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Seminars in Diagnostic Pathology Nov 2012
Topics: Autoimmune Diseases; Biomarkers; Humans; Immunoglobulin G; Immunosuppressive Agents; Male; Mikulicz' Disease; Pancreatitis, Chronic; Practice Guidelines as Topic
PubMed: 23068295
DOI: 10.1053/j.semdp.2012.07.006 -
Archivos de La Sociedad Espanola de... Aug 2014We report the case of a 48 year-old male with bilateral involvement of the salivary and lacrimal glands, which was diagnosed as Mikulicz's disease by incisional biopsy...
CASE REPORT
We report the case of a 48 year-old male with bilateral involvement of the salivary and lacrimal glands, which was diagnosed as Mikulicz's disease by incisional biopsy of the affected lacrimal glands, which was completely resolved after corticosteroid treatment.
DISCUSSION
Sclerosing sialadenitis and/or dacryoadenitis are chronic inflammatory diseases that are currently classified into IgG4-related diseases. Specifically, Mikulicz's disease is defined by a persistent and symmetrical swelling of the lacrimal and salivary glands together with elevated serum concentration of IgG4 and tissue infiltration by IgG4 (+) plasma cells.
Topics: Humans; Male; Middle Aged; Mikulicz' Disease
PubMed: 24269424
DOI: 10.1016/j.oftal.2013.06.005 -
Clinical and Experimental Rheumatology 2013This paper aims to report a case of IgG4-related Mikulicz's disease with a systematic review. (Review)
Review
OBJECTIVES
This paper aims to report a case of IgG4-related Mikulicz's disease with a systematic review.
METHODS
The relevant English literature was searched using the keywords 'Mikulicz's disease' and 'IgG4'. Original and review articles were reviewed, and the clinical scenarios were exemplified with a case report.
RESULTS
A 49-year-old Caucasian man presented with axillary lymphadenopathy and bilateral parotid/submandibular enlargement. A chest computerized tomography showed mediastinal lymphadenopathy, with low metabolic activity on the position emission tomography. A histopathological study showed an IgG4/IgG ratio of 75% in the plasma cells of the submandibular glands, associated with high levels of total serum IgG and IgG4. He had dry mouth, but minor salivary gland biopsy was negative without xerophthalmia. He had nasal obstruction and dyspnea, notably with supine position/cervical rotation, which substantially improved with glucocorticoid treatment. He had newly diagnosed diabetes mellitus with hyperlipasaemia and diffuse pancreatic swelling supportive of autoimmune pancreatitis.
CONCLUSIONS
Our case report supports the literature that there are similarities between IgG4-related Mikulicz's disease and Sjögren's syndrome, but the differences are significant. IgG4-related Mikulicz's disease is a multi-organ lymphoproliferative disease distinct from Sjögren's syndrome.
Topics: Biomarkers; Biopsy; Diagnosis, Differential; Glucocorticoids; Humans; Immunoglobulin G; Lymphoproliferative Disorders; Male; Middle Aged; Mikulicz' Disease; Positron-Emission Tomography; Predictive Value of Tests; Salivary Glands; Sialography; Sjogren's Syndrome; Tomography, X-Ray Computed; Treatment Outcome; White People
PubMed: 23406780
DOI: No ID Found -
Internal Medicine (Tokyo, Japan) 2010
Topics: Humans; Immunoglobulin G; Mikulicz' Disease; Ribonucleosides; Treatment Outcome
PubMed: 20647641
DOI: 10.2169/internalmedicine.49.3692 -
Autoimmunity Reviews Apr 2005Mikulicz's disease (MD) has been considered part of primary Sjogren's syndrome (SS) since Morgan's report in 1953. MD represents a unique condition involving enlargement... (Review)
Review
Mikulicz's disease (MD) has been considered part of primary Sjogren's syndrome (SS) since Morgan's report in 1953. MD represents a unique condition involving enlargement of the lacrimal and salivary glands, as is also seen in SS; however, MD is characterized by few autoimmune reaction and its good responsiveness to glucocorticoid. Recent reports have shown that the frequency of apoptosis in glands of MD patients is lower when compared with SS. This phenomenon reflects the histologically reversible gland secretion in MD. Elevated IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the lacrimal and salivary glands have also been confirmed in MD. Plasma cells expressing IgG4 are also detected in lymph nodes and bone marrow. MD may be a systemic disease, rather than a lacrimal and salivary gland disease. We here propose the new entity "IgG4-related plasmacytic exocrinopathy" and expect future development with regard to its relationship with autoimmune pancreatitis, which similarly presents elevated serum IgG4 levels.
Topics: Humans; Immunoglobulin G; Mikulicz' Disease; Salivary Glands, Minor; Sjogren's Syndrome
PubMed: 15893711
DOI: 10.1016/j.autrev.2004.10.005 -
Southern Medical Journal Apr 1976The pathogenesis, clinical manifestations, and historical development of Mikulicz's disease and Mikulicz's syndrome are given. It is recommended that the term...
The pathogenesis, clinical manifestations, and historical development of Mikulicz's disease and Mikulicz's syndrome are given. It is recommended that the term "Mikulicz's syndrome" be dropped to avoid confusion with the rare and well defined entity "Mikulicz's disease."
Topics: Diagnosis, Differential; Humans; Lacrimal Apparatus Diseases; Mikulicz' Disease; Salivary Gland Diseases; Syndrome; Terminology as Topic
PubMed: 1265507
DOI: 10.1097/00007611-197604000-00023 -
British Medical Journal Aug 1949
Topics: Humans; Mikulicz' Disease
PubMed: 18138450
DOI: 10.1136/bmj.2.4624.416 -
Proceedings of the Royal Society of... Feb 1948
Topics: Humans; Mikulicz' Disease
PubMed: 18903021
DOI: No ID Found -
Japanese Journal of Ophthalmology Jan 2015Immunoglobulin G4 (IgG4)-related disease is a novel clinical entity characterized by infiltration of IgG4-immunopositive plasmacytes and elevated serum IgG4... (Review)
Review
Immunoglobulin G4 (IgG4)-related disease is a novel clinical entity characterized by infiltration of IgG4-immunopositive plasmacytes and elevated serum IgG4 concentration accompanied by enlargement of and masses in various organs, including the lacrimal gland, salivary gland, and pancreas. Recent studies have clarified that conditions previously diagnosed as Mikulicz disease as well as various types of lymphoplasmacytic infiltrative disorders of the ocular adnexa are consistent with a diagnosis of IgG4-related disease. Against this background, the diagnostic criteria for IgG4-related ophthalmic disease have recently been established, based on both the clinical and the histopathologic features of the ocular lesions. This article reviews these new criteria with reference to the comprehensive diagnostic criteria for IgG4-related disease for all systemic conditions reported in 2012.
Topics: Humans; Immunoglobulin G; Lacrimal Apparatus Diseases; Mikulicz' Disease; Orbital Pseudotumor; Plasma Cells
PubMed: 25392273
DOI: 10.1007/s10384-014-0352-2 -
BMC Geriatrics Jun 2024IgG4-related diseases are very uncommon, and its diagnosis and treatment are complicated as it encompasses multiple disciplines.
BACKGROUND
IgG4-related diseases are very uncommon, and its diagnosis and treatment are complicated as it encompasses multiple disciplines.
CASE PRESENTATION
A 77-year-old woman was admitted with a jaw mass and nausea and vomiting. Laboratory tests showed elevated serum IgG4, pituitary MRI suggested thickening of the pituitary stalk, and head and neck CT suggested orbital and mandibular masses. Patients with mandibular mass were diagnosed with Mikulicz's disease with IgG4-related hypophysitis. We found no other evidence of causing thickening of the pituitary stalk. She was given oral prednisolone 30 mg daily, and her nausea and vomiting improved significantly, and the mandibular and ocular masses decreased in size.
CONCLUSION
Mikulicz's disease combined with IgG4-related hypophysitis is a rare case of IgG4-RD in elderly women. IgG4-RD is one of the causes of head and neck exocrine gland mass and pituitary stalk thickening in the elderly.
Topics: Humans; Aged; Female; Mikulicz' Disease; Immunoglobulin G4-Related Disease; Autoimmune Hypophysitis; Immunoglobulin G; Prednisolone; Magnetic Resonance Imaging
PubMed: 38880897
DOI: 10.1186/s12877-024-05142-7