Published online Nov 6, 2018. doi: 10.12998/wjcc.v6.i13.707
Peer-review started: July 31, 2018
First decision: August 24, 2018
Revised: September 16, 2018
Accepted: October 11, 2018
Article in press: October 11, 2018
Published online: November 6, 2018
A sixty-seven-year-old female presented with a progressive multi-organ involvement of IgG4-related disease (IgG4-RD) for over 19 years.
As a disorder with multiple organ involvement, IgG4-RD presents no specifically clinical manifestation for an early diagnosis, and the diagnosis largely relies on a combination of clinical manifestation, histology, imaging, and serology.
IgG4-RD has a broad spectrum of clinical manifestations and it is important to differentially diagnosis it from various diseases of the involved organs, such as Mikuri’s disease, autoimmune pancreatitis, interstitial pneumonia and retroperitoneal fibrosis.
A significantly increased serum IgG4 level (> 1350 mg/L) is a signal for serological diagnosis in IgG4-RD.
Computed tomography, magnetic resonance imaging or endoscopic retrograde cholangiopancreatography imaging showed a swollen feature or tumor-like structure in the involved organs.
IgG4-positive lymphocyte infiltration and sclerosis in the involved organs.
The patient was given methylprednisolone 200 mg/d pulse therapy for 3 d, a large dose of gamma globulin (20 g/d) intravenous infusion for 3 d and taking oral prednisolone 30 mg/d for three weeks. The prednisolone was then gradually tapered.
Patients with a clinically unclear cause of inflammation, swelling and refractory glands (such as the lacrimal gland, parotid gland and thyroid), rhinitis, pancreatitis, hypophysitis, and/or interstitial pneumonia should be considered for the possibility of IgG4-RD and recommended for plasma IgG4 test.
IgG4-RD is a systemic and clinical entity with autoimmune pathogenesis, which is characterized by high levels of circulating IgG4 and a dramatic response to steroid therapy.
Since the clinical manifestations of IgG4-RD are commonly similar to features of other diseases, it is easily misdiagnosed and improperly treated. This case emphasizes a clinical importance of the early diagnosis and interventions for IgG4-RD. From a diagnostic standpoint, the early disease symptoms are generally mild with an involvement of one or two organs for a long time before they gradually develop into multiple organ involvements with life-threatening complications. Apart from clinical symptoms, concentrations of serum IgG4, IgE, ESR, CRP and other laboratory results may provide important diagnostic data and clues for early identity disease activities. From a therapeutic standpoint, patients with IgG4-RD respond well to steroid therapy, and/or immunosuppressive agents resulting in a significant remission of disease and improved patient conditions. Notably, the disease can easily recur after the steroid is reduced or withdrawn. To reduce the adverse effects of long-term steroid therapy, it is critical to give pulse therapy with high doses of steroids in combination with immunosuppressive agents during the active disease period of the disease in clinical settings.