Archives of rheumatology最新文献

Objectives: This study aimed to analyze the differences of etiologies and clinical features between patients with autoimmune-associated hemophagocytic syndrome (AAHS) and those with other underlying diseases of hemophagocytic syndrome (HPS).

Patients and methods: The retrospective study was performed with 130 HPS patients (70 males, 60 females; mean age: 50.4±18.1 years; range, 13 to 85 years) between January 1^st, 2011, and April 1^st, 2022. The patients fulfilled at least five of the eight criteria proposed by the Histiocytosis Society in 2004. The underlying diseases related to HPS were divided into four categories: autoimmune, infection, malignancy and idiopathic diseases. And the clinical manifestations, laboratory examinations, treatments, and prognosis were analyzed respectively.

Results: Nineteen (14.6%) patients had AAHS, 45 (34.6%) had infection-associated HPS, 57 (43.8%) had malignancy-associated HPS, and nine (6.9%) had idiopathic HPS. The most common symptoms of HPS were unremitting fever in 123 (94.6%) of 130 patients and splenomegaly in 92 (70.8%). All patients manifested a decline of at least two lineages of hematopoietic cells. The absolute values of T cells and B cells of AAHS were significantly higher than that of malignancy-associated HPS. The levels of soluble CD25 (interleukin-2 receptor) of AAHS were the lowest among all-cause HPS (p<0.05). The all-cause mortality rate of hospitalized patients with HPS was 46.2%. The patients with AAHS had a better prognosis compared to other etiologies (odds ratio [OR]=0.091, 95% confidence interval [CI]: 0.011-0.775, p=0.028). Epstein-Barr virus infection (OR=4.761, 95% CI: 1.619-14.004, p=0.005) and pulmonary involvement (OR=4.555 95% CI: 1.524-13.609, p=0.007) were independent predictors of poor outcome in HPS. Thrombocytopenia (OR=0.978, 95% CI: 0.968-0.999, p=0.040) had a boundary effect on prognosis.

Conclusion: Patients with HPS secondary to autoimmune disease have better outcomes compared to patients complicated with Epstein-Barr virus infection or pulmonary involvement.

Objectives: This study aimed to determine if the presence of a pericardial effusion is associated with adverse outcomes among children admitted with juvenile idiopathic arthritis.

Patients and methods: The multicenter, retrospective cohort study was conducted with 4,332 patients (1,554 males, 2,778 females; median age: 12 years; IQR, 7, 15 years) using the Pediatric Health Information System. Data from hospital admissions between January 1, 2004, and September 15, 2015, were obtained for patients with an International Disease Classification, Ninth Revision code for juvenile idiopathic arthritis. Pericardial effusion was the primary predictor variable; the outcomes of interest were length of stay, hospital costs, and readmission within 90 days. Multivariate models were created to evaluate associations between pericardial effusion and adverse outcomes. We also analyzed factors associated with increased odds of having pericardial effusion in juvenile idiopathic arthritis.

Results: One hundred twenty (3%) patients had a code for pericardial effusion. Children with pericardial effusion had a longer median length of stay (7 days (IQR 3, 12) vs. 3 days (IQR 2,6), p<0.001), higher median costs ($17,688 (IQR 8,657, 40,623) vs. $8,456 (IQR 4,865, 16,302), p<0.001), and greater rates of readmission (22% vs. 15%, p=0.045). Multivariate analysis demonstrated no significant association between pericardial effusion and outcomes of interest. Black race and male sex were associated with increased odds of having pericardial effusion.

Conclusion: Pericardial effusion is rare among children admitted with juvenile idiopathic arthritis but is associated with significant morbidity; its presence may be a marker of disease severity. Black children and males admitted with juvenile idiopathic arthritis warrant special consideration and may benefit from screening echocardiography.

Objectives: This study aimed to analyze a group of patients with severe and refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) managed with rituximab and to report on treatment outcomes.

Patients and methods: A total of 78 patients (41 females, 37 males; mean age: 50.1±13.4 years; range, 18 to 76 years) with AAV on rituximab treatment were included in the single-center, retrospective study conducted between 2009 and 2018. The diagnosis was established based on the 1990 classification criteria of the American College of Rheumatology and the definitions of vasculitis of Chapel Hill Consensus Conference. Laboratory and immunological tests were conducted. Disease activity was determined through the Birmingham Vasculitis Activity Score.

Results: Rituximab was preferred over cyclophosphamide in 37 patients and used as a second-line therapy after cyclophosphamide in 41 cases. Rituximab treatment showed favorable outcomes with regard to serum creatinine levels, proteinuria, and hematuria, as well as in cases of isolated lung involvement. Nearly half of patients with pulmonary renal syndrome also improved, with 22.2% achieving remission. ANCAs were positive in 85.9% of patients at the onset of rituximab treatment and became negative in 82% of the positive cases. Adverse events were rare and included infusion reactions (one case of reactivation of a herpes zoster infection and one case of allergic reaction).

Conclusion: Rituximab is an efficient and safe therapeutic option in patients with AAV who are difficult to treat, have insufficient response, or have not tolerated other treatments.

Objectives: This study aimed to determine whether GCSB-5 has anti-inflammatory and antinociceptive effects in mice with collagen-induced arthritis (CIA), an animal model of rheumatoid arthritis (RA), and investigate the influence of GCSB-5 on the mitogen-activated protein kinase (MAPK) pathway.

Materials and methods: The experimental animal study was designed to include five groups: CIA mice treated with GCSB-5 (300 mg/kg), GCSB-5 (600 mg/kg), celecoxib (60 mg/kg), or saline for four weeks, and nontreated control mice. The clinical severity of arthritis was scored. Nociceptive thresholds were measured by using a von Frey dynamic plantar analgesimeter. The MAPK pathway was evaluated in mouse synovium. The expression of channels associated with pain signaling was assessed by western blot and immunohistochemical staining.

Results: GCSB-5 treatment diminished the severity of clinical arthritis and increased the nociceptive threshold in mice with CIA. Celecoxib, a positive control drug, also showed comparable changes. Clinical arthritis scores were inversely related to mechanical thresholds. GCSB-5 administration decreased the levels of anti-type II collagen antibody and inflammatory cytokines in the sera of mice with CIA. Furthermore, ERK, p38 MAPK, and JNK phosphorylation were downregulated and TRPV1 and ASIC3 expression were decreased in the synovium of GCSB-5-treated mice compared to salinetreated mice. Interleukin-6-induced TRPV1 and ASIC3 upregulation were also inhibited by GCSB-5 in human RA fibroblast-like synoviocytes in vitro.

Conclusion: GCSB-5 decreased inflammatory arthritis and pain in a murine model of RA. The results present evidence that GCSB-5 may be beneficial for relieving pain as well as decreasing inflammation in autoimmune arthritis, such as RA.

Objectives: Considering that the comorbid situations during the management of Spondyloarthritis (SpA) have been underlined in several recommendations, the main objective of this study was to evaluate the comorbid conditions of Turkish patients with SpA.

Patients and methods: This cross-sectional observational study was conducted with 1,242 SpA patients (844 males, 398 females; mean age: 43.9±11.0 years; range, 19 to 81 years) diagnosed according to the modified New York criteria for ankylosing spondylitis or the Assessment of SpondyloArthritis International Society (ASAS) criteria. The patient data were collected from the Biologic and targeted Synthetic antirheumatic drugs Registry (BioStar) between February 1, 2019, and December 29, 2020. Clinical and demographic data, including, age, sex, disease duration, body mass index (BMI), pain, patient's global assessment, physician's global assessment, Bath Ankylosing Spondylitis Disease Activity Index, Ankylosing Spondylitis Disease Activity Score, Bath Ankylosing Spondylitis Functional Index, Bath Ankylosing Spondylitis Metrology Index, and Maastricht Enthesitis Score, were recorded. Comorbid conditions were recorded by filling out a questionnaire according to the clinical history or medical records. Charlson Comorbidity Index and Rheumatic Disease Comorbidity Index scores were calculated from the gathered comorbidity information.

Results: Nine hundred thirteen patients had radiographic axial SpA, 153 had nonradiographic axial SpA, and 176 had peripheral SpA. The most common comorbidities were hypertension (HT) (n=167, 13.4%), diabetes mellitus (DM) (n=83, 6.7%), thyroid disorders (n=64, 5.6%), and depression (n=61, 4.9%). The comorbidities and the calculated comorbidity indices were significantly higher in females, in those with a BMI >25 kg/m² , and those over 60 years of age. No relationship was found between smoking and alcohol use and comorbidities. A significantly higher prevalence of HT and DM in peripheral SpA patients and a lower prevalence of thyroid disorders in radiographic axial SpA patients were observed.

Conclusion: The most commonly reported comorbidities were HT, DM, thyroid disorders, and depression in SpA patients according to the BioStar database. The frequency of comorbidities and composite comorbidity scores were higher among females, older (>60 years) patients, and overweight (BMI >25 kg/m² ) patients.

Objectives: This study aims to assess the prevalence of generalized joint hypermobility (GJH) in school children in relation to scoliosis and to identify musculoskeletal problems.

Patients and methods: This cross-sectional study included 822 school children (413 males, 409 females; mean age 12.2±1.3 years; range, 10 and 15 years). Demographic characteristics of all children were recorded. The presence of GJH was assessed by the Beighton score (≥4 was considered joint hypermobility). Scoliosis screening consisted of forward bend test (FBT) and measurement of angle of trunk rotation (ATR). Positive FBT or ATR ≥5° was referred to a portable X-ray device. The presence of musculoskeletal complaints was determined by a questionnaire.

Results: Children's Body Mass Index (BMI) was 19.6±4.1. GJH was diagnosed in 151 subjects (18.4%). No significant association was detected between sex and hypermobility. Joint hypermobility was inversely correlated with age and BMI. Scoliosis was found in 43 subjects (5.2%) and all of them except one girl had mild scoliosis. The most common scoliosis pattern was a single left thoracolumbar curve. Seventy-three subjects (8.9%) had Cobb angle under 10°, with a potential for progression. Among subjects having GJH, the most common clinical finding was pes planus (34.3%) and the most common clinical symptom was ankle sprain (31.3%).

Conclusion: Similar to that found in children from many countries, GJH is a common clinical condition in Turkish children. GJH should be assessed in the differential diagnosis of adolescents with musculoskeletal complaints for effective treatment and reducing morbidity. GJH should be considered in adolescents with scoliosis, which may be an important aspect in treatment.