Reumatologia最新文献

Introduction: Amyloidosis is a heterogeneous group of conditions associated with tissue deposition of insoluble abnormal proteins that damage vital organs. Early diagnosis, when the deposits are minimal, determines the prognosis and requires histological confirmation. The commonly adopted gold standard technique is alkaline Congo red (ACR) staining, though its sensitivity is limited. There is a need for a simple and inexpensive screening method offering a better chance of detecting minimal amyloid deposits. The aim of this study was to compare amyloid detectability with ACR and phenol Congo red (PHCR) staining techniques for early detection of minimal deposits.

Material and methods: We assessed 452 tissue specimens (including adipose tissue, gastrointestinal mucosa, labial salivary gland, myocardium, and bone marrow) from 425 patients with clinically suspected systemic or local amyloidosis, which had been sent to the Pathology Laboratory of the National Institute of Geriatrics, Rheumatology and Rehabilitation in Warsaw. Adjacent sections from each specimen were stained with ACR and PHCR. If amyloid was detected, immunohistochemical typing was conducted. The consistency of the two staining methods was expressed as Cohen's κ coefficient.

Results: A total of 169 tissue specimens (37%) yielded positive readings, with 93 cases ACR(+) and PHCR(+); 75 cases ACR(-) and PHCR(+), and 1 case ACR(+) and PHCR(-). The percentage agreement between the staining methods was 83%, with the Cohen's κ coefficient value of 0.60 (95% CI: 0.52-0.69), which corresponds to moderate agreement according to Fleiss. Additional immunohistochemical amyloid typing, conducted in ACR(-) and PHCR(+) specimens, yielded conclusive results in 82% of cases.

Conclusions: The use of PHCR staining as a screening method in suspected amyloidosis improves amyloid (light chain, transthyretin, and amyloid A protein) detectability in various tissues. The PHCR staining specificity should be verified via electron microscopy and/or mass spectrometry.

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic granulomatous vasculitis. Typical symptoms include late-onset bronchial asthma and blood and tissue eosinophilia. In addition to these characteristic symptoms, EGPA can affect important organs such as the skin, kidneys, heart, sinuses, gastrointestinal tract, and nervous system. Given the variability of the clinical presentation, EGPA is challenging to diagnose. Furthermore, EGPA often occurs in phases, with clinical manifestations and pathological findings varying depending on the affected anatomic site and stage of disease.

Material and methods: The authors reviewed the SCOPUS, MEDLINE, and PubMed medical databases to prepare an overview of the clinical manifestations and diagnosis for EGPA.

Results: This comprehensive review examines the current knowledge on the clinical course of EGPA, diagnostic options and prognostic factors.

Conclusions: We highlight the diverse organ involvement observed in EGPA, particularly in association with eosinophilic and vasculitic manifestations. Our findings underscore the importance of anti-neutrophil cytoplasm antibody status as a potential key factor influencing disease presentation.

Nutritional disorders are significant but often underestimated complications in patients with systemic sclerosis (SSc). The most prevalent nutritional disorders in SSc are malnutrition, affecting up to 62.5% of patients, and sarcopenia, with a frequency of up to 42%. Thus, clinical vigilance is recommended for the detection of eating disorders in SSc patients, particularly those with gastrointestinal involvement, cardiopulmonary complications, an advanced disease stage, and high disease activity. Nutritional treatment should be carefully tailored to the patients' clinical condition to ensure that it effectively addresses their specific needs. Studies focusing on enteral nutrition in SSc patients demonstrate its effectiveness in stabilizing or improving nutritional status in malnourished patients. In severe cases, parenteral nutrition offers viable options to support patient health. The findings highlight the importance of early nutritional assessment and intervention in improving patient outcomes and suggest that individualized nutritional therapy can be a critical component of comprehensive care for SSc patients.

Introduction: Psoriatic arthritis (PsA) is a heterogeneous disease with various manifestations such as dactylitis, enthesitis, spondylitis, and skin involvement. Minimal disease activity (MDA) has been successfully used in daily clinical practice and is considered a reasonable treatment target in patients with PsA. In this study, we aimed to evaluate the MDA status and associated factors in patients with PsA in our tertiary referral clinic.

Material and methods: This cross-sectional study included patients who met the CASPAR classification criteria and had at least 6 months of follow-up data between 2001 and 2021. Patients who met at least 5 of 7 criteria (tender joint count ≤ 1/68, swollen joint count ≤ 1/66, Psoriasis Area Severity Index [PASI] ≤ 1, Visual Analogue Scale [VAS] ≤ 15, patient global VAS ≤ 20, Health Assessment Questionnaire-Disability Index [HAQ-DI] ≤ 0.5, and enthesitis number ≤ 1) were considered to achieve MDA.

Results: Data from 172 patients (61% female) were analyzed and included in the study. While most patients had polyarticular involvement (33.7%), mono-oligoarthritis was present in 30.2%, mixed type in 26.2%, isolated distal interphalangeal arthritis in 5.8%, isolated spondylitis in 2.9%, and arthritis mutilans in 1.2%. Overall, 95 (55.2%) of the patients were observed at MDA, which was lower in tumor necrosis factor inhibitor (TNFi) users compared to only conventional synthetic disease-modifying antirheumatic drug users. In univariate analysis, MDA was associated with higher patient age, longer psoriasis duration, late-onset PsA, and continued use of first TNFi. In multivariate analysis, higher patient age, late-onset PsA, and higher continuation rate of first TNFi were associated with MDA.

Conclusions: In the study, more than half of our patients achieved MDA status. A higher MDA rate was associated with a higher continuation rate at first-line TNFi treatment. The relatively large population who could not reach MDA status in our study indicates an unmet need for monitoring and treatment of PsA.

Introduction: The objective of this cross-sectional study was to evaluate the monocyte to albumin ratio (MAR), neutrophil to albumin ratio (NAR), platelet to albumin ratio (PAR), and C-reactive protein to albumin ratio (CAR) as potential biomarkers for disease activity in patients with Behçet's disease (BD).

Material and methods: Both BD cases and healthy controls were enrolled in this study. Demographic characteristics, disease duration, and current medications were recorded for all participants. The BD Current Activity Form (BDCAF) was utilized to assess the activity of BD. Additionally, erythrocyte sedimentation rate, CRP, and serum albumin levels were measured. The MAR, NAR, PAR, and CAR were compared between the two groups. Correlation analysis and receiver operating characteristic curves (ROC) were employed to establish cut-off points for these biomarkers.

Results: In the study, both BD cases and 45 controls were included, totaling 90 participants. Significant differences were observed in the mean ±SD values of ESR, MAR, PAR, CAR, and albumin between the BD cases and controls (p = 0.008, p = 0.009, p = 0.029, p = 0.034, p = 0.006, respectively). However, despite these differences, no significant correlation was detected between BDCAF and the parameters under investigation. The cut-off point was determined as 150.59 (sensitivity 46.67%, specificity 82.22%, p = 0.008, AUC = 0.655) for MAR; as 62,013.73 (sensitivity 60.00%, specificity 66.67%, p = 0.03, AUC = 0.629) for PAR; and as 1.16 (sensitivity 35.56%, specificity of 95.567%, p = 0.03, AUC = 0.629) for CAR. The results were not able to define any cut-off points for active-inactive BD.

Conclusions: Significantly higher levels of MAR, PAR, and CAR were observed in patients with BD than controls. Monocyte to albumin ratio, PAR, and CAR were notably elevated in patients with active BD. This finding suggests that these parameters possess discriminative ability and could potentially serve as biomarkers to aid in the clinical evaluation of BD.

Introduction: Rheumatoid arthritis (RA) is a chronic autoimmune disorder that affects the joints, causing inflammation, pain, and potential joint damage. Patients with RA are at high risk of developing psychiatric morbidity; it is important to recognize these psychiatric manifestations. The relationship between psychiatric symptoms and RA is complex and can involve various factors, including the impact of chronic pain, inflammation, medications, and the overall burden of managing a chronic illness.Aim of the study was to systematically investigate and analyze the patterns and prevalence of psychiatric morbidity among individuals diagnosed with RA, with the aim of identifying common mental health conditions, understanding the interplay between RA and psychiatric disorders, and providing valuable insights for improved holistic patient care.

Material and methods: This was a prospective, observational cross-sectional study conducted over a period of three years in patients with RA. Psychiatric morbidity was assessed using International Classification of Diseases-10 criteria and Mini-Plus by dedicated psychiatrists. The diagnosis of RA was confirmed using the 2010 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) diagnostic criteria for RA and the disease activity was calculated by Disease Activity Score with 28-joint count (DAS28) using the calculator from the RheumaHelper application. The data were analyzed using SPSS, version 23.0.

Results: A total of 1,000 patients with RA were included in this study. Nearly two-thirds of the patients were female (64.8%). The majority of patients belonged to the age group of 41 to 54 years. Total 47.5% of the patients were unemployed, 27.0% were salaried, 19.0% were businessman, while 6.5% of the patients were students. More than half of the patients (53.2%) had moderate disease activity. Major depressive disorder was the most commonly observed comorbidity (41.0%), followed by somatoform disorder (28.5%), and generalized anxiety disorder was found in 13.5%. No psychiatric manifestations were found in 17% of studied individuals.

Conclusions: Psychiatric morbidity is associated with RA and there is a need for psychiatric services to be made available to these patients.

Introduction: This study compared treatment with biologic agents and Janus kinase inhibitors (JAKi) in combination with methotrexate (MTX) for rheumatoid arthritis (RA) in a real-world setting at a large center in Poland. There is a persistent shortage of such studies, and illustrating the switching of medications in search of a suitable way of treatment for a given patient is a crucial step towards future personalized therapy.

Aim of the study: This study is an extension of the initial work published in 2022 in Reumatologia, with the addition of an analysis of patients treated with upadacitinib. The study compared the effectiveness and side effects after treatment of biological disease modifying antirheumatic drugs (bDMARDs) and targeted synthetic DMARDs (tsDMARDs) in combination with MTX.

Materials and methods: A total of 130 patients with active severe RA (Disease Activity Score for 28 joints based on the erythrocyte sedimentation rate [DAS28(ESR)] value > 5.1) were treated at the Rheumatologic Outpatients Department of the Central Clinical Hospital of the Ministry of the Interior and Administration, Warsaw, Poland between January 2010 and September 2021. All patients were treated with MTX 25 mg per week. They were divided into two groups: group I (80 patients) treated with biologic agents, and group II (50 patients) treated with JAKi. Assessment of DAS28(ESR) and Simplified Disease Activity Index (SDAI) and analysis of Boolean criteria for remission were performed. Remission or low disease activity, switching between drugs and adverse events were assessed and compared between studied groups.

Results: Patients treated with tsDMARDs had previously used a higher number of conventional synthetic DMARDs (csDMARDs) and bDMARDs compared to those treated with bDMARDs. However, they achieved lower SDAI and assessment of disease activity using Visual Analogue Scale (VAS) values, and a higher proportion of patients achieved Boolean criteria for remission after treatment.

Conclusions: The results of treatment with JAKi were successful, but the potential side effects indicate that this treatment may not be equally suitable for all RA patients.

Introduction: Capillaroscopy is a simple method of nailfold capillary imaging, used to diagnose diseases from the systemic sclerosis spectrum. However, the assessment of the capillary image is time-consuming and subjective. This makes it difficult to use for a detailed comparison of studies assessed by various physicians. This pilot study aimed to validate software used for automatic capillary counting and image classification as normal or pathological.

Material and methods: The study was based on the assessment of 200 capillaroscopic images obtained from patients suffering from systemic sclerosis or scleroderma spectrum diseases and healthy people. Dinolite MEDL4N Pro was used to perform capillaroscopy. Each image was analysed manually and described using working software. The neural network was trained using the fast.ai library (based on PyTorch). The ResNet-34 deep residual neural network was chosen; 10-fold cross-validation with the validation and test set was performed, using the Darknet-YoloV3 state of the art neural network in a GPU-optimized (P5000 GPU) environment. For the calculation of 1 mm capillaries, an additional detection mechanism was designed.

Results: The results obtained under neural network training were compared to the results obtained in manual analysis. The sensitivity of the automatic tool relative to manual assessment in classification of correct vs. pathological images was 89.0%, specificity 89.4% for the training group, in validation 89.0% and 86.9% respectively. For the average number of capillaries in 1 mm the precision of real images detected within the region of interest was 96.48%.

Conclusions: The pilot software for fully automatic capillaroscopic image assessment can be a useful tool for the rapid classification of a normal and altered capillaroscopy pattern. In addition, it allows one to quickly calculate the number of capillaries. In the future, the tool will be developed and will make it possible to obtain full imaging characteristics independent of the experience of the examiner.

Introduction: Systemic lupus erythematosus (SLE) and sickle cell disease (SCD) are distinct multisystemic diseases that commonly affect blacks. There are few reports of their co-existence in Western literature and a paucity of reports in Sub-Saharan Africa. Their co-existence is associated with diagnostic delay and treatment dilemmas. The aim is to describe the clinical, laboratory, and treatment profile of Nigerian lupus with sickle cell disease.

Material and methods: A 7-year retrospective descriptive study of lupus patients with sickle cell disease was performed. Medical records of eligible patients were extracted into a proforma, transferred into SPSS, and analyzed with descriptive statistics. Sociodemographic, clinical, laboratory, and treatment data were presented as frequency and percentages.

Results: Twelve SLE-SCD cases (female 11, male 1) were identified. The mean age was 28.5 years and the mean duration of illness prior to diagnosis was 9.5 years. The median follow-up period was 3.1 years and the common presentations were mucocutaneous (66%), renal, (50%) serositis (33%), and neurological (16%) in decreasing order. All had anemia and positive antinuclear antibody, 33% had pancytopenia and 75% had positive anti-dsDNA and anti-Smith. Two are on maintenance hemodialysis, one with interstitial lung disease, and one on long-term anticoagulation due to deep vein thrombosis.

Conclusions: Sickle cell disease and SLE should be considered in SCD with atypical clinical and laboratory features. We hope this report will raise diagnostic suspicion and prompt early diagnosis and treatment to prevent multiorgan damage that may ensue from such an association.