BMC Rheumatology最新文献

Background: Granulomatosis with polyangiitis (GPA) is a rare form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that can involve multiple organ systems, including the gastrointestinal (GI) tract. Although GI manifestations are relatively uncommon, they may be associated with serious complications and adverse outcomes. This study aimed to assess the frequency and types of GI involvement in patients with GPA and to examine their relationship with disease severity, prognosis, and treatment response.

Methods: In this retrospective cohort study, clinical records of 220 patients with a confirmed diagnosis of GPA who were referred to Amir Alam Hospital between 2013 and 2021 were reviewed. Data on demographic characteristics, GI symptoms, Birmingham Vasculitis Activity Score (BVAS), therapeutic response, relapse rates, and mortality were collected and analyzed.

Results: GI involvement was observed in 18 patients (8.2%). The most common manifestations included hepatitis, mesenteric ischemia, diarrhea, pancreatitis, and elevated liver enzymes. Patients with GI involvement had significantly higher BVAS scores (mean 21 vs. 15.8, p = 0.004), a markedly increased mortality risk (hazard ratio = 3.24, p < 0.001), and a shorter time to first relapse (mean 5.2 vs. 10.3 months, p = 0.041) compared to those without GI symptoms.

Conclusion: Gastrointestinal involvement in GPA is associated with more severe disease activity, diminished treatment response, and increased mortality. Early detection and appropriate management of GI manifestations may improve clinical outcomes. Further prospective studies are warranted to elucidate the underlying mechanisms and optimize treatment strategies for this high-risk subgroup.

Background: SLE is a chronic autoimmune illness that affects several systems. Gastrointestinal abnormalities, although uncommon, can be dangerous and frequently go undiagnosed due to vague symptoms. Lupus enteritis and peritonitis are serious consequences. Lupus enteritis affects up to 9.7% of SLE patients and causes stomach discomfort, nausea, and diarrhea. It frequently involves intestinal vasculitis, which results in gut wall thickening and, in extreme instances, ischemia or perforation. Lupus peritonitis is very uncommon. Differentiating them from other causes of stomach discomfort is critical. This case emphasizes the diagnostic problems, imaging results, and the significance of early, focused therapy for better outcomes.

Case presentation: A 38-year-old lady from Hebron, Palestine, reported 3 days of increasing, colicky abdominal discomfort, bloody vomiting, and black feces. She had a three-year history of migrating polyarthralgia, photosensitivity, alopecia, and two first-trimester losses. Her cousins' family history indicated rheumatoid arthritis and hypothyroidism. Upon examination, she seemed pale and unwell, with stomach discomfort and right basal lung crepitations. Laboratory results revealed microcytic anemia, lymphopenia, hypokalemia, hypophosphatemia, increased ESR and CRP, and a positive Coombs test. High levels of ANA, anti-dsDNA, and anti-Sm antibodies proved systemic lupus erythematosus (SLE). The imaging indicated a pulmonary embolism, pleural effusion, and thickening of the jejunal wall. Methylprednisolone and anticoagulant treatment were started. Gastroscopy indicated hemorrhagic gastritis, and paracentesis revealed exudative ascitic fluid due to ascites worsening. The patient was given cyclophosphamide with mesna, which showed improvement. The patient improved on treatment with prednisolone, hydroxychloroquine, and a proton pump inhibitor after discharge. Follow-up showed continued recovery without recurrence of symptoms.

Conclusion: Systemic lupus erythematosus (SLE) is an autoimmune illness that causes uncommon gastrointestinal symptoms such as lupus enteritis and peritonitis. This case demonstrates their simultaneous occurrence, underscoring the need to include SLE in the workup for gastrointestinal symptoms. Proper diagnosis by laboratories, imaging, and gastroscopy is critical since early intervention dramatically improves patient outcomes.

Clinical trial number: Not applicable.

Background: Annual fitness assessments are performed during group exercise therapy for people with axial Spondyloarthritis (axSpA) living in Switzerland. The core strength test (CST) is time-consuming, and interpretation limited. Thus, the objectives were to 1) compare the CST-performance of people with axSpA and healthy controls, and 2) evaluate if hand grip strength can be used as a proxy for core strength.

Methods: Routinely gathered data of people with axSpA was used and data from healthy controls collected. Differences in strength were investigated using Welch Two-sample t-tests or Fisher's exact tests. The associations between grip and core strength were explored through pairwise Pearson correlations (r). Further, a linear regression model was fitted to data of people with axSpA with grip strength as the response variable, and ventral, dorsal and lateral core strength endurance, age and sex as predictors.

Results: Data from 160 healthy controls (50% male, mean age 59.3 (SD 11.47) years) and 112 people with axSpA (58% male, mean age 57.7 (SD 12.1) years) was included. People with axSpA showed lower core strength endurance (sec) than the controls: ventral core strength mean difference -28, p < 0.001; lateral core strength mean difference -17, p < 0.001; dorsal core strength mean difference -39, p < 0.001, and lower grip strength -3.7, p = 0.012. The linear regression model with hand grip as response and core strength, age, and sex as predictors explained 44% of the variability.

Conclusion: People with axSpA showed substantially lower core muscle strength endurance than healthy controls. Core strength measures have only marginal effects on grip strength in people with axSpA. Therefore, grip strength is not appropriate to be used a s a proxy for core strength in people with axSpA and healthy people.

Clinical trial number: Not applicable.

Introduction: Idiopathic inflammatory myopathies are a heterogeneous group of chronic autoimmune diseases characterized by inflammation and muscle weakness. In Colombia, these conditions have been poorly studied. Therefore, an analysis of the prevalence and demographic characteristics of the main reported inflammatory myopathies in the country (dermatomyositis and polymyositis) was carried out, based on data from the Ministry of Health.

Materials and methods: A descriptive observational study was conducted. Bayesian methods were employed for frequency analysis. Data were collected from the Comprehensive Information System of the Ministry of Health of Colombia during the period 2018 to 2021. Key diagnostic codes from the international manual of diseases (ICD-10) related to dermatomyositis and polymyositis were used.

Results: A prevalence of 8.3 cases per 100,000 inhabitants was found, with a female-to-male ratio of 2.4:1 and an average age of 51.5 years. These conditions were more common in the age group between 71 and 80 years. The highest prevalence was observed in the western area of Colombia, especially in Antioquia and in the Eje Cafetero region.

Conclusions: The point prevalence during the period from 2018 to 2021 remained relative constant. A predominance of these diseases was found in the female population of advanced age, with the Southwest region being the most affected and the Southeast being the least affected. This study is one of the few focused on describing the epidemiology of dermatomyositis and polymyositis in Colombia recently and is the first one made using a Bayesian statistical approach in this specific topic and population.

Background: Axial spondyloarthritis (axSpA) exhibits notable sex-related differences, particularly in Asian populations, influenced by genetic, cultural, and healthcare factors. This systematic review and meta-analysis assess sex-specific disparities in axSpA among Asian patients.

Methods: A systematic search in PubMed, Embase, and Google Scholar (up to January 30, 2025) included studies reporting sex-specific differences in clinical presentation, diagnostic delay, treatment response, and quality of life. The study included 31,523 males and 10,815 females. Risk of bias was assessed using the ROBINS-I tool. Meta-analysis was performed using R and Jamovi, with heterogeneity assessed via I² statistics.

Results: Thirty studies comprising 31,523 male and 10,815 female axSpA patients were included. Males had an younger age of onset and diagnosis (MD: -3.48 years; 95% CI: -4.78 to -2.17; -3.57 years; 95% CI: -5.25 to -1.89). No significant differences in diagnostic delay were observed. (MD: -0.62 years; 95% CI: -1.55 to 0.32; p = 0.0551). Females exhibited higher disease activity scores (BASDAI MD: -0.57; 95% CI: -0.86 to -0.27; p = 0.00211) and inflammatory markers (ESR MD: -3.99 mm/h; p = 0.0476), whereas males showed greater radiographic progression (BASRI MD: 2.60; 95% CI: 1.86-3.34; p < 0.001). HLA-B27 positivity was higher in males (79.5% vs. 69.2%). Treatment adherence was lower in females.

Discussion: This study highlights critical sex-based disparities in axSpA among Asian populations, particularly regarding disease onset, progression, and treatment response. The findings emphasize the need for sex-specific diagnostic and therapeutic strategies to improve patient outcomes.

Registration: The review protocol was registered on PROSPERO (CRD42025637163).

Objective: The presentation and outcomes of Systemic lupus erythematosus (SLE) are influenced by ethnicity and genetic background. The United Arab Emirates (UAE) is one of the leading countries of SLE per recent reports. In this study, we evaluated the effect of positive family history (FHx) of SLE and autoimmunity on clinical presentations and disease outcomes.

Methods: A retrospective observational study of patients seen between 2011 till 2023 was conducted. Included patients were those fulfilling the 2019 EULAR/ACR classification criteria. Comparative analyses were conducted between those with familial history of autoimmunity and SLE and those without.

Results: Out of 279 SLE patients, a total of 241 patients fulfilled the 2019 EULAR/ACR classification criteria and were included in the study. There was positive FHx of autoimmunity in 27% of the study population, and positive FHx of SLE (in first-degree relatives, "familial SLE") in 14.5% of the study population. Comparisons between positive and negative FHx of autoimmunity/SLE showed younger age at diagnosis in those with positive FHx of autoimmunity (p-value = < 0.001) and higher frequency of Raynaud's phenomonen (p-value = 0.022). Patients with familial SLE were also younger at diagnosis (p- value = 0.004) and had more mucocutaneous features (p-value = 0.042).

Conclusion: The percentage of familial SLE in our UAE study population is 14.5% which is higher than reported in non-Arab study populations. In our study population, patients with familial SLE and familial autoimmunity tend to present earlier, while patients with familial SLE tend to have more mucocutaneous features than those without familial SLE.

Clinical trial number: Not applicable.