Histopathology and expression of the chemokines CXCL10, CXCL13, and CXCR3 and the endogenous TLR-4 ligand S100A8/A9 in lymph nodes of patients with adult-onset Still’s disease

• Published in: Scientific reports 2019-05, Vol.9 (1), p.7517-11, Article 7517
• Main Authors: Kim, Hyoun-Ah, Kim, Yon Hee, Jeon, Yoon Kyung, Yang, Woo-Ick, Kwon, Ji Eun, Han, Jae Ho
• Format: Article
• Language: English
• Subjects: 13/51; 692/4023/1670/122; 692/53/2421; Adult; Calgranulin A - blood; Calgranulin A - metabolism; Calgranulin B - blood; Calgranulin B - metabolism; CD8 antigen; Chemokine CXCL10 - blood; Chemokine CXCL10 - metabolism; Chemokine CXCL13 - blood; Chemokine CXCL13 - metabolism; Chemokines; Chemokines - blood; Chemokines - metabolism; CXCL10 protein; CXCL13 protein; CXCR3 protein; Diagnosis, Differential; Differential diagnosis; Female; Fever; Histiocytic Necrotizing Lymphadenitis - diagnosis; Histopathology; Humanities and Social Sciences; Humans; Hyperplasia; Immunohistochemistry; Inflammatory diseases; Interleukin 1; Ligands; Lymph nodes; Lymph Nodes - immunology; Lymph Nodes - pathology; Lymphadenitis; Lymphadenitis - diagnosis; Lymphadenopathy; Lymphatic system; Lymphocytes T; Lymphoma; Lymphoma, T-Cell - diagnosis; Male; Middle Aged; multidisciplinary; Paraffin; Receptors, CXCR3 - metabolism; Retrospective Studies; Science; Science (multidisciplinary); Still's Disease, Adult-Onset - diagnosis; Still's Disease, Adult-Onset - immunology; Still's Disease, Adult-Onset - pathology; T-cell lymphoma; Toll-Like Receptor 4 - metabolism; Tuberculosis; Tuberculosis, Lymph Node - diagnosis; Tuberculous lymphadenitis
• ISSN: 2045-2322; 2045-2322
• DOI: 10.1038/s41598-019-44032-6

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease manifesting with a persistent high-spiking fever, a typical rash, and lymphadenopathy. Endogenous factors related to interleukin-1, such as S100A8/A9 and several chemokines including CXCL10, CXCR3, and CXCL13, potentially play roles in its pathogenesis. We describe the histopathological features and chemokine expression pattern in lymph nodes (LNs) of patients with AOSD. Formalin-fixed, paraffin-embedded excisional LN tissues from 48 patients with AOSD were histologically reviewed. CXCL10, CXCR3, CXCL13, and S100A8/A9 expression was evaluated immunohistochemically. The pathology of LN was characterized by paracortical hyperplasia with proliferation of histiocyte, immunoblast, CD8-positive lymphoid cell and blood vessel. Most cases required differential diagnosis from dermatopathic lymphadenitis (n = 16, 33.3%), T cell lymphoma (n = 11, 22.9%), and histiocytic necrotizing lymphadenitis (HNL) (n = 9, 18.8%). The expression levels of CXCL10 and CXCR3 were higher in patients with AOSD than in those with T cell lymphoma, HNL, tuberculous lymphadenitis, and reactive hyperplasia. It is important to recognize the aforementioned histopathologic findings of nodal involvement of AOSD because improper diagnosis and treatment can be avoided. Immunohistochemical staining for chemokines, CXCL10 and CXCR3, may aid in differentiating AOSD from other mimickers.