Elevated IgG4 concentrations in serum of patients with Mikulicz's disease

Abstract

Mikulicz's disease has recently been included within primary Sjögren's syndrome. It is a unique condition involving enlargement of the lacrimal and salivary glands, characterized by few autoimmune reactions. It is responsive to glucocorticoid treatment. Analysis of IgG fractions was performed in patients with Mikulicz's disease in order to determine the differences between Mikulicz's disease and Sjögren's syndrome. The study showed that serum IgG4 concentrations are elevated in patients with Mikulicz's disease, but not in those with Sjögren's syndrome.

A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details

“…Since elevated serum IgG4 was first reported in patients with MD [6], the members of the Japanese Society of Sjögren’s Syndrome have assessed the clinical symptoms, laboratory findings, and detailed histopathology in patients with MD (characterized by symmetrical swelling of the lachrymal, submandibular, and parotid glands), nationwide, since 2004. Some patients did not show typical symptoms of MD such as swelling of the lachrymal, parotid, or submandibular glands but showed elevated serum IgG4 and other indices indicative of MD according to the criteria for the diagnosis of IgG4-related MD shown in Table 2 [8].…”

Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 II. Extrapancreatic lesions, differential diagnosis

“…1) [12,26,27,33]. These lesions, which were previously thought to be a complication of Sjögren's syndrome, are now thought to be related to Mikulicz's disease or Küttner's tumor (chronic sclerosing sialadenitis) [34,35]. Useful findings for differentiating among these possibilities include the following: (1) compared to those of Sjögren's syndrome, AIPassociated lachrymal and salivary gland lesions show normal or slightly impaired exocrine function, presenting as a slight or negligible dryness in the eyes and mouth [33,36]; (2) salivary gland lesions associated with AIP appear predominantly in the submandibular gland [31], and those associated with Sjögren's syndrome frequently appear in the parotid gland [37]; (3) lachrymal and salivary gland lesions associated with AIP show negative results in tests for SS-A/Ro and SS-B/La autoantibodies [35]; (4) lachrymal and salivary gland lesions associated with AIP show numerous IgG4-positive plasma cell infiltrations in the affected tissues [35]; and (5) lachrymal and salivary gland lesions associated with AIP respond favorably to corticosteroid therapy [35].…”

IgG4-associated Multifocal Systemic Fibrosis Complicating Sclerosing Sialadenitis, Hypophysitis, and Retroperitoneal Fibrosis, but Lacking Pancreatic Involvement

“…Furthermore, these conditions of sclerosing sialadenitis were reported to be closely associated with high serum IgG4 concentration and IgG4 bearing plasma cell infiltration (7,8) …”