Browsing by Author "Doria, A"
Now showing 1 - 8 of 8
Results Per Page
Sort Options
- Antiphospholipid Syndrome: State of the Art on Clinical Practice GuidelinesPublication . Limper, M; Scirè, CA; Talarico, R; Amoura, Z; Avcin, T; Basile, M; Burmester, G; Carli, L; Cervera, R; Costedoat-Chalumeau, N; Doria, A; Dörner, T; Eurico Fonseca, J; Galetti, I; Hachulla, E; Launay, D; Lourenco, F; Macieira, C; Meroni, P; Montecucco, CM; Moraes-Fontes, MF; Mouthon, L; Nalli, C; Ramoni, V; Tektonidou, M; van Laar, JM; Bombardieri, S; Schneider, M; Smith, V; Vieira, A; Cutolo, M; Mosca, M; Tincani, AAntiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies. This review aims to identify existing clinical practice guidelines (CPG) as part of the ERN ReCONNET project, aimed at evaluating existing CPGs or recommendations in rare and complex diseases. Seventeen papers providing important data were identified; however, the literature search highlighted the scarceness of reliable clinical data to develop CPGs. With no formal clinical guidelines in place, diagnosis and treatment of APS is largely based on consensus and expert opinion. Patients' unmet need refers to the understanding of the disease and its clinical picture and implications, the need of education for patients, family members and healthcare providers, as well as to the development of monitoring pathways involving multiple healthcare providers.
- Disease Activity Assessment of Rheumatic Diseases During Pregnancy: a Comprehensive Review of Indices Used in Clinical StudiesPublication . Andreoli, L; Gerardi, MC; Fernandes, M; Bortoluzzi, A; Bellando-Randone, S; Brucato, A; Caporali, R; Chighizola, C; Chimenti, MS; Conigliaro, P; Cutolo, M; Cutro, MS; D'Angelo, S; Doria, A; Elefante, E; Fredi, M; Galeazzi, M; Gerosa, M; Govoni, M; Iuliano, A; Larosa, M; Lazzaroni, MG; Matucci-Cerinic, M; Meroni, M; Meroni, P; Mosca, M; Patanè, M; Pazzola, G; Pendolino, M; Perricone, R; Ramoni, V; Salvarani, C; Sebastiani, G; Selmi, C; Spinelli, F; Valesini, G; Scirè, CA; Tincani, APregnancy requires a special management in women with inflammatory rheumatic diseases (RDs), with the aim of controlling maternal disease activity and avoiding fetal complications. Despite the heterogeneous course of RDs during pregnancy, their impact on pregnancy largely relates to the extent of active inflammation at the time of conception. Therefore, accurate evaluation of disease activity is crucial for the best management of pregnant patients. Nevertheless, there are limitations in using conventional measures of disease activity in pregnancy, as some items included in these instruments can be biased by symptoms or by physiological changes related to pregnancy and the pregnancy itself may influence laboratory parameters used to assess disease activity. This article aims to summarize the current literature about the available instruments to measure disease activity during pregnancy in RDs. Systemic lupus erythematosus is the only disease with instruments that have been modified to account for several adaptations which might interfere with the attribution of signs or symptoms to disease activity during pregnancy. No modified-pregnancy indices exist for women affected by other RDs, but standard indices have been applied to pregnant patients. The current body of knowledge shows that the physiologic changes that occur during pregnancy need to be either adapted from existing instruments or developed to improve the management of pregnant women with RDs. Standardized instruments to assess disease activity during pregnancy would be helpful not only for clinical practice but also for research purposes.
- 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 SyndromePublication . Andreoli, L; Bertsias, GK; Agmon-Levin, N; Brown, S; Cervera, R; Costedoat-Chalumeau, N; Doria, A; Fischer-Betz, R; Forger, F; Moraes-Fontes, MF; Khamashta, M; King, J; Lojacono, A; Marchiori, F; Meroni, PL; Mosca, M; Motta, M; Ostensen, M; Pamfil, C; Raio, L; Schneider, M; Svenungsson, E; Tektonidou, M; Yavuz, S; Boumpas, D; Tincani, AOBJECTIVES: 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.
- Idiopathic Inflammatory Myopathies: State of the Art on Clinical Practice Guidelines [corrected]Publication . Meyer, A; Scirè, CA; Talarico, R; Alexander, T; Amoura, Z; Avcin, T; Barsotti, S; Beretta, L; Blagojevic, J; Burmester, G; Cavazzana, I; Cherrin, P; Damian, L; Doria, A; Fonseca, JE; Furini, F; Galetti, I; Houssiau, F; Krieg, T; Maddalena, L; Launay, D; Campanilho-Marques, R; Martin, T; Matucci-Cerinic, M; Moinzadeh, P; Montecucco, C; Moraes-Fontes, MF; Mouthon, L; Neri, R; Paolino, S; Piette, Y; Rednic, S; Tamirou, F; Tincani, A; Toplak, N; Bombardieri, S; Hachulla, E; Mueller-Ladner, U; Schneider, M; Smith, V; Vieira, A; Cutolo, M; Mosca, M; Cavagna, LIdiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
- Mixed connective tissue disease: state of the art on clinical practice guidelinesPublication . Chaigne, B; Scirè, CA; Talarico, R; Alexander, T; Amoura, Z; Avcin, T; Beretta, L; Doria, A; Guffroy, A; Guimarães, V; Hachulla, É; Krieg, T; Launay, D; Lepri, G; Moinzadeh, P; Müller-Ladner, U; Rednic, S; Rodrigues, A; Tas, SW; van Vollenhoven, RF; Vieira, A; Bombardieri, S; Fonseca, JE; Galetti, I; Schneider, M; Smith, V; Cutolo, M; Mosca, M; Fischer-Betz, RMixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients.
- Systemic Lupus Erythematosus: State of the Art on Clinical Practice GuidelinesPublication . Tamirou, F; Arnaud, L; Talarico, R; Scirè, CA; 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; Lladò, A; Macieira, C; Martin, T; Massaro, Laura; Moraes-Fontes, MF; Pamfil, C; Paolino, S; Tani, C; Tas, SW; Tektonidou, M; Tincani, A; Van Vollenhoven, RF; Bombardieri, S; Burmester, G; Eurico, FJ; Galetti, I; Hachulla, E; Mueller-Ladner, U; Schneider, M; Smith, V; Cutolo, M; Mosca, M; Costedoat-Chalumeau, NSystemic 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.
- The Impact of COVID-19 on Rare and Complex Connective Tissue Diseases: the Experience of ERN ReCONNETPublication . Talarico, R; Aguilera, S; Alexander, T; Amoura, Z; Antunes, A; Arnaud, L; Avcin, T; Beretta, L; Bombardieri, S; Burmester, G; Cannizzo, S; Cavagna, L; Chaigne, B; Cornet, A; Costedoat-Chalumeau, N; Doria, A; Ferraris, A; Fischer-Betz, R; Fonseca, J; Frank, C; Gaglioti, A; Galetti, I; Grunert, J; Guimarães, V; Hachulla, E; Houssiau, F; Iaccarino, L; Krieg, T; Limper, M; Malfait, F; Mariette, X; Marinello, D; Martin, T; Matthews, L; Matucci-Cerinic, M; Meyer, A; Montecucco, C; Mouthon, L; Müller-Ladner, U; Rednic, S; Romão, V; Schneider, M; Smith, V; Sulli, A; Tamirou, F; Taruscio, D; Taulaigo, A; Terol, E; Tincani, A; Ticciati, S; Turchetti, G; van Hagen, P; van Laar, J; Vieira, A; de Vries-Bouwstra, J; Cutolo, M; Mosca, MDuring the COVID-19 pandemic, the need to provide high-level care for a large number of patients with COVID-19 has affected resourcing for, and limited the routine care of, all other conditions. The impact of this health emergency is particularly relevant in the rare connective tissue diseases (rCTDs) communities, as discussed in this Perspective article by the multi-stakeholder European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET). The clinical, organizational and health economic challenges faced by health-care providers, institutions, patients and their families during the SARS-CoV-2 outbreak have demonstrated the importance of ensuring continuity of care in the management of rCTDs, including adequate diagnostics and monitoring protocols, and highlighted the need for a structured emergency strategy. The vulnerability of patients with rCTDs needs to be taken into account when planning future health policies, in preparation for not only the post-COVID era, but also any possible new health emergencies.
- Undifferentiated connective tissue disease: state of the art on clinical practice guidelinesPublication . Antunes, M; Scirè, CA; Talarico, R; Alexander, T; Avcin, T; Belocchi, C; Doria, A; Franceschini, F; Galetti, I; Govoni, M; Hachulla, E; Launay, D; Lepri, G; Macieira, C; Matucci-Cerinic, M; Montecucco, CM; Moraes-Fontes, MF; Mouthon, L; Paolino, S; Ramoni, V; Tani, C; Tas, SW; Tincani, A; Van Vollenhoven, R; Zen, M; Fonseca, J; Bombardieri, S; Fonseca, JE; Schneider, M; Smith, V; Cutolo, M; Mosca, M; Beretta, LThe term 'undifferentiated connective tissue disease' (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.