

Published
2018

28. EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

Author

Andreoli, L. Bertsias, G.K. Agmon-Levin, N. Brown, S. Cervera, R. Costedoat-Chalumeau, N. Doria, A. Fischer-Betz, R. Forger, F. Moraes-Fontes, M.F. Khamashta, M. King, J. Lojacono, A. Marchiori, F. Meroni, P.L. Mosca, M. Motta, M. Ostensen, M. Pamfil, C. Raio, L. Schneider, M. Svenungsson, E. Tektonidou, M. Yavuz, S. Boumpas, D. Tincani, A.

Abstract

Objectives: Develop recommendations for women's health issues and family planning in systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). Methods: Systematic review of evidence followed by modified Delphi method to compile questions, elicit expert opinions and reach consensus. Results: Family planning should be discussed as early as possible after diagnosis. Most women can have successful pregnancies and measures can be taken to reduce the risks of adverse maternal or fetal outcomes. Risk stratification includes disease activity, autoantibody profile, previous vascular and pregnancy morbidity, hypertension and the use of drugs (emphasis on benefits from hydroxychloroquine and antiplatelets/anticoagulants). Hormonal contraception and menopause replacement therapy can be used in patients with stable/inactive disease and low risk of thrombosis. Fertility preservation with gonadotropin-releasing hormone analogues should be considered prior to the use of alkylating agents. Assisted reproduction techniques can be safely used in patients with stable/inactive disease; patients with positive antiphospholipid antibodies/APS should receive anticoagulation and/or low-dose aspirin. Assessment of disease activity, renal function and serological markers is important for diagnosing disease flares and monitoring for obstetrical adverse outcomes. Fetal monitoring includes Doppler ultrasonography and fetal biometry, particularly in the third trimester, to screen for placental insufficiency and small for gestational age fetuses. Screening for gynaecological malignancies is similar to the general population, with increased vigilance for cervical premalignant lesions if exposed to immunosuppressive drugs. Human papillomavirus immunisation can be used in women with stable/inactive disease. Conclusions: Recommendations for women's health issues in SLE and/or APS were developed using an evidence-based approach followed by expert consensus. © Published by the BMJ Publishing Group Limited.

Published
2017

30. Cyclophosphamide in combination with glucocorticoids for severe neuropsychiatric systemic lupus erythematosus: A retrospective, observational two-centre study

Author

Fanouriakis, A. Pamfil, C. Sidiropoulos, P. Damian, L. Flestea, A. Gusetu, G. Rednic, S. Bertsias, G. Boumpas, D.T.

Abstract

Cyclophosphamide (CYC) is used in severe neuropsychiatric systemic lupus erythematosus (NPSLE), but long-term data regarding its efficacy and safety are lacking. We identified NPSLE cases who received CYC from two centres during the period 1999-2013 and had regular follow-up. General and neuropsychiatric outcome at last follow-up visit were determined, and major complications were documented. CYC was administered in 50 neuropsychiatric events. Median age was 45.0 years and 46% of patients were positive for antiphospholipid antibodies. Most frequent indications were psychosis (11 cases), polyneuropathy (six cases), and cerebrovascular disease, seizure disorder and cranial neuropathy (five cases). CYC was mainly administered as monthly pulses (median number: 8.0 (range 3-26), median cumulative dose: 7.2 g (range 2.4-33.8)). Cases were followed for a median of 46.5 months (range 5-408). At last follow-up, partial or complete response of NPSLE was observed in 84% of events; 10% had stable disease, whereas the remaining 6% failed to improve or worsened and were rescued with rituximab. In events that responded to CYC, maintenance therapy consisted of azathioprine in 31 events (65.9%), bimonthly or quarterly pulses of intravenous CYC in nine (19.1%), and mycophenolate mofetil in five (10.6%). Relapses were observed in six events (12%) at median eight months after initial response. No malignancies were observed, yet there were three cases of severe infections. Amenorrhea was recorded in three patients, who had not received gonadal protection. In conclusion, cyclophosphamide was efficacious and led to sustained response of severe NPSLE in a cohort with long follow-up. © SAGE Publications.

Published
2016

36. EULAR recommendations for neuropsychiatric systemic lupus erythematosus vs usual care: Results from two European centres

Author

Pamfil, C. Fanouriakis, A. Damian, L. Rinzis, M. Sidiropoulos, P. Tsivgoulis, G. Rednic, S. Bertsias, G. Boumpas, D.T.

Abstract

Objective. To compare the European League Against Rheumatism (EULAR) recommendations for the management of NPSLE with usual care in two tertiary centres and to detect potential pitfalls in their use for diagnosis and treatment. Methods. A chart-based review of NPSLE manifestations was conducted in two European centres. Diagnostic and treatment decisions were compared against the EULAR recommendations for general NPSLE and specific manifestations. Results. We studied a total of 94 patients who experienced 123 lupus-related neuropsychiatric events over 10 years. In 80% of the events, at least one EULAR-defined risk factor (previous NPSLE, generalized disease activity or aPL positivity) was present. Overall, there was good concordance between clinical care and recommendations for diagnosis and treatment (68.7% and 62.7% of events, respectively). Brain MRI was performed in the absence of a clear EULAR recommendation in 42.9% of events; therein, it was more frequently normal compared with imaging performed according to the recommendations (52.4% vs 18.5%, P = 0.008), and it did not influence management. Among patients reporting cognitive dysfunction, only 27.8% underwent the recommended neuropsychological assessment. In line with the recommendations, immunosuppressants were more frequently given in events suggestive of an inflammatory process (80.5% vs 47.6% in non-inflammatory events, P < 0.001). Notably, 52% of cerebrovascular events were managed with combined immunosuppressive/antithrombotic therapy due to either coexisting generalized lupus activity or recurrence despite prior antithrombotic treatment. Conclusion. Despite good concordance between EULAR recommendations for NPSLE and usual clinical practice, we identified a number of issues (such as overutilization of brain MRI, suboptimal evaluation of cognitive dysfunction, and frequent use of immunosuppressives in cerebrovascular disease) that need to be investigated further. © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.

Published
2015

37. EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

Author

Andreoli, L, primary, Bertsias, G K, additional, Agmon-Levin, N, additional, Brown, S, additional, Cervera, R, additional, Costedoat-Chalumeau, N, additional, Doria, A, additional, Fischer-Betz, R, additional, Forger, F, additional, Moraes-Fontes, M F, additional, Khamashta, M, additional, King, J, additional, Lojacono, A, additional, Marchiori, F, additional, Meroni, P L, additional, Mosca, M, additional, Motta, M, additional, Ostensen, M, additional, Pamfil, C, additional, Raio, L, additional, Schneider, M, additional, Svenungsson, E, additional, Tektonidou, M, additional, Yavuz, S, additional, Boumpas, D, additional, and Tincani, A, additional

Published
2016
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38. Diagnostic criteria for systemic lupus erythematosus: Has the time come?

Author

Bertsias, G.K. Pamfil, C. Fanouriakis, A. Boumpas, D.T.

Subjects
immune system diseases, skin and connective tissue diseases
Abstract

Systemic lupus erythematosus (SLE) is a multiorgan disease with protean manifestations. Because SLE is uncommon and heterogeneous, its diagnosis can pose a considerable challenge, especially for clinicians with limited expertise of the disease. This is particularly true at the early stages of SLE, when an inadequate number of features to secure the diagnosis might be present, and for patients presenting with uncommon features, which can nonetheless be severe and require prompt treatment. Furthermore, the suboptimal performance of immunological testing in patients referred for possible SLE has been highlighted. As a result, SLE remains largely a clinical diagnosis that is made after excluding alternative diagnoses. Diagnostic criteria can expedite diagnosis and treatment, but are not available for SLE. Thus, SLE classification criteria are often used, but strict adherence to these criteria could delay diagnosis. Therefore, while eagerly awaiting diagnostic criteria for this disease, we propose interim potential solutions to facilitate its diagnosis. © 2013 Macmillan Publishers Limited. All rights reserved.

Published
2013

40. OP0086 Eular Recommendations for Women's Health and the Management of Family Planning, Assisted Reproduction, Pregnancy, and Menopause in Patients With Systemic Lupus Erythematosus and/or the Antiphospholipid Syndrome

Author

Andreoli, L., primary, Bertsias, G., additional, Agmon-Levin, N., additional, Brown, S., additional, Cervera, R., additional, Costedoat-Chalumeau, N., additional, Doria, A., additional, Fischer-Betz, R., additional, Forger, F., additional, Moraes-Fontes, M., additional, Khamashta, M., additional, King, J., additional, Lojacono, A., additional, Marchiori, F., additional, Meroni, P., additional, Mosca, M., additional, Motta, M., additional, Ostensen, M., additional, Pamfil, C., additional, Raio, L., additional, Schneider, M., additional, Svenungsson, E., additional, Tektonidou, M., additional, Yavuz, S., additional, Boumpas, D., additional, and Tincani, A., additional

Published
2015
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47. Cyclophosphamide in combination with glucocorticoids for severe neuropsychiatric systemic lupus erythematosus: a retrospective, observational two-centre study.

Author

Fanouriakis, A., Pamfil, C., Sidiropoulos, P., Damian, L., Flestea, A., Gusetu, G., Rednic, S., Bertsias, G., and Boumpas, D. T.

Subjects
*SYSTEMIC lupus erythematosus treatment, *CYCLOPHOSPHAMIDE, *PHOSPHOLIPID antibodies, *CRANIAL nerve diseases, *COMBINATION drug therapy, THERAPEUTIC use of glucocorticoids
Abstract

Cyclophosphamide (CYC) is used in severe neuropsychiatric systemic lupus erythematosus (NPSLE), but long-term data regarding its efficacy and safety are lacking. We identified NPSLE cases who received CYC from two centres during the period 1999–2013 and had regular follow-up. General and neuropsychiatric outcome at last follow-up visit were determined, and major complications were documented. CYC was administered in 50 neuropsychiatric events. Median age was 45.0 years and 46% of patients were positive for antiphospholipid antibodies. Most frequent indications were psychosis (11 cases), polyneuropathy (six cases), and cerebrovascular disease, seizure disorder and cranial neuropathy (five cases). CYC was mainly administered as monthly pulses (median number: 8.0 (range 3–26), median cumulative dose: 7.2 g (range 2.4–33.8)). Cases were followed for a median of 46.5 months (range 5–408). At last follow-up, partial or complete response of NPSLE was observed in 84% of events; 10% had stable disease, whereas the remaining 6% failed to improve or worsened and were rescued with rituximab. In events that responded to CYC, maintenance therapy consisted of azathioprine in 31 events (65.9%), bimonthly or quarterly pulses of intravenous CYC in nine (19.1%), and mycophenolate mofetil in five (10.6%). Relapses were observed in six events (12%) at median eight months after initial response. No malignancies were observed, yet there were three cases of severe infections. Amenorrhea was recorded in three patients, who had not received gonadal protection. In conclusion, cyclophosphamide was efficacious and led to sustained response of severe NPSLE in a cohort with long follow-up. [ABSTRACT FROM AUTHOR]

Published
2016
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48. Thematic stream: co-morbidity (PP59-PP86): PP59. A Single Dose of Indomethacin does not Prolong Premotor Reaction Time in Young Healthy Adults: A Randomised, Placebo Controlled, Double-Blind, Cross-Over Study

Author

Ozyemisci-Taskiran, O., primary, Gunendi, Z., additional, Beyazova, M., additional, Erden, Z., additional, Zinnuroglu, M., additional, Gogus, F., additional, Cevik, F. C., additional, Yazici, S., additional, Cil, H., additional, Cevik, R., additional, Sarac, A. J., additional, Nas, K., additional, Park, W., additional, Lim, M. J., additional, Kwon, S. R., additional, Yoon, J. Y., additional, Ju, G. E., additional, Son, M. J., additional, Coskun, O., additional, Paker, N., additional, Bugdayci, D., additional, Santos-Moreno, P., additional, Cubides, M. F., additional, Guzman, C., additional, Reyes, E., additional, Londono, J., additional, Valle-Onate, R., additional, Zateri, C., additional, Kilinc, S., additional, Birtane, M., additional, Tastekin, N., additional, Sabirli, F., additional, Turan, Y., additional, Kocaaga, Z., additional, Karakoyun-Celik, O., additional, Gurgan, A., additional, Duransoy, A., additional, Filipescu, I., additional, Pamfil, C., additional, Dumitru, B., additional, Garboan, I., additional, Rednic, S., additional, Altunalan, A., additional, Sahin, M., additional, Dere, D., additional, Cidem, M., additional, Capkin, E., additional, Karkucak, M., additional, Karaca, A., additional, Akyuz, A., additional, Alver, A., additional, Turkyilmaz, A. K., additional, Zengin, E., additional, Ulusoy, H., additional, Kesikburun, S., additional, Aydemir, K., additional, Ozgul, A., additional, Sezer, N., additional, Koseoglu, F., additional, Sutbeyaz, S. T., additional, Oken, O., additional, Ozlemis, B., additional, Kibar, S., additional, Yurdakul, S., additional, Findikoglu, S. Y., additional, Hatemi, G., additional, Suzen, S., additional, Yazici, H., additional, Haroon, M., additional, Adeeb, F., additional, Devlin, J., additional, Gradaigh, D. O., additional, Walker, F., additional, Odabasi, B. B., additional, Sendur, O. F., additional, Moghimi, N., additional, Nadrian, H., additional, Moradzadeh, R., additional, Nadrian, E., additional, Rahimi, E., additional, Ediz, L., additional, Hiz, O., additional, Fethi Ceylan, M., additional, Toprak, M., additional, Tekeoglu, I., additional, Hamuryudan, V., additional, Tascilar, K., additional, Ugurlu, S., additional, Figen, A., additional, Pinar, B., additional, Figen, T., additional, Ataman, S., additional, Emlakcioglu, E., additional, Kaymak, B., additional, Ozcakar, L., additional, Tan, A. A., additional, de Brouwer, S., additional, Kraaimaat, F., additional, Sweep, F., additional, Donders, R., additional, Eijsbouts, A., additional, Radstake, T., additional, van Riel, P., additional, Evers, A., additional, Cengiz, K., additional, Ilhanli, I., additional, Durmus, D., additional, Alayli, G., additional, Karakoc, M., additional, Batmaz, I., additional, Tahtasiz, M., additional, Tekbas, E., additional, and Yildiz, I., additional

Published
2011
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49. Cardiovascular profiles of scleroderma patients with arrhythmias and conduction disorders.

Author

Muresan, L., Petcu, A., Pamfil, C., Muresan, C., Rinzis, M., Mada, R. O., Gusetu, G. N., Pop, D., Zdrenghea, D., and Rednic, S.

Abstract

Introduction: Arrhythmias and conduction disorders are common among patients with scleroderma. Their early identification is important, since scleroderma patients with arrhythmias have a higher mortality risk compared with scleroderma patients without arrhythmias. The aim of this study was to characterize the cardiovascular profiles of scleroderma patients with different types of arrhythmias and conduction disorders. Methods: One hundred and ten consecutive patients with a diagnosis of systemic sclerosis according to the ACR criteria were included in the study. Patients underwent a 12-lead ECG and a 24-hour Holter ECG monitoring for arrhythmias and conduction disorders identification. Blood sample testing, echocardiography, spirometry, chest X-ray and, when considered appropriate, high resolution chest CT were also performed. A subgroup of 21 patients underwent NT-pro BNP le vel measurements. Patients' clinical and para-clinical cha - racteristics were compared according to the pre sence or absence of arrhythmias and conduction di sorders. Results: The prevalence of arrhythmia and conduction disturbances was 60.9%. Patients with such disorders were older (54.4 ± 13.3 vs. 49.7 ± 10.1 years, p=0.05), had a higher prevalence of pulmonary hypertension (p=0.008), valve disease (p < 0.001), especially mitral and tricuspid regurgitation, chamber enlargement on echocardiography (left atrial and right ventricular, p = 0.012 and 0.005, respectively), as well as, higher NT-pro BNP levels: 265.5 ± 399.7 vs. 163 ± 264.3 pg/ml, p=0.04. Conclusion: Arrhythmias and conduction disorders are common in patients with scleroderma. Patients with such disorders are older, have a higher prevalence of pulmonary hypertension, more severe mitral and tricuspid regurgitation, left atrial and right ventricular dilation on echocardiography. [ABSTRACT FROM AUTHOR]

Published
2016

50. EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome

Author

King, J, Fischer-Betz, R, Motta, M, Meroni, P L, Pamfil, C, Marchiori, F, Khamashta, M, Boumpas, D, Moraes-Fontes, M F, Cervera, R, Schneider, M, Raio, Luigi, Andreoli, L, Ostensen, M, Doria, A, Tektonidou, M, Tincani, A, Lojacono, A, Agmon-Levin, N, Mosca, M, Costedoat-Chalumeau, N, Bertsias, G K, Brown, S, Forger, F, Svenungsson, E, and Yavuz, S

Subjects
610 Medicine & health, 3. Good health
Abstract

OBJECTIVES Develop recommendations for women's health issues and family planning in systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). METHODS Systematic review of evidence followed by modified Delphi method to compile questions, elicit expert opinions and reach consensus. RESULTS Family planning should be discussed as early as possible after diagnosis. Most women can have successful pregnancies and measures can be taken to reduce the risks of adverse maternal or fetal outcomes. Risk stratification includes disease activity, autoantibody profile, previous vascular and pregnancy morbidity, hypertension and the use of drugs (emphasis on benefits from hydroxychloroquine and antiplatelets/anticoagulants). Hormonal contraception and menopause replacement therapy can be used in patients with stable/inactive disease and low risk of thrombosis. Fertility preservation with gonadotropin-releasing hormone analogues should be considered prior to the use of alkylating agents. Assisted reproduction techniques can be safely used in patients with stable/inactive disease; patients with positive antiphospholipid antibodies/APS should receive anticoagulation and/or low-dose aspirin. Assessment of disease activity, renal function and serological markers is important for diagnosing disease flares and monitoring for obstetrical adverse outcomes. Fetal monitoring includes Doppler ultrasonography and fetal biometry, particularly in the third trimester, to screen for placental insufficiency and small for gestational age fetuses. Screening for gynaecological malignancies is similar to the general population, with increased vigilance for cervical premalignant lesions if exposed to immunosuppressive drugs. Human papillomavirus immunisation can be used in women with stable/inactive disease. CONCLUSIONS Recommendations for women's health issues in SLE and/or APS were developed using an evidence-based approach followed by expert consensus.

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