ObjectiveTo learn the serum immunoglobulin G4 (IgG4) level in patients with pemphigus vulgaris (PV) in the active phase by detecting the IgG4 concentration in the patients with PV. MethodsWe collected the serum samples from patients with active-phase PV (including acute-onset and chronic active phases) between April and December 2014. The serum IgG4 level was detected by immuno-scatter turbidmetry, and the difference of serum IgG4 level between PV patients and normal subjects was assessed by Student's t-test. ResultsThirty-two patients with pemphigus vulgaris in the active phase were enrolled for this study, among whom 22 (including 8 males and 14 females) had normal serum IgG4 level, 8 (including 3 males and 5 females) higher and 2 (including 1 male and 1 female) lower. Compared with normal subjects, the average level of serum IgG4 in PV patients was not significantly different (P>0.05). The serum IgG4 level of acute-onset and chronic active PV patients was (1.015±0.825) g/L and (1.058±1.133) g/L, respectively, with no significant difference (P>0.05). ConclusionThe serum IgG4 level of patients with pemphigus vulgaris in the active phase can be normal, higher or lower. And there is no obvious difference in the serum IgG4 level between acute-onset and chronic active PV patients.
Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity recognized at the start of this century noted to be involving many organ systems including endocrine system and thyroid in particular. It represents an immune mediated fibro inflammatory condition with characteristic histopathological appearance affecting single or multiple organs. In general, immunoglobulin G4-related thyroid disease (IgG4-RTD) is rarely considered and it may be isolated or with other organ involvement. Four subcategories of IgG4-RTD have been identified so far: Riedel thyroiditis, fibrosing variant of Hashimoto thyroiditis, immunoglobulin G4-related Hashimoto thyroiditis, and Graves disease with elevated immunoglobulin G4 levels. The diagnostic approach is complex and the work up relies on the coexistence of clinical features, histological features and serological evidence. Demonstration of the classic histopathological features is vital to diagnose IgG4-RD in most cases, and biopsy proof is preferred strongly by most disease experts before the initiation of treatment. The treatments for IgG4-RTD include medical and surgical options. Steroids are first line treatment though it may need further evaluation. Tamoxifen and rituximab are second line treatment for steroid resistant patients. Surgical excision of thyroid gland in presence of compression symptoms is the surgical option. Inspite of pathophysiology of the disease being poorly understood till now, early and prompt diagnosis and an early treatment initiation can improve the outcomes.
IgG4-related disease (IgG4-RD) is a chronic, immune-mediated inflammatory and fibrotic condition that can affect virtually any organ system. While some patients experience involvement of only a single organ, isolated IgG4-RD of the mediastinum is even rarer. This article reports a case of a 48-year-old male patient with isolated IgG4-RD of the middle mediastinum. After a biopsy failed to establish a definitive diagnosis, the patient underwent a right thoracotomy-assisted mediastinal tumor resection via video-assisted thoracoscopic surgery. The tumor was completely resected during the procedure, which lasted 130 minutes, with an estimated blood loss of approximately 50 mL. The patient had a favorable postoperative course and satisfactory clinical outcome. No glucocorticoid or other medical treatment was administered postoperatively, and there was no recurrence during a 6-month follow-up period.