-
Modern Rheumatology Feb 2012IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4+...
BACKGROUND
IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4+ plasma cells. Although IgG4-RD is not rare and is clinically important, its clinical diagnostic criteria have not been established. Comprehensive diagnostic criteria for IgG4-RD, including the involvement of various organs, are intended for the practical use of general physicians and nonspecialists.
METHODS
Two IgG4-RD study groups, the Umehara and Okazaki teams, were organized by the Ministry of Health, Labor and Welfare Japan. As IgG4-RD comprises a wide variety of diseases, these groups consist of physicians and researchers in various disciplines, including rheumatology, hematology, gastroenterology, nephrology, pulmonology, ophthalmology, odontology, pathology, statistics, and basic and molecular immunology throughout Japan, with 66 and 56 members of the Umehara and Okazaki teams, respectively. Collaborations of the two study groups involved detailed analyses of clinical symptoms, laboratory results, and biopsy specimens of patients with IgG4-RD, resulting in the establishment of comprehensive diagnostic criteria for IgG4-RD.
RESULTS
Although many patients with IgG4-RD have lesions in several organs, either synchronously or metachronously, and the pathological features of each organ differ, consensus has been reached on two diagnostic criteria for IgG4RD: (1) serum IgG4 concentration >135 mg/dl, and (2) >40% of IgG+ plasma cells being IgG4+ and >10 cells/high powered field of biopsy sample. Although the comprehensive diagnostic criteria are not sufficiently sensitive for the diagnosis of type 1 IgG4-related autoimmune pancreatitis (IgG4-related AIP), they are adequately sensitive for IgG4-related Mikulicz's disease (MD) and kidney disease (KD). In addition, the comprehensive diagnostic criteria, combined with organ-specific diagnostic criteria, have increased the sensitivity of diagnosis to 100% for IgG4-related MD, KD, and AIP.
CONCLUSION
Our comprehensive diagnostic criteria for IgG4-RD are practically useful for general physicians and nonspecialists.
Topics: Autoimmune Diseases; Female; Humans; Immunoglobulin G; Interprofessional Relations; Male; Mikulicz' Disease; Nephritis, Interstitial; Pancreatitis; Plasma Cells
PubMed: 22218969
DOI: 10.1007/s10165-011-0571-z -
Canadian Medical Association Journal Apr 1948
Topics: Humans; Mikulicz' Disease
PubMed: 18916111
DOI: No ID Found -
European Journal of Ophthalmology 2006To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management... (Review)
Review
PURPOSE
To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature.
METHODS
Retrospective nonrandomized consecutive case series, Jules Stein Eye Institute, David Geffen School of Medicine at UCLA.
RESULTS
Mikulicz's disease is characterized by symmetric lacrimal, parotid, and submandibular gland enlargement with associated lymphocytic infiltrations. The authors noted two cases of Mikulicz's disease. The diagnosis of Mikulicz's disease was based on the following criteria: 1) symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular); and 2) the exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders.
CONCLUSIONS
Mikulicz's disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. The condition is self-limiting and most often, the diagnosis is a clinical one. Previously, Mikulicz's disease was often considered as a subtype of Sjögren's syndrome (SS). Clinical and immunologic differences between Mikulicz's disease and SS may warrant further consideration of Mikulicz's disease as a specific autoimmune phenomenon separate from SS, and Mikulicz's disease may be amenable to different treatment modalities than those employed in patients with SS.
Topics: Adult; Diagnosis, Differential; Humans; Lacrimal Apparatus; Magnetic Resonance Imaging; Male; Middle Aged; Mikulicz' Disease; Ophthalmologic Surgical Procedures; Retrospective Studies; Salivary Glands; Tomography, X-Ray Computed
PubMed: 16703534
DOI: 10.1177/112067210601600201 -
Auris, Nasus, Larynx Feb 2012Since Morgan's report in 1953, Mikulicz's disease (MD) has been considered part of primary Sjögren's syndrome (SS). However, MD has a unique presentation, including... (Review)
Review
Since Morgan's report in 1953, Mikulicz's disease (MD) has been considered part of primary Sjögren's syndrome (SS). However, MD has a unique presentation, including persistent swelling of the lacrimal and salivary glands, and is characterized by good responsiveness to glucocorticoids, leading to recovery of gland function. Recently, it has been revealed that MD patients show elevated serum immunoglobulin G4 (IgG4) levels and prominent infiltration of IgG4-positive plasmacytes. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Thus, MD is a systemic "IgG4-related disease." In addition, recent analyses have revealed that Küttner's tumor (KT), a chronic sclerosing sialadenitis that presents with asymmetrical firm swelling of the submandibular glands, is also associated with prominent infiltration of IgG4-positive plasmacytes. MD and KT differ from SS and are thought to be singular systemic IgG4-related plasmacytic diseases. Here we discuss the results of recent studies and provide an overview of MD as an IgG4-related disease.
Topics: Adult; Aged; Aged, 80 and over; Female; Glucocorticoids; History, 19th Century; History, 20th Century; Humans; Hypergammaglobulinemia; Immunoglobulin G; Lacrimal Apparatus; Male; Middle Aged; Mikulicz' Disease; Parotid Gland; Sialadenitis; Sjogren's Syndrome; Submandibular Gland
PubMed: 21571468
DOI: 10.1016/j.anl.2011.01.023 -
World Neurosurgery Jan 2018We report a case of Mikulicz disease (MD), an immunoglobulin G4 (IgG4)-related disease that affects the lacrimal and salivary glands. IgG4-related disease is a...
We report a case of Mikulicz disease (MD), an immunoglobulin G4 (IgG4)-related disease that affects the lacrimal and salivary glands. IgG4-related disease is a relatively new clinical entity and is not commonly encountered by neurosurgeons. MD sometimes mimics intraorbital tumors such as malignant lymphoma but responds well to corticosteroid treatment. Thus it is important to recognize the clinical and radiographic features of MD.
Topics: Aged; Anti-Inflammatory Agents; Diagnosis, Differential; Eye Neoplasms; Female; Humans; Immunoglobulin G; Lacrimal Apparatus; Magnetic Resonance Imaging; Mikulicz' Disease; Prednisolone; Treatment Outcome
PubMed: 28987847
DOI: 10.1016/j.wneu.2017.09.173 -
Modern Rheumatology 2006Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren's syndrome (SS), but it represents a unique condition involving persistent enlargement... (Review)
Review
Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren's syndrome (SS), but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands characterized by few autoimmune reactions and good responsiveness to glucocorticoids, leading to the recovery of gland function. Mikulicz's disease was recently reported to be associated with elevated immunoglobulin G4 (IgG4) concentrations in the serum and prominent infiltration of plasmacytes expressing IgG4 into the lacrimal and salivary glands. The following features were used for diagnosis: (1) visual confirmation of symmetrical and persistent swelling in more than two lacrimal and major salivary glands; (2) prominent mononuclear cell infiltration of lacrimal and salivary glands; and (3) exclusion of other diseases that present with glandular swelling, such as sarcoidosis and lymphoproliferative disease. These features are not observed in most SS cases. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Mikulicz's disease thus differs from SS and may be a systemic IgG4-related plasmacytic disease.
Topics: Adult; Autoimmune Diseases; Female; Glucocorticoids; History, 19th Century; History, 20th Century; Humans; Immunoglobulin G; Lacrimal Apparatus; Leukocytes, Mononuclear; Male; Middle Aged; Mikulicz' Disease; Plasma Cells; Rheumatology; Salivary Glands; Sjogren's Syndrome
PubMed: 17164992
DOI: 10.1007/s10165-006-0518-Y -
The Pan African Medical Journal 2020Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome; however,...
Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome; however, Mikulicz's disease is clinically characterized by infrequent autoimmune reactions and responsiveness to glucocorticoid treatment. The ultrasound features of the lacrimal and salivary glands in patients with IgG4-Mikulicz's disease were characterized by multiple hypoechoic areas of varying sizes within the enlarged glands. IgG4 serum level was also elevated, in contrast to the detection of normal levels in Sjogren's syndrome. In this article, we intended to illustrate a case of Mikulicz's disease with clinical and imaging features.
Topics: Adult; Diagnosis, Differential; Female; Humans; Immunoglobulin G; Lacrimal Apparatus; Mikulicz' Disease; Salivary Glands; Sjogren's Syndrome; Ultrasonography
PubMed: 33598067
DOI: 10.11604/pamj.2020.37.252.26835 -
The British Journal of Dermatology Nov 2014IgG4-related disease (IgG4-RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4(+) plasma... (Review)
Review
IgG4-related disease (IgG4-RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4(+) plasma cells. IgG4-RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and lacrimal glands. The skin lesions of IgG4-RD have been poorly characterized and may stem not only from direct infiltration of plasma cells but also from IgG4-mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of the limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek and mandible regions), (3) Mikulicz disease (palpebral swelling, sicca syndrome and exophthalmos), (4) psoriasis-like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura) and (7) ischaemic digit (Raynaud phenomenon and digital gangrene). It is considered that subtypes 1-3 are induced by direct infiltration of IgG4(+) plasma cells, while the other types (4-7) are caused by secondary mechanisms. IgG4-related skin disease is defined as IgG4(+) plasma-cell-infiltrating skin lesions that form plaques, nodules or tumours (types 1-3), but may manifest secondary lesions caused by IgG4(+) plasma cells and/or IgG4 (types 4-7).
Topics: Angiolymphoid Hyperplasia with Eosinophilia; Autoimmune Diseases; Erythema; Fingers; Humans; Immunoglobulin G; Ischemia; Mikulicz' Disease; Plasma Cells; Plasmacytoma; Pseudolymphoma; Psoriasis; Purpura, Hyperglobulinemic; Skin Diseases; Skin Diseases, Papulosquamous; Urticaria; Vasculitis
PubMed: 25065694
DOI: 10.1111/bjd.13296 -
IgG4-related diseases including Mikulicz's disease and sclerosing pancreatitis: diagnostic insights.The Journal of Rheumatology Jul 2010Since the first report of serum IgG4 elevation in sclerosing pancreatitis in 2001, various systemic disorders have been reported to elevate IgG4, and many names have... (Review)
Review
Since the first report of serum IgG4 elevation in sclerosing pancreatitis in 2001, various systemic disorders have been reported to elevate IgG4, and many names have been proposed from the perspective of the systemic condition. Despite similarities in the organs damaged in IgG4-related Mikulicz's disease and Sjögren's syndrome, there are marked clinical and pathological differences between the 2 entities. The majority of cases diagnosed with autoimmune pancreatitis in Japan are IgG4-related sclerosing pancreatitis, and it should be recognized that this is distinct from the Western type. Diagnosis of IgG4-related disease is defined by both elevated serum IgG4 (> 1.35 g/l) and histopathological features, including lymphocyte and IgG4+ plasma cell infiltration (IgG4+ plasma cells/IgG+ plasma cells > 50% on a highly magnified slide checked at 5 points). Differential diagnosis from other distinct disorders is necessary: these include sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, cancer, and other existing conditions. The Japanese IgG4 research group has begun multicenter prospective studies to improve diagnostic criteria and treatment strategies.
Topics: Diagnosis, Differential; Female; Humans; Immunoglobulin G; Japan; Male; Mikulicz' Disease; Multicenter Studies as Topic; Pancreatitis; Prospective Studies; Salivary Glands; Sclerosis; Sjogren's Syndrome
PubMed: 20436071
DOI: 10.3899/jrheum.091153 -
Transactions of the American... 1947
PubMed: 16693444
DOI: No ID Found