Publications by authors named "Ruiz-Irastorza G"

: Prolonged remission on low-dose glucocorticoids (GC) is a main goal in patients with systemic lupus erythematosus (SLE). The aim of this study is to assess whether GC ≤ 5 mg/d increases the risk of damage accrual in patients with SLE in prolonged remission. : Observational study of routine clinical care data of the inception Lupus Cruces-Bordeaux cohort.

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  • - This study examined the occurrence and effects of antinuclear antibodies (ANA) in patients with antiphospholipid antibodies (aPL) but without other systemic autoimmune diseases, using data from the APS ACTION Registry.
  • - Among the 430 analyzed patients, 56% tested positive for ANA, revealing significant links between ANA positivity and various autoimmune features like hematologic issues and joint involvement.
  • - Despite the presence of these autoimmune characteristics in ANA-positive patients, the study found no connection between ANA status and complications related to thrombosis or pregnancy; interestingly, ANA-negative patients had more pregnancies and live births.
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Introduction: Antimalarials (AMs) are old drugs with a wide range of beneficial effects in systemic lupus erythematosus (SLE) beyond the control of activity. The most recent debate is focused on defining the optimal doses to assure the best benefit/risk ratio.

Areas Covered: We have reviewed the pharmacological basis underlying the various therapeutic effects of AMs and the beneficial and toxic effects of HCQ, also discussing the role of mepacrine not only as a substitute in cases of maculopathy, but also as a very effective therapy combined with HCQ.

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We aimed to determine the prevalence and clinical correlations of mood disorders in a sample of systemic lupus erythematosus (SLE) patients. Hence, we hypothesized that the prevalence of mood disorders would be lower than reported in the literature and that patients would remain clinically stable and show less damage accrual despite low-dose corticosteroid prescription. In total, 92 SLE outpatients gave informed consent to participate in this cross-sectional study.

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  • Glucocorticoids (GCs) are commonly used to treat systemic lupus erythematosus (SLE), but their long-term use can lead to damage; therefore, tailored dosing and tapering strategies are crucial.
  • Recent guidelines suggest starting at lower doses and increasing tapering speed to minimize damage, with an emphasis on keeping maintenance doses below 5 mg/day.
  • Additional treatments, like methylprednisolone pulses and immunosuppressive drugs, can enhance effectiveness and reduce GC reliance, with a focus on finding suitable candidates for potential withdrawal in stable remission phases.
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  • Antiphospholipid syndrome (APS) can lead to serious issues like venous thromboembolism (VTE) and stroke, requiring careful management that includes evaluating antiphospholipid antibodies and other autoimmune conditions.
  • The main treatment for thrombotic APS is anticoagulation with vitamin K antagonists, while low-dose aspirin is recommended for primary prevention in asymptomatic patients and as part of combination therapy for arterial thrombosis.
  • Direct oral anticoagulants (DOACs) may be used in specific low-risk cases but are not advised for arterial thrombosis or those with triple positive antiphospholipid antibodies, with additional treatments like hydroxychloroquine and statins considered for more
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Antiphospholipid syndrome (APS) is the most frequent acquired thrombophilia of autoimmune basis. Pregnancy complications of APS may include recurrent miscarriage, and placental dysfunction presenting as fetal death, prematurity, intrauterine growth restriction and preeclampsia. For the management of obstetric APS, a coordinated medical-obstetric management is essential, and this should start for a preconceptional visit in order to estimate the individual risk for complications, adjust therapies and establish the indications for preconceptional and first-trimester therapy.

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  • - The study aimed to update the 1998 Systemic Lupus Erythematosus (SLE) Core Outcome Set by evaluating existing domains and generating new ones, involving both patients and collaborators in the process.
  • - A survey collected responses from 100 patients and 145 collaborators, revealing that patients focused on life-impact domains while collaborators emphasized clinical aspects, highlighting a need for balanced input from both groups.
  • - Findings showed agreement on some domains for inclusion in the updated SLE Core Outcome Set, while also identifying areas that need more explanation and suggesting new domains for consideration.
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Objectives: This study aims to determine the independent impact of definitions of remission/low disease activity (LDA) on direct/indirect costs (DCs, ICs) in a multicentre inception cohort.

Methods: Patients from 31 centres in 10 countries were enrolled within 15 months of diagnosis and assessed annually. Five mutually exclusive disease activity states (DAS) were defined as (1) remission off-treatment: clinical (c) SLEDAI-2K=0, without prednisone/immunosuppressants; (2) remission on-treatment: cSLEDAI-2K=0, prednisone ≤5 mg/day and/or maintenance immunosuppressants; (3) LDA-Toronto Cohort (TC): cSLEDAI-2K≤2, without prednisone/immunosuppressants; (4) modified lupus LDA state (mLLDAS): SLEDAI-2K≤4, no activity in major organs/systems, no new activity, prednisone ≤7.

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  • Cranial neuropathies (CN) are a rare manifestation of neuropsychiatric lupus, and the study investigates the association of anti-KIF20B antibodies as a possible biomarker for this condition within a large cohort of SLE patients.
  • The research involved 795 patients from a larger cohort, revealing that 29.8% were positive for anti-KIF20B, with a significantly higher positivity rate (70%) in those with CN compared to those without (29.3%).
  • Findings suggest that anti-KIF20B positivity is linked to CN in SLE patients, indicating its potential as a biomarker, though further research is required to confirm these results.
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Aim: This study addresses the challenge of predicting the course of Adult-onset Still's disease (AoSD), a rare systemic autoinflammatory disorder of unknown origin. Precise prediction is crucial for effective clinical management, especially in the absence of specific laboratory indicators.

Methods: We assessed the effectiveness of combining traditional biomarkers with the k-medoids unsupervised clustering algorithm in forecasting the various clinical courses of AoSD-monocyclic, polycyclic, or chronic articular.

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  • - VEXAS syndrome is a new autoinflammatory disease that often affects various organs, with noticeable inflammatory issues in the eyes and orbits.
  • - In a study of 59 VEXAS patients, 45.8% exhibited orbital/ocular problems, with periorbital edema and episcleritis being the most common conditions observed.
  • - There is a significant link between relapsing polychondritis and eye involvement in VEXAS, and patients with eye issues showed higher mortality rates, indicating the need for closer monitoring by healthcare providers.
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Objective: The objective of this study was to analyze the effect of methylprednisolone pulses (MP), given during the first year after the diagnosis of systemic lupus erythematosus (SLE), in achieving prolonged remission according to the degree of lupus activity at presentation.

Methods: We conducted an observational study of routine clinical care data from the Lupus-Cruces-Bordeaux cohort. The end point was prolonged remission (ie, during five consecutive yearly visits).

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In this review, we discuss the current evidence on classic and newer oral anticoagulant therapy, older drugs such as HCQ and statins, and new potential treatment targets in APS. Vitamin K antagonists (VKAs) remain the cornerstone treatment for thrombotic events in APS. In patients fulfilling criteria for definite APS presenting with a first venous thrombosis, treatment with VKAs with a target international normalized ratio (INR) 2.

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Background: Long-term anticoagulant therapy is generally recommended for thrombotic antiphospholipid syndrome (TAPS) patients, however it may be withdrawn or not introduced in routine practice.

Objectives: To prospectively evaluate the risk of thrombosis recurrence and major bleeding in non-anticoagulated TAPS patients, compared to anticoagulated TAPS, and secondly, to identify different features between those two groups.

Patients/methods: Using an international registry, we identified non-anticoagulated TAPS patients at baseline, and matched them with anticoagulated TAPS patients based on gender, age, type of previous thrombosis, and associated autoimmune disease.

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Introduction: Hydroxychloroquine (HCQ) and glucocorticoids (GCs) constitute the oldest and more used drugs in the treatment of systemic lupus erythematosus (SLE). Despite this long experience, both are still subject to a number of uncertainties, mainly regarding the dose.

Areas Covered: We review the main mechanisms of action, the clinical and toxic effects of HCQ and GCs and analyze the recommendations for the use of both in guidelines published since 2018.

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Systemic lupus erythematosus (SLE) is a systemic autoimmune inflammatory disease of unknown cause, with heterogeneity in its clinical presentation, as well as variability in its clinical course and prognosis. The current goal of treatment is to achieve disease remission or a state of low activity, and thereby improve the patient's quality of life. Biological therapy in lupus, unlike other entities, although it has not been fully established, in recent years it has burst onto the scene with important therapeutic novelties.

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Background: Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) and post-COVID condition can present similarities such as fatigue, brain fog, autonomic and neuropathic symptoms.

Methods: The study included 87 patients with post-COVID condition, 50 patients with ME/CFS, and 50 healthy controls (HC). The hemodynamic autonomic function was evaluated using the deep breathing technique, Valsalva maneuver, and Tilt test.

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Glucocorticoids (GCs) continue to be essential agents for the management of systemic lupus erythematosus, since there are no other drugs able to active remission of active disease so rapidly. However, their potential for causing irreversible damage greatly limit their use. Fortunately, some strategies may help take advantage of their huge anti-inflammatory power while limiting GC-induced side effects.

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Glucocorticoids (GCs) remain a cornerstone of the treatment of Systemic Lupus Erythematosus (SLE). Numerous studies have emphasized the risk of damage accrual in SLE patient treated with GC, but currently, it is not possible to dissociate favorable and undesirable effects of GCs because their underlying mechanisms are entangled at the molecular level. Here, we review whether available data suggest that it is possible, feasible and desirable to taper and discontinue GC treatment in SLE.

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: To analyze the characteristics and the predictive factors of the use of rituximab and belimumab in daily practice in patients from the inception cohort Registro Español de Lupus (RELES). : The study included 518 patients. We considered patients treated with biologics who received at least one dose of rituximab or belimumab, and possible indications of those manifestations registered at the same time or in the previous 2 months of the start of the therapy.

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Objective: The goals of this study were to assess the associations of severe nonadherence to hydroxychloroquine (HCQ), objectively assessed by HCQ serum levels, and risks of systemic lupus erythematosus (SLE) flares, damage, and mortality rates over five years of follow-up.

Methods: The Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort is an international multicenter initiative (33 centers throughout 11 countries). The serum of patients prescribed HCQ for at least three months at enrollment were analyzed.

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