BACKGROUND/AIMS: The objective of this study was to compare the clinical characteristics of patients with autoimmune pancreatitis (AIP) with or without Mikulicz's disease (MD) and with MD alone. METHODS: We investigated the clinical findings in 15 AIP patients with MD (group A+M), 49 AIP only patients (group A), and 14 MD only patients (group M). RESULTS: The male-female ratio was significantly higher in group A+M (73%, p<0.05) and group A (78%, p<0.01) than group M (21%). Serum immunoglobulin G (IgG) levels were significantly higher in group A+M than in group A (p<0.01) and group M (p<0.05). Serum IgG4 levels were significantly higher in group A+M than in group A (p<0.01). Other organ involvement was observed in 73% (11/15) of patients in group A+M. The number of patients with diabetes mellitus was significantly higher in group A+M (66%, p<0.01) and group A (51%, p<0.05) than in group M (7%). All of the patients responded well to steroid therapy, but the relapse rate in group A+M (33%) was significantly higher than that in group A (3%, p<0.01). Salivary gland function was impaired in all groups compared with the control group, but the degree of dysfunction was less in group A compared with group A+M and group M. CONCLUSIONS: The relapse rate of AIP in MD patients was significantly higher than that of AIP in patients without MD.
Diabetes Mellitus ; Humans ; Immunoglobulin G ; Mikulicz' Disease ; Pancreatitis ; Recurrence ; Salivary Glands

Chronic sclerosing sialadenitis, Mikulicz disease or Kuttner tumor has been recently recognized as a spectrum of IgG4-related sclerosing disease. IgG4-related disease is characterized by a high serum IgG4 level and tissue infiltration of IgG4-positive plasmacytes. We report three cases of chronic sclerosing sialadenitis with variably associated systemic involvement. All patients presented with a submandibular mass or swelling, and all the resected submandibular glands showed diffuse lymphocytic infiltration, lymphoid follicles, and septal fibrosis. Two of the specimens revealed numerous IgG-positive plasma cells, most of which were IgG4-positive on immunohistochemical staining. One of them was associated with dacryoadenitis and hypophysitis. The other patient had ureterorenal lesions. Immunohistochemical study was unavailable in remaining one case, but the histologic features along with elevated IgG level and associated pancreatitis supported the diagnosis. All patients received steroid therapy postoperatively and are doing well. Salivary gland involvement in IgG4-related fibrosclerosis should be recognized in systemic medical pathology.
Dacryocystitis ; Fibrosis ; Humans ; Immunoglobulin G ; Mikulicz' Disease ; Pancreatitis ; Plasma Cells ; Salivary Glands ; Sialadenitis ; Submandibular Gland

Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Mikulicz' Disease ; Salivary Gland Diseases

Diagnosis, Differential ; Head ; Humans ; Immunoglobulin G ; Mikulicz' Disease/diagnosis* ; Submandibular Gland

PURPOSE: To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a. CASE SUMMARY: A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland's posterior side and the lateral rectus muscle's lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.
Biopsy ; Dacryocystitis ; Exophthalmos ; Eye ; Humans ; Immunochemistry ; Immunoglobulin G ; Lymphoma ; Magnetic Resonance Imaging ; Middle Aged ; Mikulicz' Disease ; Muscles ; Optic Nerve ; Steroids ; Visual Acuity

PURPOSE: To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a. CASE SUMMARY: A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland's posterior side and the lateral rectus muscle's lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.
Biopsy ; Dacryocystitis ; Exophthalmos ; Eye ; Humans ; Immunochemistry ; Immunoglobulin G ; Lymphoma ; Magnetic Resonance Imaging ; Middle Aged ; Mikulicz' Disease ; Muscles ; Optic Nerve ; Steroids ; Visual Acuity

Immunoglobulin G4 (IgG4)-related disease is a newly named fibroinflammatory condition characterized by tumefactive lesions that contain dense lymphoplasmacytic infiltrates rich in IgG4-positive cells. Elevated serum IgG4 concentrations also often accompany IgG4-related disease. Many medical conditions that were long viewed as diseases confined to single organs (e.g., Mikulicz disease, type 1 autoimmune pancreatitis, Riedel's thyroiditis, multifocal fibrosclerosis, inflammatory pseudotumor, mediastinal fibrosis, retroperitoneal fibrosis, and etc.) are now designated as part of the spectrum of IgG4-related disease. The preferred nomenclature suggested by a committee of international experts uses the prefix "IgG4-related-" for individual organ involvement, regardless of the organ system affected. One exception is type 1 autoimmune pancreatitis (IgG4-related pancreatitis). Comprehensive diagnostic criteria for IgG4-related disease and organ-specific diagnostic criteria (e.g., IgG4-related dacryoadenitis and sialadenitis, type 1 autoimmune pancreatitis, IgG4-related kidney disease and IgG4-related sclerosing cholangitis) can aid clinicians in the diagnosis of this erratic condition.
Autoimmune Diseases ; Dacryocystitis ; Diagnosis ; Fibrosis ; Granuloma, Plasma Cell ; Immunoglobulin G ; Immunoglobulins* ; Kidney Diseases ; Mikulicz' Disease ; Pancreatitis ; Retroperitoneal Fibrosis ; Sialadenitis ; Thyroid Gland ; Thyroiditis

Aged, 80 and over ; Autoimmune Diseases ; blood ; diagnosis ; Humans ; Immunoglobulin G ; blood ; Lymphatic Diseases ; blood ; diagnosis ; Male ; Mikulicz' Disease ; blood ; diagnosis ; Pancreatitis ; blood ; diagnosis

BACKGROUND: Immunoglobulin G4 (IgG4)-related sclerosing disease is a recently described clinicopathological entity with diverse manifestations including, amongst others, autoimmune pancreatitis, sclerosing cholangitis, sclerosing sialadenitis and retroperitoneal fibrosis. Elevated serum IgG4 concentration has been described as the hallmark of this condition with reported good sensitivity and specificity. OBJECTIVE: We sought to establish the utility of serum IgG4 concentrations in the diagnosis of IgG4-related sclerosing disease by determining how many serum samples with elevated IgG4 from an unselected population would originate from patients who fulfilled criteria for this diagnosis. METHODS: The clinical features and laboratory characteristics of patients whose serum IgG4 concentration was greater than 1.30 g/L were analysed retrospectively from a total of 1,258 IgG subclass measurements performed in a tertiary hospital diagnostic laboratory. RESULTS: Eighty patients (6.4%) had elevated IgG4 concentrations greater than 1.30 g/L. Nine of 61 patients had the diagnosis of IgG4-related sclerosing disease, giving a poor positive predictive value of 15%. The median serum IgG4 concentrations of those with and without IgG4-related sclerosing disease were 2.16 g/L and 1.86 g/L, respectively (p = 0.22). CONCLUSION: Serum IgG4 concentration has poor positive predictive value in the diagnosis of IgG4-related sclerosing disease and, therefore, the clinical significance of elevated serum IgG4 concentration alone must be interpreted with caution.
Autoimmune Diseases ; Cholangitis, Sclerosing ; Diagnosis ; Humans ; Immunoglobulin G ; Immunoglobulins ; Mikulicz' Disease ; Pancreatitis ; Retroperitoneal Fibrosis ; Retrospective Studies ; Sensitivity and Specificity ; Sialadenitis ; Tertiary Care Centers