IgG4-related disease: Effectiveness evaluation through Umehara-Okazaki 2011 and ACR/EULAR 2019 diagnostic criteria.

BACKGROUND: IgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting multiple organs. It presents with common clinical, radiological, and serological characteristics. This study aims to compare the latest two IgG4-RD classification and diagnostic criteria: Umehara-Okazaki 2011 and ACR/EULAR 2019. MATERIAL AND METHODS: In a retrospective cross-sectional study conducted across two centers from January 2010 to July 2023, we included patients suspected of having IgG4-RD from various hospital departments. Patients finally diagnosed with other pathologies were excluded. The remaining suspected IgG4-RD cases were evaluated using both Umehara-Okazaki 2011 and ACR/EULAR 2019 criteria. RESULTS: Out of 34 patients with a clinical diagnosis of IgG4-RD, the Umehara-Okazaki 2011 classified 20 patients: 5 as definitive, 7 as probable, and 8 as possible cases. Applying the ACR/EULAR 2019 criteria to the same cohort resulted in the diagnosis of 9 patients. Notably, retroperitoneal fibrosis and aortitis were the most prevalent form of presentation, accounting for 25% and 22.2% of cases classified under the 2011 and 2019 criteria, respectively. DISCUSSION: The more recent and stringent ACR/EULAR 2019 criteria focus on histopathology, various forms of presentation, and analytical data, allow for a more accurate classification of patients.

Real-world effectiveness and persistence of secukinumab in the treatment of patients with psoriatic arthritis.

Introduction: Psoriatic arthritis (PsA) is a complex and heterogeneous inflammatory disease. Secukinumab, a biologic disease-modifying antirheumatic drug (bDMARD), has extensive clinical evidence of efficacy and safety in the treatment of PsA but data in clinical practice are still limited. This study aims to provide real-world evidence on secukinumab use, effectiveness, and persistence in PsA. Methods: A retrospective, multicenter study was conducted on patients diagnosed with PsA and treated with secukinumab up to June 2021 at 12 centers in the Valencian Community (Spain). Data on DAS28-CRP, DAPSA, Tender and Swollen Joint Counts (TJC, SJC), enthesitis, dactylitis, skin and nail involvement, pain, patient and physician global assessment (ptGA, phGA) using 100-mm visual analog scale (VAS), and persistence for up to 24 months were collected. Results: A total of 178 patients were included (49% men; mean [standard deviation, SD] age: 51.4 [10.5] years; 39% obese). Secukinumab was used as a first-, second-, or ≥ third-line bDMARD in 37, 21, and 42% of patients, respectively. The percentage of patients achieving at least low disease activity (DAS28-CRP ≤ 3.2) increased from 25% at baseline to 66% at month 6 (M6) and was maintained (75%) up to M24. Mean (SD) DAS28-CRP baseline values (3.9 [1.2]) decreased to 2.9 (1.1) (p < 0.001) at M6 and remained low through M24 (2.6 [1.1]) (p < 0.001). Secukinumab also improved peripheral arthritis increasing the percentage of patients with TJC = 0 (20% baseline; 57% M24) and SJC = 0 (37% baseline; 80% M24). Treatment reduced the percentage of patients with enthesitis (25% baseline; 6% M24), dactylitis (20% baseline; 4% M24), and skin (70% baseline; 17% M24), and nail (32% baseline; 2% M24) involvement. Additionally, we observed improvements in the mean pain VAS (-26.4 mm M24), ptGA (-26.2 mm M24), and phGA (-24.8 mm M24). Secukinumab showed an overall 24-month persistence rate of 67% (95% confidence interval [CI]: 60-74%). Patients receiving first-line secukinumab showed the highest 24-month persistence rate (83, 95% CI: 73-92; p = 0.024). Conclusion: Secukinumab showed long-term effectiveness across the six key PsA domains thus reducing disease activity and pain, which are major treatment goals. This was accompanied by high persistence rates, especially in bDMARD naive patients.

Safety and effectiveness of bDMARDs during pregnancy in patients with rheumatic diseases: Real-world data from the BIOBADASER registry.

INTRODUCTION: Inflammatory rheumatic diseases usually affect women of childbearing age treated with biologic drugs. However, there is a lack of literature on the efficacy and toxicity of biologic disease-modifying drugs during pregnancy. The aim of this study was to determine the presence of pregnant patients treated with bDMARDs in a real-world dataset and to examine the impact of pregnancy and lactation on the evolution of rheumatic disease in a registry of Spanish patients. METHOD: This was a multicentre prospective study with a real-world setting. Information was obtained from BIOBADASER registry. Patients included are women who got pregnant until November 2020 from 19 rheumatology units. We conducted proportions, means, and standard deviations (SD) to describe the study population and the use of treatments. T-test and Chi-square test were applied to assess differences between groups. RESULT: Ninety cases of pregnancy were registered (n=68 full-term pregnancies; n=22 spontaneous miscarriages). Most of the cases discontinued bDMARDs during pregnancy (78.9%) but 13 cases continued treatment during pregnancy, mainly using certolizumab pegol. These cases were obtaining better management of rheumatic disease, although the differences were not statistically significant [DAS28-CRP, 2.9 (SD: 1.6) vs. 2.0 (1.2), p=.255; DAS28-ESR, 2.2 (1.0) vs. 1.7 (.5), p=.266]. No serious adverse events were reported during pregnancy and lactation. CONCLUSION: Being pregnant is still an uncommon condition in patients with rheumatic diseases and using bDMARDs. Our results show that rheumatic disease tended to progress better during pregnancy in patients who continued to take bDMARDs.

Real-world experience with secukinumab in the entire axial spondyloarthritis spectrum.

Background: Secukinumab is a biologic disease-modifying antirheumatic drug (bDMARD) that has demonstrated efficacy in the treatment of axial spondyloarthritis (axSpA, i.e., ankylosing spondylitis and non-radiographic axSpA) across various clinical trials. However, data of secukinumab in clinical practice is still limited. Here, we aimed to provide real-world data on secukinumab use, effectiveness, and persistence in axSpA. Patients and methods: Retrospective, multicenter study of patients with a diagnosis of axSpA treated with secukinumab at 12 centers up to June 2021 in the Valencian Community (Spain). Information was gathered on BASDAI measurement, pain, patient and physician global assessment (ptGA, phGA) using a 100-mm visual analog scale (VAS), persistence and other secondary variables by treatment line (first, second, and ≥ third) for up to 24 months. Results: 221 patients were included (69% men; mean age [standard deviation, SD]: 46.7 [12.1] years old). Secukinumab was used as a first-line bDMARD in 38% of patients, as a second-line in 34% and as a ≥ hird-line in 28%. The percentage of patients achieving low disease activity (BASDAI<4) increased from 9% at baseline to 48% at month 6 and was maintained (49%) up to month 24. The greatest improvement in BASDAI was observed in naïve patients (month 6: -2.6; month 24: -3.7), followed by second-line (month 6: -1.9; month 24: -3.1) and ≥ third-line (month 6: -1.3; month 24: -2.3) patients. Reductions in mean pain VAS (-23.3; -31.9), ptGA (-25.1; -31.9) and phGA (-25.1; -31) were also observed at 6 and 24 months. Secukinumab showed an overall 12-months persistence rate of 70% (95% confidence interval [CI]: 63-77%) and a 24-months persistence rate of 58% (95% CI, 51-66%). Patients receiving first-line secukinumab had the highest 24-months persistence rate (p = 0.05). Conclusion: Secukinumab improved disease activity in axSpA patients, especially in naive, and second-line patients, which was accompanied by high persistence rates up to 24 months.

Long-term retention of golimumab treatment in clinical practice in a large cohort of patients with rheumatoid arthritis, axial spondyloarthritis and psoriatic arthritis.

AIM: To assess the golimumab retention rate during up to 8 years of follow up, and any associated factors. METHODS: Retrospective analysis of the BIOBADASER (Spanish registry of biological drugs) database, assessing all adults who had ever started golimumab >6 months before the analysis for an approved indication (rheumatoid arthritis [RA], axial spondyloarthritis [SpA] or psoriatic arthritis [PsA]). RESULTS: Among 885 patients (RA 267, axial SpA 370, PsA 248) receiving 944 cycles of golimumab, the retention rate of golimumab was 71.1% (95% confidence interval: 68.0-73.9) at year 1% and 37.7% (95% CI: 33.3-42.1) at year 7 and at year 8. Retention was higher when golimumab was used as the first biological drug (81.7% at year 1, 49.9% at year 7, p < 0.001). In Cox regression analysis, factors associated with golimumab retention included use as first-line therapy (hazard ratio [HR] for discontinuation 1.52 for second- and 1.79 for third/later-line vs. first-line), use in axial SpA or PsA rather than RA (HR for axial SpA vs. RA 0.59, for PsA vs. Rheumatoid arthritis 0.67), and treatment with concomitant methotrexate (HR 0.67). Factors associated with golimumab discontinuation were corticosteroid use (HR 1.46) and disease activity above median (HR 1.29) at golimumab initiation. CONCLUSION: Based on this retrospective analysis of the BIOBADASER registry, nearly two-fifths (37.7%) of adult rheumatology patients initiating golimumab will remain on treatment for 8 years, with a higher probability of retention in axial SpA or PsA indications and when golimumab is used as first biologic.

Recurrent pericarditis after Covid-19.

Patient with rheumatoid arthritis who has Covid-19 with recurrent pericaditis debut, differential diagnosis.

Psoriasis induced by biological therapy.

OBJECTIVE: To describe a multicentre case series of new onset or worsening of psoriasis in patients treated with biological drugs. MATERIAL AND METHODS: Descriptive study. We reviewed the clinical history of patients with chronic inflammatory disease (CID) treated with biological drugs, who developed new onset or worsening of psoriasis during the follow-up period. RESULTS: Twenty-six cases of paradoxical psoriasis (PP) were recorded. Ninety-three percent of the patients were treated with anti-TNFα and adalimumab was responsible for 50% of the cases. Only 5 patients had a personal history of psoriasis. The biological drug was discontinued in 13 patients. Lesion recurrence was more frequent when another anti-TNFα was reintroduced. CONCLUSIONS: The PP is a reversible adverse effect that can be observed in patients exposed to biological drugs, mainly anti-TNFα.

Recurrent Pericarditis After Covid-19. / Pericarditis efusivo-constrictiva recidivante tras COVID-19.

Patient with rheumatoid arthritis who has Covid-19 with recurrent pericaditis debut, differential diagnosis.

Organisational and Clinical Approach to Osteoporosis in Rheumatology: OP-SER-Excellence Survey and Consensus. / Enfoque organizativo y clínico de la osteoporosis en reumatología: encuesta y consenso OP-SER-Excellence.

OBJECTIVE: To determine and analyse the organisational approach adopted by Spanish rheumatologists to osteoporosis (OP) to define strategic priorities. MATERIAL AND METHOD: A group of experts designed a questionnaire on OP in the rheumatologist practice. The survey was sent to the Spanish Society of Rheumatology (SER) members. Through the Delphi round, strategic priorities were agreed upon in OP. RESULTS: The priorities are: 1) The SER should promote the inclusion of OP in 100% of the services and expand the training offer; 2) Rheumatology services should promote the role of the nurse in OP, promote quality indicators and referral protocols agreed with primary care in addition to promoting their training in this area; 3) The SER and Rheumatology services should promote electronic consultation, OP monographic clinics and participation in Fracture Liaison Service units. CONCLUSIONS: Strategic priorities in OP help identify areas of improvement at organisational, structural and quality standards level in this pathology.

Prospective multicentre study of experience in real-world clinical practice in monitoring reported outcome measures (PROMs) of patient with a diagnosis of psoriatic and/or spondyloarthritis and initiating treatment with secukinumab. / Estudio prospectivo multicéntrico de experiencia en práctica clínica real en el control de medidas de desenlace reportadas por el paciente (PRO) diagnosticado de artritis psoriásica y/o espondiloartritis y que inicia tratamiento con secukinumab.

OBJECTIVE: To analyse the effect of secukinumab on self-reported variables of patients diagnosed with psoriatic arthritis and/or ankylosing spondylitis in relation to their health status, pain, fatigue, sleep and quality of life. METHODS: A six-month, observational, longitudinal, prospective, multicentre study was conducted with 39 patients who initiated treatment with secukinumab as therapy for psoriatic arthritis and/or spondylitis. The main variables were changes in patient-reported measures and they were evaluated by means of the questionnaires: FACIT-fatigue, Insomnia Severity Index, EuroQol-3L-5D and PsAQoL. In addition, depending on the type of disease (peripheral psoriasis or spondyloarthritis) the DAS28 with ESR or the BASDAI were calculated, respectively. RESULTS: Levels of fatigue, moderate and severe insomnia significantly reduced after 6months of treatment with secukinumab. At the same time, patient-reported quality of life increased significantly (P=.006). Data on pain and discomfort also show significant improvement after the treatment. CONCLUSIONS: Patients with psoriatic arthritis and/or ankylosing spondylitis who start treatment with secukinumab show improvement at 6months in all effect sizes of the treatment, particularly in sleep, fatigue and quality of life. Furthermore, patient-reported outcome measures are of additional clinical value and allow more accurate and closer assessment of their real status of health and well-being.

Psoriasis Induced by Biological Therapy. / Psoriasis inducida por terapia biológica.

OBJECTIVE: To describe a multicentre case series of new onset or worsening of psoriasis in patients treated with biological drugs. MATERIAL AND METHODS: Descriptive study. We reviewed the clinical history of patients with chronic inflammatory disease (CID) treated with biological drugs, who developed new onset or worsening of psoriasis during the follow-up period. RESULTS: Twenty-six cases of paradoxical psoriasis (PP) were recorded. Ninety-three percent of the patients were treated with anti-TNFα and adalimumab was responsible for 50% of the cases. Only 5 patients had a personal history of psoriasis. The biological drug was discontinued in 13 patients. Lesion recurrence was more frequent when another anti-TNFα was reintroduced. CONCLUSIONS: The PP is a reversible adverse effect that can be observed in patients exposed to biological drugs, mainly anti-TNFα.

Factors associated with long-term retention of treatment with golimumab in a real-world setting: an analysis of the Spanish BIOBADASER registry.

The retention rate of a biological drug (percentage of patients remaining on treatment over time) provides an index of a drug's overall effectiveness. The golimumab retention rate as first-line biological therapy was high in clinical trial extensions lasting 5 years. Real-world studies also indicate good retention rates but have been of shorter duration. The probability of retention with golimumab treatment was assessed, as any line of anti-tumor necrosis factor-alpha therapy, for up to 5 years in patients with rheumatoid arthritis (RA), axial spondyloarthritis (SpA) or psoriatic arthritis (PsA), associated factors were analyzed. A retrospective database analysis of the Spanish registry of patients with rheumatic disorders receiving biological drugs (BIOBADASER) was performed. Among 353 patients, 29.8% had RA, 41.6% SpA and 28.6% PsA. Golimumab was the first biological drug in 40.1% of patients, second in 30.1% and third/later in 29.8%. The overall probability of retention of golimumab at years 1, 2, 3, 4 and 5 was 85.9% (95% confidence interval 81.4-89.5%), 73.7% (67.1-79.1%), 68.5% (60.5-75.1%), 60.6% (50.2-69.5%) and 57.1% (44.9-67.5%), respectively. Retention was similar across indications (p = 0.070) but was greater when golimumab was used as the first biological agent compared with later therapy lines (p < 0.001). Factors associated with higher retention of golimumab treatment (Cox regression) were use as a first-line biological and concomitant methotrexate treatment; corticosteroid need was associated with lower retention. The long-term probability of golimumab retention was high in this real-world study of patients with rheumatic diseases, especially when used as the first biological drug.

Change in the discontinuation pattern of tumour necrosis factor antagonists in rheumatoid arthritis over 10 years: data from the Spanish registry BIOBADASER 2.0.

OBJECTIVE: To investigate in rheumatoid arthritis (RA) the rate and reason of discontinuation of tumour necrosis factor (TNF) antagonists over the past decade. METHODS: RA patients in BIOBADASER 2.0 were stratified according to the start date of their first TNF antagonist into 2000-3, 2004-6 and 2007-9 interval years. Cumulative incidence function of discontinuation for inefficacy or toxicity was estimated with the alternative reason as competing risk. Competing risks regression models were used to measure the association of study groups with covariates and reasons for discontinuation. Association is expressed as subhazard ratios (SHR). RESULTS: 2907 RA patients were included in the study. Competing risk regression for inefficacy shows larger SHR for patients starting treatment in 2004-6 (SHR 2.57; 95% CI 1.55 to 4.25) and 2007-9 (SHR 3.4; 95% CI 2.08 to 5.55) than for those starting in 2000-3, after adjusting for TNF antagonists, clinical activity and concomitant treatment. Competing risk regression analysis for adverse events revealed no differences across the three time intervals. CONCLUSIONS: In RA, the discontinuation rate of TNF antagonists in the first year of treatment is higher more recently than a decade ago, inefficacy being the main reason for the increased rate. The rate of discontinuation for adverse events has remained stable.

[Efficacy of adalimumab in Behçet's disease. Description of 6 cases]. / Eficacia del adalimumab en la enfermedad de Behçet: descripción de 6 casos.

Behçet's disease (BD) is a systemic vasculitis, with a more aggressive course in young males. Orogenital ulcers, uveitis and cutaneous lesions are the most frequent manifestations. We analyzed the effects of adalimumab on six patients with BD pretreated with inmunosupressive therapy, two of whom had received infliximab. We observed a good clinical response in all patients. To date, after a mean follow-up of 26.8 months, patients continue receiving adalimumab, with good clinical control, no adverse effects have been reported with adalimumab.