Scandinavian Journal of Rheumatology最新文献

Objectives: To evaluate the relationship between anti-neutrophil cytoplasmic antibody (ANCA) positivity and the disease characteristics, treatment, and prognosis of eosinophilic granulomatosis with polyangiitis (EGPA).

Method: We conducted a retrospective cohort study of patients with new-onset or severely relapsing ANCA-associated vasculitis, enrolled in the J-CANVAS registry. The clinical characteristics at baseline, treatments, and prognoses of ANCA-positive and ANCA-negative patients were assessed.

Results: Three patients with positive proteinase-3 ANCA were excluded, and 166 patients with EGPA (new onset, 159; severe relapse, seven) were included. Sixty-two patients were myeloperoxidase (MPO)-ANCA positive and 104 patients were negative. No differences in age or sex were observed between the two groups. The MPO-ANCA-positive group had significantly more frequent mucous membrane and eye involvement, more frequent renal involvement, higher total Birmingham Vasculitis Activity Score, higher neutrophil counts, and higher C-reactive protein levels at baseline. Although rituximab was administered more frequently in the MPO-ANCA-positive group, no other differences in treatment were found. Both groups had comparable estimated glomerular filtration rates and prednisolone doses at weeks 24 and 48. The incidence rates of severe relapse, minor relapse, and serious infectious diseases were comparable. Cox regression analysis revealed that MPO-ANCA positivity was not a significant factor in serious infectious diseases and relapse.

Conclusion: Patients with MPO-ANCA-positive EGPA demonstrated different baseline clinical characteristics from MPO-ANCA-negative patients. However, subsequent relapses and serious infectious diseases were comparable.

Objective: Systemic lupus erythematosus (SLE) is associated with increased cancer risk. However, the patterns of cancer incidence remain unclear. This study aimed to evaluate the cancer risk in patients with SLE.

Method: This population-based cohort study identified 24 241 patients with newly diagnosed SLE between 2004 and 2020 using Korean National Health Insurance Service data. Patients were followed up until cancer diagnosis, death, or December 2021. Standardized incidence ratios (SIRs) were calculated to compare cancer risk between patients with SLE and the general population. Subgroup analyses were performed based on the age at diagnosis, follow-up duration, and use of immunosuppressive agents.

Results: Patients with SLE had higher risks of overall [SIR 3.3, 95% confidence interval (CI) 3.2-3.4], solid (SIR 3.1, 95% CI 3.0-3.2), and haematological (SIR 9.8, 95% CI 8.9-10.9) cancers compared with the general population. Among solid cancers, liver cancer had the highest risk, followed by ovarian cancer. The relative cancer risk peaked among patients aged 20-39 years (SIR 4.9, 95% CI 4.6-5.2) and during the first year after diagnosis (SIR 4.7, 95% CI 4.3-5.1). The SIRs for haematological, cervical, and lung cancers in cyclophosphamide-treated patients were higher than those for the corresponding cancers in the overall SLE population.

Conclusion: Patients with SLE have increased cancer risk compared with the general population. Increased relative cancer risk is associated with younger age, first year post-diagnosis, and cyclophosphamide treatment.

Objective: Raynaud's phenomenon (RP) is a common symptom and may be an early sign of systemic sclerosis (SSc). To identify biomarkers distinguishing whether RP is associated with definitive SSc, we used phosphospecific flow cytometry to measure phosphorylated (p) signalling molecules [signal transducers and activators of transcription (pSTAT3, pSTAT6, pSTAT4), pSmad2/3, nuclear factor-κB (pNF-κB)] in peripheral blood leucocytes of RP patients undergoing nailfold videocapillaroscopy.

Method: Leucocyte subsets (CD14⁺ monocytes, CD4⁺ and CD8⁺ T cells, CD19⁺ B cells) were identified by surface markers, and phosphorylation was measured after cytokine or lipopolysaccharide stimulation. Medical records were reviewed 9-10 years later, comparing RP subjects who developed SSc (SSc⁺, n = 8) with those who did not (SSc^-, n = 17) and healthy controls (HCs, n = 8).

Results: SSc⁺ patients had significantly higher constitutive pSmad2/3 levels in CD4⁺ T cells than SSc^- or HCs (p = 0.005 and p = 0.034). SSc⁺ and SSc^- had higher stimulated pSTAT3(pY705) levels in CD4⁺ T cells than HCs (p = 0.001 and p = 0.026). SSc⁺ had higher stimulated pSTAT6 levels in CD4⁺ T cells compared with HCs (p = 0.017) and in CD19⁺ B cells compared with SSc^- and HCs (p = 0.006 and p < 0.001). SSc⁺ had higher stimulated pSTAT4 levels in CD4⁺ T cells compared with SSc^- and HCs (p = 0.004 and p = 0.037) and in CD8⁺ T cells compared with SSc^- (p = 0.007). No significant differences were found in pNF-κB and pSTAT3(pS727) levels.

Conclusion: The results give insights into the pathogenesis of SSc. Smad2/3, STAT3(pY705), STAT6, and STAT4 pathways may serve as novel SSc biomarkers.

Objective: Becoming ill with a chronic disease during young adulthood is characterized by significant physical, psychological, and psychosocial transformations. These dynamic changes can add to the complexity of managing a newly diagnosed chronic disease. The aim of this study was to explore how young adults experienced and managed the disease and daily life during the first year after being diagnosed with a chronic inflammatory joint disease (IJD).

Method: A qualitative interview study based on semi-structured interviews was conducted and then interpreted using qualitative content analysis, including both manifest content and interpretations of underlying latent meaning. A total of 14 young adults (eight women and six men), ranging in age from 18 to 25 years, participated in the study within 1 year of being diagnosed with a chronic IJD.

Results: The analysis resulted in five categories and 12 subcategories that described the young adults' experiences during the first year after being diagnosed with chronic IJD. The five categories were: Processing towards diagnosis, Struggling with the consequences of the disease, Finding ways to manage the new situation, Needing received support, and Accepting the situation as a process.

Conclusion: The young adults demonstrated a strong fighting spirit as they adapted to new circumstances and found solutions to problems related to the disease. Their fighting spirit, combined with support from their significant others and healthcare professionals, helped them in this challenging time when their lifeworld was changing.

Objective: This study aimed to investigate the diagnostic value of inflammatory markers and the delta neutrophil index (DNI) in differentiating familial Mediterranean fever (FMF) attacks from acute appendicitis (AA).

Method: This retrospective analysis evaluated patients aged 0-18 years presenting with FMF attacks or suspected AA to Ankara Bilkent City Hospital Children's Hospital between 2019 and 2024. The FMF group comprised patients meeting ICD Eurofever/PRINTO classification criteria during an attack, while the AA group comprised pathologically confirmed AA cases. White blood cell count (WBC), absolute neutrophil count (ANC), absolute lymphocyte count (ALC), platelet count (PLT), C-reactive protein (CRP), DNI, and neutrophil-to-lymphocyte ratio (NLR) were recorded retrospectively.

Results: The study included 600 patients (200 FMF, 400 AA). WBC, ANC, NLR, and DNI values were significantly higher in the AA group (p < 0.001), whereas ALC and CRP were markedly elevated in the FMF group (p < 0.001). In receiver operating characteristics curve analysis, WBC, ANC, and NLR demonstrated high diagnostic performance, while DNI had limited value in distinguishing between the conditions. However, multivariate logistic regression showed that increases in DNI and ANC, and decreases in CRP and PLT levels, were statistically significant predictors of AA (p < 0.001).

Conclusion: In differentiating FMF attacks from AA in children, physical examination and imaging can be supported by blood count-based parameters. Combined evaluation of multiple laboratory markers may accelerate decision making in paediatric patients and contribute to effective management. However, their utility in routine practice should be interpreted with caution and supported by further research.

Objectives: To explore and compare the prevalences of a wide range of comorbidities in axial spondyloarthritis (axSpA) versus the general population, and in non-radiographic versus radiographic axSpA (nr-axSpA/r-axSpA).

Method: Well-characterized axSpA patients (n = 246) from the SPARTAKUS cohort (55% men; mean age/disease duration 52/26 years; nr-axSpA/r-axSpA = 82/164) were included, and matched to comparators from the general population (five/patient) randomly drawn from the Swedish Population Register. Fifty-nine comorbidities were determined from ICD-10 diagnosis registrations in primary/specialized care, with data retrieved for the 10 year period preceding SPARTAKUS inclusion (for patients/their respective comparators). Differences in comorbidity prevalences were analyzed using logistic regression and controlled for multiple comparisons.

Results: Most investigated comorbidities were numerically overrepresented in axSpA versus comparators with significantly higher prevalences of known extra-musculoskeletal manifestations [anterior uveitis (28% vs 1%), IBD (10%/1%), psoriasis (10%/3%)], and ischaemic heart disease (7%/3%), along with less explored conditions such as fibromyalgia/chronic pain (12%/3%) and nephrolithiasis (8%/3%). Patients displayed significantly higher proportions of well-known side-effects of non-steroidal anti-inflammatory drugs (NSAIDs) [gastritis (21%/10%), hypertension (31%/19%)] and glucocorticoids [cataract (11%/7%), glaucoma (7%/3%), osteoporosis/vertebral compression (4%/1%)]. No significant between-group difference (patients/comparators) was observed for Charlson Comorbidity Index (assessing short-term mortality risk) when excluding rheumatic disease. Moreover, no significant difference in comorbidity was found between nr-axSpA and r-axSpA.

Conclusion: axSpA is linked to several comorbidities, whereas no difference was observed between nr-axSpA and r-axSpA. Potential causes of enhanced comorbidity include long-standing inflammation and therapy side-effects (including from NSAIDs/glucocorticoids), both to be considered when aiming to optimize axSpA treatment.