Effectiveness of janus kinase inhibitors in relapsing giant cell arteritis in real-world clinical practice and review of the literature.

BACKGROUND: A substantial proportion of patients with giant cell arteritis (GCA) relapse despite standard therapy with glucocorticoids, methotrexate and tocilizumab. The Janus kinase/signal transducer and activator of transcription (JAK/STAT) signalling pathway is involved in the pathogenesis of GCA and JAK inhibitors (JAKi) could be a therapeutic alternative. We evaluated the effectiveness of JAKi in relapsing GCA patients in a real-world setting and reviewed available literature. METHODS: Retrospective analysis of GCA patients treated with JAKi for relapsing disease at thirteen centers in Spain and one center in United States (01/2017-12/2022). Outcomes assessed included clinical remission, complete remission and safety. Clinical remission was defined as the absence of GCA signs and symptoms regardless of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values. Complete remission was defined as the absence of GCA signs and symptoms along with normal ESR and CRP values. A systematic literature search for other JAKi-treated GCA cases was conducted. RESULTS: Thirty-five patients (86% females, mean age 72.3) with relapsing GCA received JAKi therapy (baricitinib, n = 15; tofacitinib, n = 10; upadacitinib, n = 10). Before JAKi therapy, 22 (63%) patients had received conventional synthetic immunosuppressants (e.g., methotrexate), and 30 (86%) biologics (e.g., tocilizumab). After a median (IQR) follow-up of 11 (6-15.5) months, 20 (57%) patients achieved and maintained clinical remission, 16 (46%) patients achieved and maintained complete remission, and 15 (43%) patients discontinued the initial JAKi due to relapse (n = 11 [31%]) or serious adverse events (n = 4 [11%]). A literature search identified another 36 JAKi-treated GCA cases with clinical improvement reported for the majority of them. CONCLUSIONS: This real-world analysis and literature review suggest that JAKi could be effective in GCA, including in patients failing established glucocorticoid-sparing therapies such as tocilizumab and methotrexate. A phase III randomized controlled trial of upadacitinib is currently ongoing (ClinicalTrials.gov ID NCT03725202).

Satisfaction and effectiveness of switching from intravenous to subcutaneous belimumab treatment in daily clinical practice.

BACKGROUND: In 2017, belimumab (BEL) was approved in subcutaneous (SQ) administration. The effectiveness after switching from intravenous (IV) to SQ and patient satisfaction in daily clinical practice has not been studied. During the pandemic, patient follow-up and treatment were significantly affected, and some patients need a change from IV to SQ. Our aim was to evaluate daily clinical practice satisfaction to SQ BEL therapy in patients previously treated IV BEL. We hypothesized that SQ BEL in SLE patients previously treated with IV BEL was similar in effectiveness and conferred higher satisfaction. METHODS: Observational, multicenter study, conducted in 7 reference centers in Catalonia. We included stable SLE patients (EULAR/ACR 2019) on treatment with SQ BEL and previous use of IV BEL (at least 3 months on IV BEL before switching). Since there are no well-validated tools for SQ BEL treatment satisfaction, we used RASQ-SQ, validated in patients with lymphoma who switched from IV Rituximab to SQ treatment, and modified for BEL treatment. RESULTS: Twenty-seven patients were included. The more prevalent clinical manifestations observed were related to the skin and joints and the patients had a mean baseline SLEDAI of 2.96 (SD 2.4) and SLICC score of 0.67 (SD 0.88). The median time from treatment with IV BEL before switching to SQ was 21 months (range). 84% of patients reported confidence in SQ BEL. 85.2% felt that treatment with SQ BEL was convenient or very convenient. 85% felt they had gained time with the change. 89% would recommend the SQ injection to other patients. Disease activity (mean SLEDAI) and remission rates remain stable after switching. No major new adverse effects were reported. CONCLUSIONS: Overall satisfaction, satisfaction with via of administration, and satisfaction with the time taken to receive BEL were higher for SQ BEL treatment. A switching SQ strategy is a reasonable alternative for BEL patients.

Usefulness of cardiac screening in patients with systemic lupus erythematosus and anti-Ro/SSA antibodies.

OBJECTIVES: Recent data suggest that some adult patients with autoimmune rheumatic diseases may develop cardiac conduction and repolarization abnormalities mediated by anti-Ro/SSA antibodies. We aim to investigate the utility of a cardiac screening in patients with systemic lupus erythematous (SLE) and anti-Ro/SSA positivity. METHODS: SLE patients who consecutively attended a Rheumatology clinic during 1 year where evaluated for the presence and levels of anti-Ro/SSA antibodies, and clinical and biological markers of organ damage and disease activity. All participants underwent a cardiovascular anamnesis and physical examination, ECG, echocardiography, and 24-hour Holter. RESULTS: Of the 145 recruited patients, 49 (32%) had anti-Ro/SSA positivity. None had any degree of atrioventricular block in the ECG or Holter monitoring. No significant differences were observed between anti-Ro/SSA-positive vs. negative patients in terms of PR, QRS or QTc intervals. No clinically significant arrhythmias were recorded during Holter monitoring and no differences in average heart rate, heart rate variability, or atrial or ventricular ectopy burden were observed. Finally, no differences were found in echocardiographic measurements. CONCLUSIONS: In this study of SLE patients, anti-Ro/SSA positivity was not associated with significant alterations in ECG, echocardiography, or 24-hour Holter. These findings do not support ordinary cardiac evaluation in these patients. (Clinicaltrials.gov registration number: NCT02162992).

Late-onset versus early-onset systemic lupus: characteristics and outcome in a national multicentre register (RELESSER).

OBJECTIVE: The aim of the present study was to describe the demographic, clinical and immunological characteristics of patients with late-onset (≥50 years) SLE vs patients with early-onset SLE (<50 years). METHODS: We performed a cross-sectional retrospective study of 3619 patients from the RELESSER database (National Register of Patients with Systemic Lupus Erythematosus of the Spanish Society of Rheumatology). RESULTS: A total of 565 patients (15.6%) were classified as late-onset SLE and 3054 (84.4%) as early-onset SLE. The male-to-female ratio was 5:1. Mean (s.d.) age at diagnosis in the late-onset group was 57.4 (10.4) years. At diagnosis, patients with late-onset SLE had more comorbid conditions than patients with early-onset SLE; the most frequent was cardiovascular disease (P <0.005). Furthermore, diagnostic delay was longer in patients with late-onset SLE [45.3 (3.1) vs 28.1 (1.0); P <0.001]. Almost all patients with late-onset SLE (98.7%) were Caucasian. Compared with early-onset SLE and after adjustment for time since diagnosis, patients with late-onset SLE more frequently had serositis, major depression, thrombotic events, cardiac involvement and positive lupus anticoagulant values. They were also less frequently prescribed immunosuppressive agents. Mortality was greater in late-onset SLE (14.3% vs 4.7%; P <0.001). CONCLUSION: Late-onset SLE is insidious, with unusual clinical manifestations that can lead to diagnostic errors. Clinical course is generally indolent. Compared with early-onset disease, activity is generally reduced and immunosuppressants are less commonly used. Long-term prospective studies are necessary to determine whether the causes of death are associated with clinical course or with age-associated comorbidities in this population.

Espondilodiscitis sin endocarditis causada por Streptococcus mitis / Spondylodiscitis without endocarditis caused by Streptoccocus mitis

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Enfermedad de Behçet pseudotumoral / Pseudotumoral Behçet's disease

La enfermedad de Behçet es una vasculitis caracterizada por úlceras bucales y genitales. La afectación neurológica o neuro-Behçet es una manifestación infrecuente, de predominio en el género masculino y que aparece de 2 a 4 años después de la primera manifestación clínica. El neuro-Behçet cursa ocasionalmente lesiones cerebrales pseudotumorales. Presentamos 2 casos de pacientes diagnosticados de neuro-Behçet tras la detección de lesiones cerebrales pseudotumorales y se realiza una revisión de la literatura (AU)

Behçet‘s disease is a systemic vasculitis characterized by the presence of oral and genital ulcers. Neurological involvement or neuro-Behçet is an uncommon manifestation. It manifestation has predominance in the male gender appearing 2 to 4 years after the first clinical manifestation. However, neuro-Behçet disease sometimes occurs with pseudotumoral brain lesions. Herein, we present the cases of two patients diagnosed with neuro-Behçet after detection of pseudotumoral brain lesions. A review of the literature is performed (AU)

Quistes pulmonares asociados a síndrome de Sjögren primario / Pulmonary cysts associated with primary Sjögren‘s syndrome

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Pseudotumoral Behçet's disease.

Behçet's disease is a systemic vasculitis characterized by the presence of oral and genital ulcers. Neurological involvement or neuro-Behçet is an uncommon manifestation. It manifestation has predominance in the male gender appearing 2 to 4 years after the first clinical manifestation. However, neuro-Behçet disease sometimes occurs with pseudotumoral brain lesions. Herein, we present the cases of two patients diagnosed with neuro-Behçet after detection of pseudotumoral brain lesions. A review of the literature is performed.

Tratamiento con rituximab e inmunoglobulinas en la hemorragia alveolar difusa recurrente en lupus eritematoso sistémico / Recurrent diffuse alveolar hemorrhage in systemic lupus erytematosus treated with rituximab and immunoglobulins

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Polimialgia reumática con derrame pleuropericárdico: una asociación infrecuente / Rheumatic polymyalgia with pleuropericardial effusion: an uncommon association

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¿Cuál es la evolución de las artritis indiferenciadas? / What is the outcome of undifferentiated arthritis?

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Artritis séptica de la articulación acromioclavicular: una localización atípica / Septic arthritis of the acromioclavicular joint: an uncommon location

La artritis séptica de la articulación acromioclavicular (ACV) es una entidad poco frecuente que se presenta en pacientes inmunosuprimidos o con discontinuidad de las barreras de defensa. En la literatura únicamente se han descrito 15 casos hasta la fecha. El diagnóstico se basa en la presencia de clínica compatible junto al aislamiento del germen en la articulación o en los hemocultivos. Las técnicas de imagen (resonancia magnética, ecografía o gammagrafía) pueden ser útiles en la localización del proceso. El tratamiento antibiótico es el mismo que en la artritis séptica de otra localización y Staphylococcus aureus es también el microorganismo aislado con más frecuencia. Se describen las características clínicas, el tratamiento y la evolución de los pacientes diagnosticados de artritis séptica de la articulación ACV en un servicio de Reumatología, mediante estudio retrospectivo de revisión de historias clínicas de los pacientes atendidos por artritis séptica en dicha unidad (101 pacientes) en el periodo de 1989-2012. Seis enfermos (6%) tuvieron infección en la articulación ACV con confirmación microbiológica (AU)

Septic pyogenic arthritis of the acromioclavicular joint is a rare entity that occurs in immunosuppressed patients or those with discontinuity of defense barriers. There are only 15 cases described in the literature. The diagnosis is based on clinical features and the isolation of a microorganism in synovial fluid or blood cultures. The evidence of arthritis by imaging (MRI, ultrasound or scintigraphy) may be useful. Antibiotic treatment is the same as in septic arthritis in other locations. Staphylococcus aureus is the microorganism most frequently isolated. Our objective was to describe the clinical features, treatment and outcome of patients diagnosed with septic arthritis of the acromioclavicular joint at a Rheumatology Department. We developed a study with a retrospective design (1989-2012). The medical records of patients with septic arthritis were reviewed (101 patients). Those involving the acromioclavicular joint were selected (6 patients; 6%) (AU)

Septic arthritis of the acromioclavicular joint: an uncommon location.

Septic pyogenic arthritis of the acromioclavicular joint is a rare entity that occurs in immunosuppressed patients or those with discontinuity of defense barriers. There are only 15 cases described in the literature. The diagnosis is based on clinical features and the isolation of a microorganism in synovial fluid or blood cultures. The evidence of arthritis by imaging (MRI, ultrasound or scintigraphy) may be useful. Antibiotic treatment is the same as in septic arthritis in other locations. Staphylococcus aureus is the microorganism most frequently isolated. Our objective was to describe the clinical features, treatment and outcome of patients diagnosed with septic arthritis of the acromioclavicular joint at a Rheumatology Department. We developed a study with a retrospective design (1989-2012). The medical records of patients with septic arthritis were reviewed (101 patients). Those involving the acromioclavicular joint were selected (6 patients; 6%).