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CC-chemokine ligand 18 is a useful biomarker associated with disease activity in IgG4-related disease
  1. Mitsuhiro Akiyama,
  2. Hidekata Yasuoka,
  3. Keiko Yoshimoto,
  4. Tsutomu Takeuchi
  1. Department of Internal Medicine, Division of Rheumatology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan
  1. Correspondence to Dr Mitsuhiro Akiyama, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan; hhhirooo{at} and Dr Tsutomu Takeuchi, Division of Rheumatology, Department of Internal Medicine, Keio UniversitySchool of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan; tsutake{at}

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IgG4-related disease (IgG4-RD) is a systemic disorder characterised by elevated serum IgG4 levels, tissue infiltration by IgG4+ plasma cells and severe fibrosis.1 2 However, biomarkers for IgG4-RD disease activity are lacking.3 A recent report demonstrated that CC-chemokine ligand 18 (CCL18) was a substantial biomarker for fibrotic diseases.4 Here, we investigated the correlation between serum CCL18 levels and clinical features of patients with IgG4-RD.

Written informed consent was obtained from all patients and healthy controls. Twenty-eight consecutive patients with active, untreated IgG4-RD diagnosed based on the 2011 comprehensive diagnostic criteria5 and 16 healthy controls were enrolled. Diagnosis of IgG4-RD was biopsy proven in 26 patients (93%). Disease activity was assessed using the IgG4-RD responder index (IgG4-RD RI).6 Healthy controls had no autoimmune diseases, atopic diseases or active infections at enrolment. Serum CCL18 levels were measured using a human CCL18/PARC Quantikine ELISA Kit (R&D Systems, Minneapolis, Minnesota, USA).

Characteristics of patients are shown in table 1. The mean age of patients with IgG4-RD and healthy controls was 59.7 and 47.3 years, and the proportion of females were 50% (14/28) and 69% (11/16), respectively.

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Table 1

Characteristics of patients with IgG4-related disease

Serum CCL18 levels in patients …

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