Original Article

Modern Rheumatology

, Volume 22, Issue 3, pp 419-425

Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases

  • Motohisa YamamotoAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine Email author 
  • , Tetsuya TabeyaAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
  • , Yasuyoshi NaishiroAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
  • , Hidetaka YajimaAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
  • , Keisuke IshigamiAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
  • , Yui ShimizuAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
  • , Mikiko ObaraAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
  • , Chisako SuzukiAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
  • , Kentaro YamashitaAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
    • , Hiroyuki YamamotoAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
    • , Toshiaki HayashiAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
    • , Shigeru SasakiAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
    • , Toshiaki SugayaAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
    • , Tadao IshidaAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
    • , Ken-ichi TakanoAffiliated withDepartment of Otolaryngology, Sapporo Medical University School of Medicine
    • , Tetsuo HimiAffiliated withDepartment of Otolaryngology, Sapporo Medical University School of Medicine
    • , Yasuo SuzukiAffiliated withDepartment of Ophthalmology, Teine Keijinkai Hospital
    • , Norihiro NishimotoAffiliated withLaboratory of Immune Regulation, Wakayama Medical University
    • , Saho HondaAffiliated withDepartment of Internal Medicine and Rheumatology, JR Sapporo Hospital
    • , Hiroki TakahashiAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine
    • , Kohzoh ImaiAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of MedicineAdvanced Clinical Research Center, The Institute of Medical Science, The University of Tokyo
    • , Yasuhisa ShinomuraAffiliated withFirst Department of Internal Medicine, Sapporo Medical University School of Medicine

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Abstract

IgG4-related disease (IgG4-RD) is a novel disease entity that includes Mikulicz’s disease, autoimmune pancreatitis (AIP), and many other conditions. It is characterized by elevated serum IgG4 levels and abundant IgG4-bearing plasmacyte infiltration of involved organs. We postulated that high levels of serum IgG4 would comprise a useful diagnostic tool, but little information is available about IgG4 in conditions other than IgG4-RD, including rheumatic diseases. Several reports have described cutoff values for serum IgG4 when diagnosing IgG4-RD, but these studies mostly used 135 mg/dL in AIP to differentiate from pancreatic cancer instead of rheumatic and other common diseases. There is no evidence for a cutoff serum IgG4 level of 135 mg/dL for rheumatic diseases and common diseases that are often complicated with rheumatic diseases. The aim of this work was to re-evaluate the usual cutoff serum IgG4 value in AIP (135 mg/dL) that is used to diagnose whole IgG4-RD in the setting of a rheumatic clinic by measuring serum IgG4 levels in IgG4-RD and various disorders. We therefore constructed ROC curves of serum IgG4 levels in 418 patients who attended Sapporo Medical University Hospital due to IgG4-RD and various rheumatic and common disorders. The optimal cut-off value of serum IgG4 for a diagnosis of IgG4-RD was 144 mg/dL, and the sensitivity and specificity were 95.10 and 90.76%, respectively. Levels of serum IgG4 were elevated in IgG4-RD, Churg–Strauss syndrome, multicentric Castleman’s disease, eosinophilic disorders, and in some patients with rheumatoid arthritis, systemic sclerosis, chronic hepatitis, and liver cirrhosis. The usual cut-off value of 135 mg/dL in AIP is useful for diagnosing whole IgG4-RD, but high levels of serum IgG4 are sometimes observed in not only IgG4-RD but also other rheumatic and common diseases.

Keywords

Autoimmune pancreatitis Churg–Strauss syndrome IgG4 Mikulicz’s disease Rheumatoid arthritis