Reumatismo最新文献

Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by multisystem involvement. Patients can be stratified into an indolent or rapidly progressive disease course. A progressive course warrants early and more aggressive treatment to prevent irreversible organ damage. Therapeutic strategies should be tailored to the presenting symptoms and organ involvement. Autologous hematopoietic stem cell transplantation (AHSCT) has proven to be an effective treatment modality for specific phenotypes of SSc, especially progressive diffuse cutaneous SSc. However, the optimal timing for the transplantation remains unknown. We present two cases of rapidly progressive diffuse cutaneous SSc (dcSSc) treated with AHSCT following inadequate response to conventional immunosuppressive therapy. While both patients experienced significant cutaneous improvement post-AHSCT, internal organ involvement progressed in one case, ultimately resulting in a fatal outcome due to severe sepsis, whereas the second patient remained clinically stable and without immunosuppressive therapy during long-term follow-up. This report contributes to the growing body of evidence supporting AHSCT as a therapeutic option in carefully selected cases of progressive dcSSc. To our knowledge, our cases are the first successful experiences with this treatment modality in Croatia and among the Slavic populations of the Balkan Peninsula, promoting the need for earlier interventions in patients who develop a progressive disease course, particularly with skin involvement.

Sjögren's disease (SjD) was first described in a middle-aged female patient with chronic rheumatism in 1930. Membranous nephropathy (MN) is the most commonly identified type of glomerulonephritis in older adults with nephrotic syndrome. One of the autoimmune diseases that causes secondary MN is SjD. A 68-year-old female patient with a medical history of 25 years of hypertension, 9 years of SjD, depressive mood disorder, and intracoronary stent placement applied with peripheral edema. Hypoalbuminemia, hypothyroidism, hematuria, proteinuria, and albuminuria were also detected. In the autoantibody panel, antinuclear antibodies, anti-Ro-52 antibody, anti-Ro/SS-related antigen A antibody, and anticentromere antibody were positive. Kidney biopsy revealed MN. Anti-phospholipase A2 receptor antibody was negative. Methylprednisolone, cyclosporine, hydroxychloroquine, nifedipine, metoprolol, valsartan, L-thyroxine, acetylsalicylic acid, artificial tear drops, and fluoxetine were administered. Partial remission was detected in the first month of treatment. However, the patient, who had all vaccinations, developed swine flu infection and subsequently widespread candidiasis, and despite amphotericin B treatment and discontinuation of immunosuppressives, died in the fifth month due to septic shock. Anti-PLA2R antibody negative MN is one of the kidney manifestations of SjD. The poor prognosis of our patient was due to high SjD disease activity and severe infectious complications, which are independent risk factors for overall mortality.

Objective: To develop and test an algorithm with the aim of optimizing the implementation of ultrasound in the diagnostic workflow in clinical practice.

Methods: Through a consensus among the Musculoskeletal Ultrasound (MSUS) Study Group of the Italian Society for Rheumatology, we identified clinical and laboratory variables to be included in 1000minds surveys to develop an algorithm driving clinical diagnostic suspicion. The algorithm would identify potential differential diagnoses where MSUS protocols targeted for specific diseases (rheumatoid arthritis, psoriatic arthritis, gout, calcium pyrophosphate deposition disease, polymyalgia rheumatica, and osteoarthritis) could be applied. The joint sites and elementary lesions for each disease were selected based on a previously performed systematic literature review (SLR) and consensus. Finally, we conducted a pilot study on patients with new-onset arthritis to assess the performance of the algorithm, comparing the algorithm-based diagnosis with the final clinical diagnosis.

Results: Based on the consensus and the surveys, age, the number of involved joints, anti-citrullinated protein antibody, rheumatoid factor, C-reactive protein, and erythrocyte sedimentation rate were included in the algorithm. The pilot study included 59 patients: median (interquartile range) age 62.2 (54.1-72.6) years, 78% female. The agreement between the diagnosis selected by the algorithm and the final diagnosis by the rheumatologist was 88.1%. The elementary lesions and joint sites included in the different MSUS protocols were selected based on the best diagnostic accuracy, as shown by the SLR and defined by the working group.

Conclusions: The developed algorithm was accurate in identifying the correct diagnosis. Thus, it could reliably drive the decision on the MSUS assessment to perform. The RADIAL study will further investigate the feasibility and added value of MSUS in the diagnostic workflow according to this newly developed clinical suspicion-driven algorithm.

Objective: Drug survival rate and time are important to demonstrate the effectiveness of treatment in patients with axial spondyloarthritis (axSpA) in real life. Therefore, we aimed to evaluate drug survival rate and predictors of discontinuation of certolizumab and secukinumab in axSpA patients.

Methods: This single-center retrospective cohort study included patients treated with certolizumab (n=239) and secukinumab (n=64) among axSpA patients followed up at the rheumatology clinic. Clinical, laboratory, and imaging findings, treatment duration, and reasons for discontinuation were evaluated between April 2019 and December 2022. Drug survival rate and time were analyzed using Kaplan-Meier analysis, and predictive factors associated with drug discontinuation were analyzed using multivariable Cox regression analysis.

Results: At 12 months, drug retention rates were 76% in the secukinumab group and 73% in the certolizumab group. The overall retention rate was similar in both groups (p=0.641). The median survival time was 66.0 months in the secukinumab group vs. 62.8 months in the certolizumab group. A comparison of the patients who discontinued certolizumab treatment with those who continued showed that patients who discontinued certolizumab treatment had a higher frequency of female sex, peripheral arthritis, and inflammatory bowel disease. Comparison of the patients who discontinued secukinumab treatment with those who continued revealed that patients who discontinued secukinumab treatment only had a higher frequency of male sex. Multivariable Cox regression showed that male sex was independently associated with a lower risk of certolizumab discontinuation [hazard ratio (HR): 0.634, 95% confidence interval (CI): 0.41-0.97, p=0.036] and with a higher risk of secukinumab discontinuation (HR: 2.77, 95% CI: 1.18-6.49, p=0.018).

Conclusions: Our data showed that the drug survival rate of certolizumab and secukinumab was similar in patients with AxSpA. There was a lower risk of certolizumab discontinuation and a higher risk of secukinumab discontinuation in males.

Objective: Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases including dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASS). Treatment typically involves high-dose corticosteroids (CCS) and conventional synthetic disease-modifying antirheumatic drugs (csDMARD). Rituximab (RTX) has shown effectiveness in refractory cases. Our real-life study aimed to assess the safety and effectiveness of RTX treatment in IIM patients.

Methods: We conducted a retrospective study including patients with IIM refractory to both high-dose CCS and csDMARD. Patients were treated with a full RTX dose (2 g every 6 months). Laboratory and clinical data, along with the total improvement score (TIS), were assessed to evaluate RTX effectiveness and safety. Data were analyzed using GraphPad Prism (v. 9.5.1).

Results: A total of 41 patients received the full RTX dose (15 DM, 15 ASS, 5 PM, and 6 IMNM). This treatment regimen significantly reduced daily CCS usage from 15 mg [interquartile range (IQR) 12.5-25 mg] at baseline to 5 mg (IQR 5-5 mg) after 1 year of treatment (p<0.001). Additionally, over 90% of patients achieved at least a minimal TIS at 12 months, which was maintained at 24 months. At 1 year, RTX persistence was 68.3%. Although reductions in serum immunoglobulins (Ig)A and IgM levels were observed, no cases of severe hypogammaglobulinemia (IgG<400 mg/dL) occurred. The most common reason for treatment interruption was an adverse skin reaction (6 cases) during RTX infusion, while infections most frequently involved the respiratory tract (5 cases).

Conclusions: RTX demonstrated effectiveness in various subsets of IIMs, often leading to clinical improvement and significantly reducing the CCS dose.

Objective: We aimed to describe the frequency of intimate partner violence (IPV) in reproductive-age women and pregnant-postpartum women with autoimmune rheumatic diseases (ARDs) and compare it with those without ARDs (controls).

Methods: A descriptive, cross-sectional, and comparative study was conducted among pregnant-postpartum patients and reproductive-age women (18-45 years) with and without ARDs who attended the Hospital Universitario in Monterrey, Mexico, and answered the survey Hurt-Insult-Threaten-Scream (HITS) scale in the validated Spanish version, from June 2023 to May 2024.

Results: A total of 120 women were included: 60 with ARDs and 60 controls. In both groups, 30 patients were reproductive-age women and 30 were pregnant-postpartum women. A total of 44 (36%) women reported being victims of IPV. No significant differences were found in reported IPV between the control group and the group of women with ARDs (n=21, 35% vs. n=23, 38%, p=0.85). There was no statistically significant difference between the ARD group compared to the control group in the HITS score (p=0.537), nor between the pregnant-postpartum subgroups (p=0.356) or the reproductive-age subgroups (p=0.972). These findings indicate that IPV rates did not significantly differ by ARD status or reproductive stage in this sample.

Conclusions: Nearly one in every three women experienced IPV, but our research showed that there was no difference in the frequency of IPV between the ARD group and the control group. Pregnant and postpartum women were more likely to report IPV than women of reproductive age. These findings highlight that IPV is a significant concern for all women in Mexico and the need for increased attention and support for them.

Rheumatoid arthritis (RA) is rarely reported among patients with sickle cell disease (SCD). RA treatment in these patients is believed to be more challenging due to the fear of increasing the risk of infection and complications of SCD. We are reporting 7 patients with concurrent SCD and RA. The average age at the time of the diagnosis of RA was 33.3±12.6 years (ranging from 16 to 53 years), and most were women (5/7). Most of the patients were positive for rheumatoid factor (6/7) or anticyclic citrullinated peptide (6/7). Four patients were treated with hydroxyurea. The most used antirheumatic drugs were methotrexate (7/7), biologic agents (5/7), and prednisone (4/7). Two patients were in remission, four had low and one had high disease activity. Four patients (4/7) had avascular necrosis, two in the shoulders and two in the hip joints. Four patients had emergency visits or hospitalizations within one year of the diagnosis of RA, but none had blood transfusions, infections, or death. The start of antirheumatic medications was not associated with an increased risk of infection, blood transfusions, emergency visits, or hospitalizations, nor with a worsening of laboratory measures. The findings suggest that the treatment of RA in patients with SCD should follow the same strategy as in patients without SCD. However, treatment should be individualized according to the individual patient's risk of infection and SCD complications.

Patients with dermatomyositis (DM) are particularly susceptible to opportunistic infections due to immunosuppression induced by the disease itself and its treatment. We describe three patients who met the diagnostic criteria for DM and developed tuberculous myositis. The first case, a 54-year-old woman, had a positive polymerase chain reaction (PCR) for Mycobacterium tuberculosis detected in a post-mortem muscle biopsy. A second patient was diagnosed with a positive Ziehl-Neelsen stain in bronchoalveolar lavage, and a third patient, with multiple collections in the thorax and lower limbs, had positive Ziehl-Neelsen stain and PCR for Mycobacterium tuberculosis. In inflammatory myopathies, muscle and soft tissue infection by tuberculosis may produce symptoms similar to the underlying disease. The differential diagnosis of tuberculosis superinfection can be difficult.

Dear Editor, The number of cases of cancer patients diagnosed as having immune checkpoint inhibitor-mediated polymyalgia rheumatica is increasing...

Objective: Idiopathic inflammatory myopathies (IIM) are rare autoimmune diseases that primarily affect striated muscles; skin, joints, and lungs may be involved with different degrees of severity. Traditional treatment relies on high-dose glucocorticoids and conventional synthetic disease-modifying antirheumatic drugs.

Methods: A growing amount of evidence is demonstrating the potential role of novel treatments in the management of IIM. We report our experience with Janus kinase inhibitors (JAKi) in these conditions and review the current evidence for the use of small molecules in real-life clinical practice.

Results: A total of 41 papers were retrieved from PubMed, 37 papers concerning IIM and JAKi, and 4 papers concerning IIM and apremilast.

Conclusions: An overall good efficacy was evidenced in IIM-associated skin lesions, including rash, ulcers, and calcinosis. If present, muscle and joint involvement demonstrated a good response to therapy, while it was not possible to draw any conclusion about dysphagia. No life-threatening adverse events were reported.