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1.
J Clin Med ; 13(16)2024 Aug 22.
Article in English | MEDLINE | ID: mdl-39201105

ABSTRACT

Background/Objectives: The prevalence of Interstitial Lung Disease (ILD) and risk factors for its development in patients with primary Sjögren's syndrome (pSS) are still debated, possibly due to the existence of heterogeneous pSS-related ILD phenotypes. The aims of this study were: 1. To investigate the prevalence and predictive factors for ILD development in a single-center pSS cohort; 2. To characterize different pSS-ILD phenotypes. Methods: Clinical, laboratory and imaging data of pSS patients attending our center from January 2019 to September 2023 were retrospectively analyzed. ILD presence was confirmed on HRCT. Results: Forty-three out of 474 enrolled pSS patients presented ILD (M:F = 6:37), accounting for an overall ILD prevalence of 9.1%. In 19 cases, ILD was the first manifestation of pSS (ILD-onset), while in 24 ILD was diagnosed after pSS (ILD-incident). Compared to ILD-onset, ILD-incident patients more often presented pSS-related hematologic abnormalities (p = 0.012), cutaneous involvement (p = 0.027), inflammatory arthralgias (p = 0.026), C4 hypocomplementemia (p = 0.012) and positive RF (p = 0.031). On the other hand, ILD-onset patients were significantly older at pSS diagnosis (p = 0.008) and presented more severe fibrosis on HRCT (p = 0.008). On the univariate analysis, higher ESSDAI (p = 0.011), Raynaud's phenomenon (p = 0.009), anti-Ro52 (p = 0.031), hypergammaglobulinemia (p = 0.011), Rheumatoid Factor (RF) (p = 0.038) and C4 hypocomplementemia (p = 0.044) at baseline were associated to ILD development during follow-up. On the multivariate analysis, the ESSDAI at baseline (p = 0.05) and Raynaud's phenomenon (p = 0.013) at baseline were the only independent predictors of ILD development. Conclusions: ILD is a relatively common and clinically heterogenous pSS manifestation. Elevated disease activity at pSS onset is a risk factor for ILD development, prompting careful follow-up and intriguingly suggesting that immunomodulatory therapies may prevent ILD.

2.
Int J Mol Sci ; 25(13)2024 Jul 02.
Article in English | MEDLINE | ID: mdl-39000379

ABSTRACT

Hyperinflammatory Coronavirus disease 2019 (COVID-19) and rapidly-progressive interstitial lung diseases (RP-ILD) secondary to inflammatory myopathies (IIM) present important similarities. These data support the use of anti-rheumatic drugs for the treatment of COVID-19. The aim of this study was to compare the efficacy of combining baricitinib and pulse steroids with the Standard of Care (SoC) for the treatment of critically ill COVID-19 patients. We retrospectively enrolled consecutive patients admitted to the Intensive Care Unit (ICU) with COVID-19-pneumonia. Patients treated with SoC (dexamethasone plus remdesivir) were compared to patients treated with baricitinib plus 6-methylprednisolone pulses (Rheuma-group). We enrolled 246 patients: 104/246 in the SoC and 142/246 in the Rheuma-group. All patients presented laboratory findings suggestive of hyperinflammatory response. Sixty-four patients (26.1%) died during ICU hospitalization. The mortality rate in the Rheuma-group was significantly lower than in the SoC-group (15.5 vs. 40.4%, p < 0.001). Compared to the SoC-group, patients in the Rheuma-group presented significantly lower inflammatory biomarker levels after one week of treatment. Higher ferritin levels after one week of treatment were strongly associated with mortality (p < 0.001). In this large real-life COVID-19 cohort, baricitinib and pulse steroids led to a significant reduction in mortality, paralleled by a prompt reduction in inflammatory biomarkers. Our experience supports the similarities between hyperinflammatory COVID-19 and the IIM-associated RP-ILD.


Subject(s)
Azetidines , COVID-19 Drug Treatment , COVID-19 , Drug Therapy, Combination , Intensive Care Units , Methylprednisolone , Purines , Pyrazoles , SARS-CoV-2 , Sulfonamides , Humans , Purines/therapeutic use , Purines/administration & dosage , Male , Female , Azetidines/therapeutic use , Azetidines/administration & dosage , Sulfonamides/therapeutic use , Sulfonamides/administration & dosage , Pyrazoles/therapeutic use , Pyrazoles/administration & dosage , Middle Aged , Aged , Retrospective Studies , Methylprednisolone/therapeutic use , Methylprednisolone/administration & dosage , COVID-19/mortality , COVID-19/complications , Dexamethasone/therapeutic use , Dexamethasone/administration & dosage , Adenosine Monophosphate/analogs & derivatives , Adenosine Monophosphate/therapeutic use , Adenosine Monophosphate/administration & dosage , Treatment Outcome , Alanine/analogs & derivatives , Alanine/therapeutic use , Alanine/administration & dosage
3.
Lupus Sci Med ; 11(2)2024 Jul 29.
Article in English | MEDLINE | ID: mdl-39074950

ABSTRACT

OBJECTIVE: To assess the impact of different disease activity patterns-long quiescent (LQ), chronically active (CA) and relapsing-remitting (RR)-on health-related quality of life (HRQoL) in a cohort of patients with systemic lupus erythematosus (SLE). METHODS: A retrospective, monocentric analysis of prospectively collected data. Adult SLE outpatients were enrolled between 2017 and 2021.For each year of follow-up, three disease activity patterns were defined: LQ if at each visit clinical Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Activity Index (SELENA-SLEDAI)=0, Physician Global Assessment (PGA)=0; CA if at each visit clinical SELENA-SLEDAI >0, PGA >0; RR if patients presented active disease in at least one visit during the observation period, interspersed with periods of remission. These patterns were applied to the year and the 3 years before enrolment.At enrolment, each patient completed: Short Form 36 (SF-36), Lupus Impact Tracker, Functional Assessment of Chronic Illness Therapy (FACIT), Hospital Anxiety and Depression Scale (HADS). The correlation between disease patterns and Patient-Reported Outcomes was analysed. RESULTS: 241 SLE patients were enrolled, of which 222 had complete clinical data for the 3-year period before enrolment. Both in the year and during the 3 years before enrolment, the most frequent disease pattern was the LQ (154/241 and 122/222 patients, respectively), followed by RR (53/241 and 92/222 patients, respectively) and CA (34/241 and 8/222 patients, respectively).At baseline, fibromyalgia, organ damage, age and daily glucocorticoid dose were associated with worse HRQoL.At the multivariable analysis, after adjusting for confounding factors, patients with LQ disease during the 3 years before enrolment presented a better physical HRQoL (SF-36 physical component summary, regression coefficient=3.2, 95% CI 0.51-5.89, p=0.02) and minor depressive symptoms (HADS-D, regression coefficient=-1.17, 95% CI -2.38 to 0.0.27, p=0.055), compared with patients with CA/RR disease. CONCLUSION: A persistently quiescent disease may have a positive impact on patients' physical HRQoL and on depressive symptoms. However, this condition appears insufficient to obtain a significant improvement in mental health, fatigue and disease burden among patients with SLE.


Subject(s)
Lupus Erythematosus, Systemic , Quality of Life , Humans , Lupus Erythematosus, Systemic/psychology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/physiopathology , Quality of Life/psychology , Female , Adult , Male , Middle Aged , Retrospective Studies , Severity of Illness Index , Patient Reported Outcome Measures , Depression/psychology , Depression/epidemiology
4.
Clin Exp Rheumatol ; 42(7): 1321-1332, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38976289

ABSTRACT

OBJECTIVES: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of systemic pauci-immune necrotising vasculitides involving small vessels, characterised by the presence of specific ANCA autoantibodies directed to leukocyte proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) and subdivided into three clinical entities: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The aetiology of AAV is unknown and many genetic, epigenetic and environmental factors have been reported to be involved in pathogenesis. Smoking is widely recognised as a risk factor for the development of many autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus. This systematic review will analyse known data about the role of smoking in the development, clinical presentation and outcome of AAV. METHODS: Articles that examined interactions between tobacco smoking and AAV (GPA, MPA, EGPA) were included. All articles selected were in English. No limitation on publication date was established. Case reports were excluded. The systematic search was performed using PubMed/Medline and Cochrane Library databases. RESULTS: The search provided a total of 131 articles. Three studies were added, obtained from the review of the reference lists of articles. 70 were removed because they were duplicated or written in languages other than English. The title and abstract of 64 articles were screened. Of these, 30 were excluded as the title and/or abstract did not meet the inclusion criteria. Thus, 34 remained for full-text review, of which 8 were excluded. 26 articles were therefore included in this review. The role of smoking in AAV development is unclear. AAV patients current smoking appear appear to be younger and more frequently males, with a lower prevalence of EGPA and MPA than GPA. Ever smokers show higher relapse rate. Smoking seems to be associated with a higher risk of cardiovascular events during follow-up. Smokers incur an increased risk of infections. Finally, many data support smoking as a risk factor for end stage renal disease and mortality in AAV patients. CONCLUSIONS: Current data support the hypothesis that smoking influences prevalence, clinical phenotype and prognosis of ANCA-associated vasculitis. However, further studies are required to fully determine its role.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Tobacco Smoking , Humans , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/epidemiology , Risk Factors , Tobacco Smoking/adverse effects , Antibodies, Antineutrophil Cytoplasmic/blood , Prognosis , Microscopic Polyangiitis/immunology , Microscopic Polyangiitis/epidemiology , Risk Assessment , Granulomatosis with Polyangiitis/immunology , Granulomatosis with Polyangiitis/epidemiology , Granulomatosis with Polyangiitis/etiology , Biomarkers/blood
5.
Lupus Sci Med ; 11(2)2024 Jul 23.
Article in English | MEDLINE | ID: mdl-39043606

ABSTRACT

OBJECTIVES: To analyse the response to immunosuppressants (IS) in extrarenal flares of SLE to determine the most appropriate timing during follow-up for a change in therapeutic strategy. METHODS: Observational cohort study including a total of 81 patients with SLE with extrarenal flares requiring a change in IS over the period 2015-2022. Baseline clinical variables were described, and follow-up data at 1, 3, 6 and 12 months time-points were collected. RESULTS: Among patients flaring that achieved lupus low disease activity state (LLDAS5) at 12 months of follow-up, we identified two subgroups ('late responders' and 'early responders'), which showed no significant differences in demographic characteristics, baseline clinical data, cumulative dosage of glucocorticoids or type of IS. Cox model analysis revealed a significant association of a change in IS (p=0.019) and achieving LLDAS5. Contingency table analysis indicated a significant relationship (p=0.004) between IS change at 6 months and individuals achieving LLDAS5 and remission at 12 months. CONCLUSIONS: Our findings suggest that clinical improvement of extrarenal flares typically occurs within 6 months of initiating IS. This timeframe could represent an appropriate timing to evaluate the response in a treat-to-target approach in SLE.


Subject(s)
Immunosuppressive Agents , Lupus Erythematosus, Systemic , Humans , Lupus Erythematosus, Systemic/drug therapy , Female , Male , Immunosuppressive Agents/therapeutic use , Adult , Middle Aged , Time Factors , Glucocorticoids/therapeutic use , Treatment Outcome , Cohort Studies , Severity of Illness Index , Follow-Up Studies
6.
Clin Exp Rheumatol ; 42(5): 1104-1114, 2024 05.
Article in English | MEDLINE | ID: mdl-38743446

ABSTRACT

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations and a relapsing-remitting course. SLE pathogenesis is the result of complex interactions between ethnic, genetic, epigenetic, immunoregulatory, hormonal and environmental factors, and several aspects of these multifactorial connections are still unclear. Overall, for the disease development, an environmental trigger may induce immunological dysfunction in genetically predisposed individuals. This review aims to summarise the most relevant data on the impact of environmental factors on the incidence of SLE and on disease activity and damage in patients with an established diagnosis of SLE.


Subject(s)
Gene-Environment Interaction , Lupus Erythematosus, Systemic , Humans , Lupus Erythematosus, Systemic/immunology , Lupus Erythematosus, Systemic/genetics , Lupus Erythematosus, Systemic/diagnosis , Risk Factors , Genetic Predisposition to Disease , Incidence , Environmental Exposure/adverse effects , Environment
7.
RMD Open ; 10(2)2024 Apr 12.
Article in English | MEDLINE | ID: mdl-38609320

ABSTRACT

OBJECTIVE: The objective is to evaluate perscriptions of belimumab (BEL), how these have changed over the years and their impact on clinical outcomes in patients with systemic lupus erythematosus (SLE). METHODS: This is a retrospective analysis of prospectively collected data. We retrieved demographic and clinical data and concomitant therapies at BEL starting (baseline). Disease activity was assessed at baseline and after 6 and 12 months and organ damage at baseline and at the last visit. RESULTS: From 422 patients followed in the Pisa SLE cohort, 102 patients received BEL and were included and 22 (21.6%) were immunosuppressant (IS)-naïve. Lupus Low Disease Activity State (LLDAS) with a glucocorticoid (GC) dosage ≤5 mg/day (LLDAS5) and remission were achieved by 47% and 38% of patients at 6 months, and by 75% and 66% at 12 months. Comparing IS-naïve patients with those who received BEL after at least one conventional IS, we did not find significant differences in baseline characteristics and in the achievement of LLDAS5 and remission. Despite at baseline we did not observe significant differences in mean GC daily dosage, IS-naïve patients were taking a significantly lower GC daily dose at 6 and 12 months. Interestingly, IS-naïve patients were more common in the most recent years. CONCLUSIONS: Our data confirm that BEL is effective in controlling disease activity, and in recent years BEL has been considered as an earlier treatment option before other IS. Early introduction of BEL can be at least as effective as a step-up approach and can help to reduce the GC dosage.


Subject(s)
Antibodies, Monoclonal, Humanized , Lupus Erythematosus, Systemic , Humans , Retrospective Studies , Antibodies, Monoclonal, Humanized/therapeutic use , Glucocorticoids/therapeutic use , Immunosuppressive Agents/therapeutic use , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/epidemiology , Prescriptions
8.
RMD Open ; 10(2)2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38670557

ABSTRACT

OBJECTIVES: Undifferentiated connective tissue diseases (UCTDs) are systemic autoimmune conditions that cannot be diagnosed nor classified as defined CTD; the majority maintains an undifferentiated profile (stable UCTD, sUCTD) over time. Data on long-term outcomes of sUCTD are lacking. METHODS: Retrospective longitudinal analysis of an inception cohort of 141 patients with sUCTD.Disease evolution and damage accrual were evaluated at 1, 5 and 10 years. Partial least square (PLS) regression was used to identify the basal variables contributing to damage accrual at 1, 5 and 10 years of follow-up. Trend of damage over time was compared with a cohort of age-matched and sex-matched patients with systemic lupus erythematosus (SLE) by means of Nelson-Aalen analysis. RESULTS: 11.3% of patients evolved to a definite CTD after a median 11 years (IQR 6-25) from the first symptom. At last visit, 10% were on glucocorticoids and 6% on immunosuppressive therapy. In 27.3%, at least one item of organ damage was recorded according to the SLICC/DI score (mean score 1.19±0.46). At PLS analysis, age at diagnosis and age at first symptoms were related to damage at 1 year, not taking antimalarials and taking immunosuppressants were associated with damage at 5 years.The mean survival without damage was 9.3 years in sUCTD and 8.4 years in SLE. The 10-year probability without damage was 62% and 23% in SLE and sUCTD, respectively (p=0.015). CONCLUSIONS: Although less significantly impacted than in patients with SLE, in the long-term UCTDs can accumulate organ damage and evolve into defined connective tissue diseases.


Subject(s)
Disease Progression , Lupus Erythematosus, Systemic , Undifferentiated Connective Tissue Diseases , Humans , Male , Female , Adult , Middle Aged , Retrospective Studies , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/drug therapy , Undifferentiated Connective Tissue Diseases/complications , Undifferentiated Connective Tissue Diseases/epidemiology , Undifferentiated Connective Tissue Diseases/diagnosis , Longitudinal Studies , Immunosuppressive Agents/therapeutic use , Severity of Illness Index , Glucocorticoids/therapeutic use
9.
Clin Exp Rheumatol ; 42(3): 583-592, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38545801

ABSTRACT

Systemic lupus erythematosus (SLE) is classically regarded as the landmark of systemic autoimmune diseases, characterised by protean, multi-systemic manifestations and a highly variable clinical course.Over the last years, both clinical and translational clinical research efforts led to significant steps forward in management and treatment of SLE. However, numerous aspects of SLE, from pathogenesis to treatment, still remain challenging, and several unmet needs persist for both patients and physicians. Following the previous annual reviews of this series, herewith, we aim to report the most relevant new updates on SLE, issued in 2023. In particular, we focused on biomarkers, clinical aspects and outcomes, comorbidities, as well as new treatment targets and real-world evidence.


Subject(s)
Lupus Erythematosus, Systemic , Physicians , Humans , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/drug therapy , Biomarkers , Comorbidity
10.
Clin Exp Rheumatol ; 42(3): 666-670, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37812486

ABSTRACT

OBJECTIVES: To evaluate adherence to medication in patients with systemic autoimmune diseases (SAD), comparing pregnant and non-pregnant women. METHODS: 200 patients with SAD were consecutively enrolled, 100 pregnant and 100 non-pregnant women. Each patient completed the 8-item Morisky Medication Adherence Scale (MMAS-8), one copy for hydroxychloroquine (HCQ) and one for other treatments for rheumatic disease, and Hospital Anxiety and Depression Scale (HADS). RESULTS: No significant differences were found in ongoing therapies between pregnant and non-pregnant women. 148 patients (74.0%) were taking HCQ and 160 (80.0%) other therapies for rheumatic disease. The mean MMAS-8 score was >6 in all groups indicating a good adherence, on average. The rate of patients with good medication adherence was higher in pregnant patients (73.9% vs. 63.3% and 76.5% vs. 64.5%, for HCQ and other therapies, respectively) although this difference was not statistically significant. Eight patients had very poor medical adherence, and all were non-pregnant women. Anxiety (15% of patients) was associated to low medication adherence for drugs other than HCQ (p=0.02), while depression (4% of patients) did not seem to have an impact on adherence. CONCLUSIONS: In our cohort we recorded a good adherence to prescribed medication, although adequate adherence was not achieved in about 30% of patients, confirming that non-adherence is an important issue in SAD. It is difficult to define a profile of patients at risk of poor adherence, but it appears important to implement communication and adherence monitoring strategies since strict monitoring also during pregnancy could improve medical adherence.


Subject(s)
Autoimmune Diseases , Rheumatic Diseases , Pregnancy , Female , Humans , Prospective Studies , Hydroxychloroquine/therapeutic use , Rheumatic Diseases/drug therapy , Medication Adherence , Autoimmune Diseases/drug therapy , Autoimmune Diseases/chemically induced
11.
Rheumatology (Oxford) ; 63(2): 376-384, 2024 Feb 01.
Article in English | MEDLINE | ID: mdl-37094218

ABSTRACT

OBJECTIVES: To describe phenotypes and outcomes of extra-renal flares in SLE, to identify clusters of extra-renal flares based on baseline features, and to develop a machine learning (ML) tool capable of predicting 'difficult to treat' (D2T) flares. METHODS: Extra-renal flares that occurred in our cohort over the last five years with at least one year of follow-up were included. Baseline clinical variables were described and flares assigned to clusters. Attainment of remission and low disease activity state (LLDAS) at 12 months were compared. Flares were then considered 'D2T' in case of non-attainment of LLDAS at 6 and 12 months. Baseline features were used to train a ML model able to predict future D2T-flares, at admission. Traditional approaches were then compared with informatic techniques. RESULTS: Among 420 SLE patients of the cohort, 114 flares occurred between 2015 and 2021; 79 extra-renal flares, predominantly mucocutaneous (24.1%) and musculoskeletal (45.6%), were considered. After 12 months, 79.4% and 49.4% were in LLDAS and in remission, respectively, while 17 flares were classified as D2T (21.5%); D2T flares received a higher cumulative and daily dose of glucocorticoids. Among the clusters, cluster 'D' (mild-moderate flares with mucocutaneous manifestations in patients with history of skin involvement) was associated with the lowest rate of remission. Among clinical data, not being on LLDAS at 3 months was the unique independent predictor of D2T flares. CONCLUSIONS: Our clusterization well separates extra-renal flares according to their baseline features and may propose a new identification standard. D2T flares, especially refractory skin manifestations, are frequent in SLE and represent an unmet need in the management of the disease as they are associated with higher glucocorticoid (GC) dosage and risk of damage accrual. Our ML model could help in the early identification of D2T flares, flagging them to elevate the attention threshold at admission.


Subject(s)
Lupus Erythematosus, Systemic , Humans , Longitudinal Studies , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/complications , Glucocorticoids/therapeutic use , Kidney , Risk Assessment , Severity of Illness Index
12.
Ann Rheum Dis ; 2023 Nov 22.
Article in English | MEDLINE | ID: mdl-38050029

ABSTRACT

OBJECTIVES: Fatigue is prevalent in people with inflammatory rheumatic and musculoskeletal diseases (I-RMDs) and recognised as one of the most challenging symptoms to manage. The existence of multiple factors associated with driving and maintaining fatigue, and the evidence about what improves fatigue has led to a multifaceted approach to its management. However, there are no recommendations for fatigue management in people with I-RMDs. This lack of guidance is challenging for those living with fatigue and health professionals delivering clinical care. Therefore, our aim was to develop EULAR recommendations for the management of fatigue in people with I-RMDs. METHODS: A multidisciplinary taskforce comprising 26 members from 14 European countries was convened, and two systematic reviews were conducted. The taskforce developed the recommendations based on the systematic review of evidence supplemented with taskforce members' experience of fatigue in I-RMDs. RESULTS: Four overarching principles (OAPs) and four recommendations were developed. OAPs include health professionals' awareness that fatigue encompasses multiple biological, psychological and social factors which should inform clinical care. Fatigue should be monitored and assessed, and people with I-RMDs should be offered management options. Recommendations include offering tailored physical activity and/or tailored psychoeducational interventions and/or, if clinically indicated, immunomodulatory treatment initiation or change. Patient-centred fatigue management should consider the individual's needs and preferences, their clinical disease activity, comorbidities and other psychosocial and contextual factors through shared decision-making. CONCLUSIONS: These 2023 EULAR recommendations provide consensus and up-to-date guidance on fatigue management in people with I-RMDs.

13.
J Clin Med ; 12(9)2023 May 08.
Article in English | MEDLINE | ID: mdl-37176788

ABSTRACT

Treat-to-target is a therapeutic approach based on adjustments to treatment at set intervals in order to achieve well-defined, clinically relevant targets. This approach has been successfully applied to many chronic conditions, and in rheumatology promising results have emerged for rheumatoid arthritis. For systemic lupus erythematosus (SLE), defining the most meaningful treatment targets has been challenging, due to disease complexity and heterogeneity. Control of disease activity, the reduction of damage accrual and the patient's quality of life should be considered as the main targets in SLE, and several new drugs are emerging to achieve these targets. This review is focused on describing the target to achieve in SLE and the methods to do so, and it is also aimed at discussing if treat-to-target could be a promising approach also for this complex disease.

14.
Lupus Sci Med ; 10(1)2023 04.
Article in English | MEDLINE | ID: mdl-37185240

ABSTRACT

BACKGROUND: SLE is an autoimmune disease that predominantly affects women. As most epidemiological and interventional studies are on populations with a clear female prevalence, the influence of gender in disease course, drug response and damage accrual is yet to be fully explored and comprehended. OBJECTIVES: To describe gender differences in disease course, comorbidities, use of medications and long-term outcomes of a large cohort of patients with SLE. METHODS: Retrospective gender-based analysis of prospectively collected data from a monocentric cohort of Caucasian patients with SLE with at least 1 year of follow-up. RESULTS: 417 patients were included, 51 men and 366 women. Men displayed a significantly higher median age at disease onset and diagnosis and a higher prevalence of late-onset SLE, serositis at disease onset, antiphospholipid syndrome (APS) and use of mycophenolate within the first year of disease. Women had a higher prevalence of haematological abnormalities, a higher cumulative exposure to azathioprine and higher cumulative dose of glucocorticoids at 5 years. Male patients had a shorter time to first damage item and a higher prevalence of damage at 1 and 5 years, but this association was no longer significant when late-onset patients were excluded. No differences were found in prevalence of childhood onset, delay between onset and diagnosis, time to renal involvement and histology, cumulative autoantibody positivity, number of flares and hospitalisations, median SLE Damage Index score, type of damage, age and time to first cardiovascular event, chronic kidney disease and death. CONCLUSIONS: In our cohort, clinical manifestations and disease course were similar in male and female patients; however, male patients displayed higher prevalence of APS and early damage accrual probably due to the later disease onset. These data highlight the importance of an intensive follow-up, prevention and treatment of complications in this category of patients, especially in the first years of disease.


Subject(s)
Antiphospholipid Syndrome , Lupus Erythematosus, Systemic , Humans , Male , Female , Retrospective Studies , Sex Factors , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/epidemiology , Glucocorticoids/adverse effects , Disease Progression
15.
J Clin Med ; 12(10)2023 May 12.
Article in English | MEDLINE | ID: mdl-37240535

ABSTRACT

Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren's Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Presence of lung involvement in pSS subjects can remain subclinical for a long time; therefore, patients should be actively screened, and lung ultrasound is currently being investigated as a potential low cost, radiation-free, easily repeatable screening tool for detection of ILD. In contrast, rheumatologic evaluation, serology testing, and minor salivary gland biopsy are crucial for the recognition of pSS in apparently idiopathic ILD patients. Whether the HRCT pattern influences prognosis and treatment response in pSS-ILD is not clear; a UIP pattern associated with a worse prognosis in some studies, but not in others. Many aspects of pSS-ILD, including its actual prevalence, association with specific clinical-serological characteristics, and prognosis, are still debated by the current literature, likely due to poor phenotypic stratification of patients in clinical studies. In the present review, we critically discuss these and other clinically relevant "hot topics" in pSS-ILD. More specifically, after a focused discussion, we compiled a list of questions regarding pSS-ILD that, in our opinion, are not easily answered by the available literature. We subsequently tried to formulate adequate answers on the basis of an extensive literature search and our clinical experience. At the same, we highlighted different issues that require further investigation.

16.
Clin Exp Rheumatol ; 41(5): 997-1008, 2023 May.
Article in English | MEDLINE | ID: mdl-37133502

ABSTRACT

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations and a relapsing-remitting course. New data regarding pathogenic pathways, biomarkers and clinical manifestations of SLE are emerging, and new drugs and therapeutic protocols have been proposed to improve the control of disease activity. Furthermore, new insights into comorbidities and reproductive health in SLE patients are constantly emerging.This annual review aims to summarise the most relevant data on SLE that was published in 2022.


Subject(s)
Autoimmune Diseases , Lupus Erythematosus, Systemic , Humans , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/epidemiology , Comorbidity , Biomarkers/metabolism
17.
JAMA Dermatol ; 159(2): 224-226, 2023 02 01.
Article in English | MEDLINE | ID: mdl-36427219

ABSTRACT

This case report describes 2 women with severe and refractory discoid lupus erythematosus that was treated with anifrolumab.


Subject(s)
Lupus Erythematosus, Discoid , Lupus Erythematosus, Systemic , Humans , Antibodies, Monoclonal, Humanized/therapeutic use , Lupus Erythematosus, Discoid/drug therapy
18.
Clin Exp Rheumatol ; 41(1): 145-150, 2023 Jan.
Article in English | MEDLINE | ID: mdl-35894063

ABSTRACT

OBJECTIVES: Several studies show that age at onset has an impact on the clinical-serological presentation, comorbidities and disease course of patients with systemic lupus erythematosus (SLE). We evaluated whether, in patients with recent onset SLE, the age at onset correlates with clinical-serological manifestations and with comorbidities. METHODS: We analysed 171 patients with a SLE diagnosis obtained within 12 months of diagnosis enrolled in the Early Lupus project. Based on the age of onset of the first disease symptom, they were stratified into 2 groups: early onset (18-45 years) and late onset (>45 years). The analysis was replicated by stratifying patients based on age at diagnosis (fulfillment of ACR classification criteria). Each comparison was made at baseline and at 36 months of follow-up. RESULTS: Baseline: patients with late onset displayed comorbidities (hypertension, dyslipidemia and osteoporosis) more frequently than early onset group. 11.4% of late onset patients had a malignancy in medical history, not recorded in the early onset cohort. The two groups differed neither in organ involvement (domain BILAG) nor in disease activity (ECLAM). Patients with early onset showed a disease with signs of higher serologic activity (higher frequency of anti-dsDNA positivity and lower mean C3 and C4 levels) and had malar rash more frequently than the late onset group (36.2% vs. 18.2%, p=0.042). Similar results were obtained by stratifying patients by age of diagnosis (18-45 years and >45 years), except for the higher frequency of discoid rash in the group with age at diagnosis >45 years (18% vs. 6.6%, p=0.045). 36 months: the 2 groups of patients independently of the stratification applied did not differ in the accumulation of damage, but showed a different pattern of 8 organ involvement. Musculoskeletal involvement was more frequent both in the late onset group (18.6% vs. 7.3%, p=0.043) and in the group with age at diagnosis >45 years (20.4% vs. 5.9%, p=0.009) compared to their counterparts, while renal involvement was more frequent in the group with age at diagnosis 18-45 years (21.4% vs. 6.1%, p=0.03).A sub analysis at 36 months on patients without hypertension and osteoporosis at enrollment showed that patients with older age at onset had a higher frequency of these comorbidities, compared to their counterparts. CONCLUSIONS: In our cohort, younger disease SLE onset seems to correlate with a more active immunological profile, while late onset with a higher incidence of comorbidities.


Subject(s)
Hypertension , Lupus Erythematosus, Systemic , Osteoporosis , Humans , Adolescent , Young Adult , Adult , Middle Aged , Age of Onset
19.
Best Pract Res Clin Rheumatol ; 37(4): 101939, 2023 12.
Article in English | MEDLINE | ID: mdl-38485601

ABSTRACT

Systemic Lupus Erythematosus (SLE) imposes a great burden on the lives of patients. Patients' and physicians' concerns about the disease diverge considerably. Physicians focus on controlling disease activity to prevent damage accrual, while patients focus on symptoms that impact on Health-Related Quality of Life (HRQoL). We explored the physicians' and patients' perspective and the potential role of Patient Reported Outcomes (PROs). Physicians are aware of the theoretical usefulness of PROs to collect information deriving from the patients' perspective. However, they often do not know how to interpret and use these questionnaires in a real shared therapeutic strategy. For the patients, it's important to be seen as a whole person with a true consideration of how they feel and function. Strategies to help bridge the communication gap could include: better use of time during visits, preparing for the consultation, a more understandable lay language used by the doctor, a dedicated nurse.


Subject(s)
Communication , Lupus Erythematosus, Systemic , Patient Reported Outcome Measures , Physician-Patient Relations , Quality of Life , Humans , Lupus Erythematosus, Systemic/therapy , Lupus Erythematosus, Systemic/psychology , Lupus Erythematosus, Systemic/physiopathology , Surveys and Questionnaires
20.
Lupus Sci Med ; 9(1)2022 06.
Article in English | MEDLINE | ID: mdl-35701044

ABSTRACT

OBJECTIVE: It is still a matter of debate whether low-dose acetylsalicylic acid (LDASA) should be prescribed to all patients with SLE during pregnancy. This study aimed at investigating the impact of LDASA on pregnancy outcomes in patients with SLE without history of renal involvement and without antiphospholipid antibodies (aPL). METHODS: This is a retrospective analysis of prospectively monitored pregnancies at seven rheumatology centres. Previous/current renal involvement and aPL positivity were the exclusion criteria. Adverse pregnancy outcome (APO) is the composite outcome of the study and included proteinuric pre-eclampsia, preterm delivery <37 weeks, small-for-gestational age infant, low birth weight <2500 g, intrauterine growth restriction and intrauterine fetal death after 12 weeks of gestation of a morphologically normal fetus. RESULTS: 216 pregnancies in 187 patients were included; 82 pregnancies (38.0%) were exposed to LDASA treatment. No differences in terms of age at conception, disease duration, clinical manifestations, comorbidities and disease flare during pregnancy were observed between patients taking LDASA and those who did not take LDASA during pregnancy. APO was observed in 65 cases (30.1%), including 13 cases (6.1%) of pre-eclampsia. The incidence of all complications was similar in the two groups. However, it is interesting to note that pre-eclampsia had lower frequency in patients taking LDASA versus those not taking LDASA (2.4% vs 8.3%, p=0.14). CONCLUSIONS: In pregnant patients with SLE without renal involvement and were aPL-negative, there is a low risk of severe obstetric complications, such as early pre-eclampsia. LDASA treatment does not provide a statistically significant advantage over these complications. However, a careful individual risk-benefit balance is warranted.


Subject(s)
Lupus Erythematosus, Systemic , Pre-Eclampsia , Aspirin/adverse effects , Female , Humans , Infant, Newborn , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/epidemiology , Pre-Eclampsia/drug therapy , Pre-Eclampsia/epidemiology , Pregnancy , Pregnancy Outcome/epidemiology , Retrospective Studies
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