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Definition of Low Disease Activity State based on the SLE-DAS: Derivation and v...

Assunção, Helena; Jesus, Diogo; Larosa, Maddalena; Henriques, Carla; Matos, Ana; Le Guern, Véronique; Rubiño, Francisco; Silva, José A P da

Objectives To derive and validate a definition of low disease activity (LDA) for SLE based on the SLE Disease Activity Score (SLE-DAS), in a real-life multicentre cohort of SLE patients. Methods Derivation was conducted using data from a monocentric cohort of SLE (Portugal), and validation was performed in a multicentre cohort (Italy, France and Spain). The Lupus Low Disease Activity State (LLDAS) was used as c...


Rare clinical manifestations in systemic lupus erythematosus: a review on frequ...

Tani, Chiara; Elefante, Elena; Arnaud, Laurent; Barreira, Sofia; Bulina, Inita; Cavagna, Lorenzo; Costedoat-Chalumeau, Nathalie; Doria, Andrea

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 pair of authors to perform a literature search and article review. Resul...


The impact of COVID-19 on rare and complex connective tissue diseases : the exp...

Talarico, Rosaria; Aguilera, Silvia; Alexander, Tobias; Amoura, Zahir; Antunes, Ana M.; Arnaud, Laurent; Avcin, Tadej; Beretta, Lorenzo

During 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 Ra...


Derivation and validation of the SLE Disease Activity Score (SLE-DAS): a new SL...

Jesus, Diogo; Cristina Bico Rodrigues de Matos, Ana; Henriques, C.; Zen, Margherita; Larosa, Maddalena; Iaccarino, Luca; Da Silva, José António Pereira

Objectives To derive and validate a new disease activity measure for systemic lupus erythematosus (SLE), the SLE Disease Activity Score (SLE-DAS), with improved sensitivity to change as compared with SLE Disease Activity Index (SLEDAI), while maintaining high specificity and easiness of use. Methods We studied 520 patients with SLE from two tertiary care centres (derivation and validation cohorts). At each visi...


Mixed connective tissue disease : state of the art on clinical practice guidelines

Chaigne, Benjamin; Scirè, Carlo Alberto; Talarico, Rosaria; Alexander, Tobias; Amoura, Zahir; Avcin, Tadej; Beretta, Lorenzo; Doria, Andrea

Mixed 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 ...


Undifferentiated connective tissue disease : state of the art on clinical pract...

Antunes, Margarida; Scirè, Carlo Alberto; Talarico, Rosaria; Alexander, Tobias; Avcin, Tadej; Belocchi, Chiara; Doria, Andrea; Franceschini, Franco

The 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 p...


Idiopathic inflammatory myopathies : state of the art on clinical practice guid...

Meyer, Alain; Scirè, Carlo Alberto; Talarico, Rosaria; Alexander, Tobias; Amoura, Zahir; Avcin, Tadej; Barsotti, Simone; Beretta, Lorenzo

Idiopathic 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)...


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