Zeitschrift fur Rheumatologie最新文献

Background: Gastrointestinal involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA) and has a poor prognosis.

Objectives: The aim of our study was to determine the density of eosinophils in the gastrointestinal tract that should be considered pathological in patients with EGPA, and how clinically and prognostically relevant intramucosal eosinophil accumulation is in patients with EGPA.

Methods: Forty-nine subjects were included in the study: 18 with EGPA (9 women) and as a control group 31 with other rheumatological inflammatory diseases. Two hundred and forty-four biopsies from the gastrointestinal tract of the study participants were analyzed.

Results: Histologically, there was no significant difference in eosinophil counts between patients with EGPA and the control group; however, there was an increased density when macroscopic gastrointestinal inflammation was already present.

Conclusions: Biopsies from macroscopically unremarkable mucosa appear to have limited diagnostic utility.

Objective: This study aimed to evaluate and compare the diagnostic performance of immunoglobulin G4/Immunoglobulin G (IgG 4/IgG) and immunoglobulin G4 (IgG 4) alone in identifying immunoglobulin G4-related disease (IgG 4-RD).

Methods: A systematic review and meta-analysis were conducted using data from Medline, Embase, and the Cochrane Library from inception to November 2024. Two meta-analyses were performed to assess the diagnostic accuracies of IgG 4/IgG and IgG 4 in IgG 4-RD patients.

Results: Eight studies encompassing 754 IgG 4-RD patients and 9496 non-IgG 4-RD controls were included in the analysis. IgG 4/IgG demonstrated a sensitivity of 89% and a specificity of 91.6%, accurately detecting IgG 4-RD in 89% of cases and correctly identifying non-IgG 4-RD in 91.6% of cases. IgG 4 alone exhibited a higher sensitivity (94.9%) and a similar specificity (91%), indicating a slightly improved ability to identify IgG 4-RD cases. The positive likelihood ratio (PLR) and the negative likelihood ratio (NLR) for IgG 4/IgG were 7580 and 0.132, respectively, while IgG 4 alone had a PLR of 6403 and a lower NLR of 0.066, confirming the high diagnostic reliability. The diagnostic odds ratio (DOR) was 62.97 for IgG 4/IgG compared to 105.6 for IgG 4 alone, reflecting enhanced accuracy. The area under the curve (AUC) was 0.949 for IgG 4/IgG and 0.986 for IgG 4. The Q* index was 0.889 for IgG 4/IgG and 0.949 for IgG 4, further underscoring the diagnostic effectiveness of IgG 4 alone.

Conclusion: Both IgG 4/IgG and IgG 4 are highly accurate markers for diagnosing IgG 4-RD, with IgG 4 alone showing marginally higher sensitivity, DOR, and AUC. This suggests that IgG 4 alone may offer a slight advantage as a diagnostic marker for IgG 4-RD.

Objective: This study aimed to ascertain and compare the diagnostic efficacy of ferritin and glycosylated ferritin in adult-onset Still's disease (AOSD).

Methods: A thorough meta-analysis was conducted using data extracted from MEDLINE, Embase, and the Cochrane Library. Separate meta-analyses were performed to assess the diagnostic accuracies of ferritin and glycosylated ferritin in patients with AOSD.

Results: Eight studies encompassing 556 AOSD patients and 763 non-AOSD controls were included in the analysis. Ferritin cut-off values ranged widely, from 400 to 5000 μg/L, with some studies using a relative threshold, such as ≥ 5 times the normal upper limit. Glycosylated ferritin was reported, with thresholds ranging from 16 to 33%. Ferritin displayed a sensitivity of 60.4% and a specificity of 85.7%, accurately identifying AOSD in 60.4% of cases and accurately excluding non-AOSD in 85.7% of cases. The sensitivity in ferritin-specific studies escalated to 66.8%, while specificity was maintained at 84.7%. Glycosylated ferritin exhibited a higher sensitivity of 74% and a slightly lower specificity of 80.5%, indicating superior detection of AOSD cases. The positive likelihood ratio (PLR) and negative likelihood ratio (NLR) for ferritin were 4.179 and 0.399, respectively; for glycosylated ferritin alone, these values were 3.604 and 0.357, demonstrating moderate diagnostic reliability. Diagnostic odds ratios (DORs) were 11.32 for ferritin and 10.14 for glycosylated ferritin. The area under the curve (AUC) was 0.778 for ferritin and 0.835 in ferritin-specific studies, indicating moderate diagnostic precision. The AUC for glycosylated ferritin was not available. The Q* index was 0.717 for ferritin and 0.768 for the ferritin-only group, reflecting a slight improvement when focusing exclusively on ferritin.

Conclusion: Both ferritin and glycosylated ferritin serve as valuable markers for diagnosing AOSD, with ferritin demonstrating superior sensitivity and specificity in targeted studies. Nonetheless, their moderate diagnostic effectiveness suggests that these biomarkers should be used in conjunction with other clinical criteria to ensure a comprehensive diagnosis.

Background: Interstitial lung diseases (ILD) represent an interdisciplinary clinical challenge and are not uncommonly associated with rheumatological diseases. Interstitial lung disease multidisciplinary meetings (ILD-MDM) provide a structured platform for interdisciplinary case discussions and decision making. Despite their great importance in patient care, data on the prevalence, structure and function of ILD-MDM in Germany are lacking.

Objective: The aim of the study was to assess the current status of ILD-MDM in German hospitals to gain insights into their composition, processes and potential for optimization.

Material and methods: A web-based survey was conducted via SurveyMonkey under the auspices of the German Society for Rheumatology and Clinical Immunology (DGRh) and in collaboration with the German Respiratory Society (DGP) and the German Radiological Society (DRG). A standardized questionnaire captured information on the participating specialist disciplines, organizational structures as well as the content and challenges of local ILD-MDM. The analysis was conducted descriptively.

Results: A total of 125 physicians from 15 federal states in Germany participated. Pulmonologists (93.6%), radiologists (86.4%), rheumatologists (59.2%) and pathologists (57.6%) are the most commonly represented members of ILD-MDM. The majority of ILD-MDMs are conducted either in person (50%) or in a hybrid format (31.5%) and are held on a weekly basis (41.1%). Of all patient cases discussed, two thirds receive a definitive diagnosis and treatment recommendation.

Conclusion: The findings reveal a high acceptance and prevalence of ILD-MDM in Germany but also highlight potential areas for improvement, particularly regarding interdisciplinary participation, technical infrastructure and standardization.

Objective: This study aimed to reassess the diagnostic contribution of minor salivary gland biopsy (MSGB) in patients with clinically suspected Sjögren's disease (csSjD) and to assess its association with serological status, clinical features, and fulfillment of classification criteria.

Methods: We retrospectively analyzed 313 patients who underwent MSGB for evaluation of csSjD between 2021 and 2024. All patients were evaluated according to 2016 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria at the end of the diagnostic workup. The MSGB was defined as positive if the focus score was ≥ 1/4 mm². Subgroup analyses were performed according to anti-Ro/SSA antibody status. Predictors of MSGB positivity were examined using logistic regression, and diagnostic performance was evaluated by receiver operating characteristic (ROC) analysis.

Results: The overall MSGB positivity rate was 24.6%. Rates were comparable between anti-Ro-positive (27.8%) and negative (24.2%) patients (p = 0.655). In seronegative patients, older age and longer symptom duration independently predicted biopsy positivity (p < 0.05), although the diagnostic discrimination was limited (AUC: 0.511). Notably, MSGB enabled fulfillment of classification criteria in 22.3% of seronegative cases. No clinical or laboratory variables predicted biopsy outcome in seropositive patients. Across the entire cohort, only CRP showed a weak correlation with histological severity (p = 0.05); MSGB positivity was not associated with systemic manifestations.

Conclusion: Minor salivary gland biopsy provides substantial diagnostic value in seronegative csSjD, where clinical and serological data are insufficient. In contrast, its additional contribution in seropositive patients is limited. While biopsy does not reliably predict systemic disease activity, it remains a cornerstone diagnostic tool, especially in seronegative patients with objective sicca symptoms. Integration of MSGB with salivary ultrasonography and molecular biomarkers may further optimize future diagnostic pathways.