Objective: Promising therapeutic approaches have emerged in chronic periaortitis, whereas peripheral blood biomarkers are lacking. CC-chemokine ligand 18 (CCL18) is known as a marker of fibrotic activity and prognosis in pulmonary fibrosis. We investigated whether CCL18 levels are increased in patients with chronic periaortitis and are associated with clinical, laboratory, and imaging findings.
Methods: In this retrospective study, serum concentrations of CCL18 were assessed in 30 patients with chronic periaortitis and related to clinical data, laboratory variables, and imaging studies. Serum levels were compared to 15 apparently healthy volunteers and 15 controls with aortic sclerosis.
Results: Serum concentrations of CCL18 were increased in patients with chronic periaortitis (median 197.6 ng/ml, range 73.7-301.0) compared to healthy volunteers (median 34.6 ng/ml, range 22.6-70.4; p < 0.0001) and controls with aortic sclerosis (median 50.4 ng/ml, range 24.5-141.2; p < 0.0001). CCL18 levels correlated with (n = 30; r = 0.461, p = 0.01) and increased with the transversal diameter of the periaortic mantle < 5, 5-10, and ≥ 10 mm (p = 0.008). Contrast enhancement (p = 0.044), treatment naivety (p = 0.042), and the occurrence of systemic symptoms (p = 0.007) were associated with higher CCL18 levels. During followup, changes of CCL18 correlated with changes of the transverse diameter of the periaortic mantle (n = 17; r = 0.512, p = 0.033).
Conclusion: Serum concentration of CCL18 reflects fibroinflammatory activity and extent of disease in patients with chronic periaortitis.
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http://dx.doi.org/10.3899/jrheum.111143 | DOI Listing |
Int J Gen Med
January 2025
Department of Urology, Peking University People's Hospital, Beijing, 100044, People's Republic of China.
Objective: This study investigated the efficacy of comprehensive management and predictable inflammatory markers for idiopathic retroperitoneal fibrosis (iRPF)-related hydronephrosis outcomes.
Methods: Patients with iRPF-related hydronephrosis underwent surgical (ureteral stent and/or nephrostomy tube placement) and medical (corticosteroid-based multiple immunosuppressants) management were classified according to stent-indwelling outcomes. Univariate analysis of clinical profiles was conducted to screen possible predictors of hydronephrosis remission.
Zhong Nan Da Xue Xue Bao Yi Xue Ban
July 2024
Department of Endocrinology, Third Xiangya Hospital, Central South University, Changsha 410013, China.
IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can affect multiple organs throughout the body, predominantly in middle-aged and elderly males, with a male-to-female ratio of 2꞉1 to 3꞉1. IgG4-related retroperitoneal fibrosis (IgG4-RPF), a rare subtype of IgG4-RD, has an unclear etiology, and its comorbidity with type 2 diabetes mellitus is also uncommon. A lack of awareness of this condition in clinical practice can easily lead to misdiagnosis.
View Article and Find Full Text PDFDis Mon
January 2025
Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow-226025, (U.P.), India. Electronic address:
Erdheim-Chester disease (ECD) is an extremely rare non-Langerhans cell disorder that is believed to include both inflammatory and neoplastic characteristics. It is caused due to genetic mutations in proto-oncogenes like BRAF and MEK, while immunological pathways have an essential role in the onset and progression of the disease. Despite its rarity, ECD poses significant diagnostic and therapeutic challenges due to its heterogeneous clinical presentation and limited understanding of its underlying pathophysiology.
View Article and Find Full Text PDFClin Rheumatol
January 2025
Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China.
Objective: Retroperitoneal fibrosis (RPF) is a rare condition marked by inflammation and fibrosis affecting the peritoneal and retroperitoneal soft tissues. In recent years, the identification of IgG4-related diseases has brought to light a significant association with fibrous disorders, including RPF, which were once considered independent. In this comprehensive cohort study, we performed a comparative analysis of the demographic, clinical, laboratory, histopathological, and therapeutic characteristics between patients with IgG4-related RPF and those with idiopathic retroperitoneal fibrosis (iRPF).
View Article and Find Full Text PDFCureus
November 2024
Department of Internal Medicine/Rheumatology, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, USA.
Retroperitoneal fibrosis (RPF) is a rare disease with a nonspecific presentation. RPF can be classified into Idiopathic, the most common, or secondary due to malignancy and various medications resulting in chronic inflammation and fibrosis in the retroperitoneum. The complications arise due to the compression of structures in the retroperitoneum.
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