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IgG4-Related Dacryoadenitis and Sialadenitis

Mikulicz Disease


Presentation

  • In the present study, keratoconjunctivitis sicca was present in only half of all patients with MD, and the symptoms were very mild. The sialography in patients with MD did not show the apple-tree sign in any.[doi.org]
  • 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[ncbi.nlm.nih.gov]
  • In the present study, we used immunohistological techniques to compare the roles of cytotoxic T lymphocytes (CTLs) in MD and primary Sjogren's syndrome (SS).[ncbi.nlm.nih.gov]
  • It is our ambition to present a complete survey of all medical phenomena named for a person, with a biography of that person. Disclaimer: Whonamedit? does not give medical advice.[whonamedit.com]
  • Patient 3 was a 33-year-old Filipino male with a history of hemimandibulectomy for ameloblastoma in 1989 who presented with painless enlargement of the lacrimal and parotid glands for 18 months.[acrabstracts.org]
Fatigue
  • A third and poorly understood form of fatigue is called “chronic fatigue syndrome.” In recent years, there has been a great deal of research into the neurochemistry of depression and chronic fatigue.[dry.org]
  • Fatigue and weight loss of 5-10 kg can occur over months, whereas patients with IgG4-related disease rarely present with fever [ 3 , 8 ].[kjpbt.org]
Dysphagia
  • [excludes (diagnosed) Sjögren's syndrome] 527.1 Hypertrophy of salivary glands 527.7 Disturbance of salivary secretion (Xerostomia) 719.4x Pain in joint (requires fifth digit for site) 780.7 Malaise and fatigue 785.6 Enlargement of lymph nodes 797.2 Dysphagia[dry.org]
Parotid Swelling
  • Case 2 – A 28-year-old female referred to our clinic with bilateral proptosis and parotid swelling from three months ago. She had a history of bilateral parotid swelling from six months ago, as well as chronic sinusitis and mild asthma.[archivesofrheumatology.org]
Eczema
  • Another common presentation involves atopic disease – asthma, allergic rhinitis, eczema, eosinophilia, nasal polyps – developing out of the blue in middle age or later life.[mdedge.com]
Blister
  • Neonatal pemphigus vulgaris: IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborns. J Am Acad Dermatol 2003 ; 48 : 623 –5. 24 Warren SJ, Arteaga LA, Rivitti EA et al.[doi.org]
Absent Lacrimation
  • - A dictionary of medical eponyms Related people Henrik Samuel Conrad Sjögren Jan Mikulicz-Radecki Benign and chronic dacryoadenitis with bilateral painless swelling of lacrimal and salivary glands and decreased or absent lacrimation.[whonamedit.com]

Workup

  • The diagnosis agreed upon after the analysis of clinical findings and workup was IgG4-RD with extensive renal, lymph node, and pancreatic involvement. The patient was started on prednisone 60 mg/day.[scielo.br]
Anergy
  • CONCLUSIONS: Cytotoxic granule-positive CTLs were in the minority in D salivary glands, and this regulation might relate to PD-1 signals like the state of exhaustion and anergy.[ncbi.nlm.nih.gov]
  • Cytotoxic granule-positive CTLs were in the minority in MD salivary glands, and this regulation might relate to PD-1 signals like the state of exhaustion and anergy.[biomedsearch.com]
  • .: The Anergy Induction of M3 Muscarinic Acetylcholine Receptor-Reactive CD4 T Cells Suppresses Experimental Sialadenitis-like Sjogren's Syndrome.[md.tsukuba.ac.jp]
Candida
  • Painful mouth lesions can result from Candida (yeast) infections of the lips (angular cheilitis) that are more frequent in dry mouth patients.[dry.org]

Treatment

  • Half of the cases experienced relapses within 7 years of initial treatment. CONCLUSION: We analyzed the clinical features and treatments of IgG4-DS using SMART, providing useful information for everyday clinical practice.[ncbi.nlm.nih.gov]
  • 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[ncbi.nlm.nih.gov]
  • (a) Serum concentrations of both IgG1 and IgG4 from patients with MD decreased after treatment.[doi.org]
  • Abstract We describe a 64-year-old woman with chronic sclerosing sialadenitis and dacryoadenitis, which developed during treatment for cervical lymph node tuberculosis.[infona.pl]
  • Anti-tuberculosis treatment did not improve the swelling in the lacrimal and submandibular glands, and a biopsy specimen of the lacrimal gland showed inflammation, with abundant lymphoid follicles with fibrosis and granuloma without caseous necrosis.[ryumachi-jp.com]

Prognosis

  • Prognosis is usually good; rarely this condition may devolve into lymphoma, or could actually represent 'occult' lymphoma from the outset.[en.wikipedia.org]
  • METHODS: Clinical features, laboratory findings, radiological findings, associated diseases, treatment, and prognosis were prospectively examined in 12 patients (seven men, five women; mean age, 60.9   15.1 years) with IgG4-related dacryoadenitis.[ncbi.nlm.nih.gov]
  • Early diagnosis and treatment improve the prognosis of affected patients. References 1 Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539-51.[scielo.br]
  • Prognosis The prognosis of IgG4-RD is not yet fully established. This is probably due to it being a new entity in the world of medicine.[omjournal.org]

Etiology

  • Schaffer and Jacobsen in 1927 reviewed the subject comprehensively and offered an etiologic classification. Sjögren's syndrome is entered as a separate entity under Henrik Samuel Conrad Sjögren, Swedish ophthalmologist, 1899-1986.[whonamedit.com]
  • This case suggests that an abnormal immunological reaction to tuberculosis may be one of the etiological candidates for IgG4-related disease. Authors User assignment Assign yourself or invite other person as author.[infona.pl]
  • This case suggests that an abnormal immunological reaction to tuberculosis may be one of the etiological candidates for IgG4-related disease.[ryumachi-jp.com]
  • The etiology and pathogenesis are not well understood; however, the role of regulatory T cells (Treg) has been suggested. We evaluated the phenotype and function of Tregs in IgG4-RD patients with lacrimal and salivary gland involvement.[acrabstracts.org]
  • Further study is necessary to conclude whether these features reflect different manifestations of a single disease entity or suggest different underlying etiologic factors.[insights.ovid.com]

Epidemiology

  • Immunosurveillance and cancer: epidemiological evidence. Br Med J. 1970 Nov 14; 4 (5732):420–422. [ PMC free article ] [ PubMed ] [ Google Scholar ] GODWIN JT.[ncbi.nlm.nih.gov]
  • Written by experts in their field, Crohn’s Disease and Ulcerative Colitis: From Epidemiology and Immunobiology to a Rational Diagnostic and Therapeutic Approach, Second Edition is of great value to gastroenterologists, surgeons, internists, pediatricians[books.google.es]
  • […] and pathogenesis of autoimmune pancreatitis” Chaired by Kazuichi Okazaki (Kansai Medical University) and Myung-Hwan Kim (Asan Medical Center) Part 1 1) A nationwide epidemiological survey of autoimmune pancreatitis in Japan (15 min) Atsushi Kanno (Tohoku[shinshu-u.ac.jp]
  • Clinical and epidemiological research Lesional CD4 IFN-γ cytotoxic T lymphocytes in IgG4-related dacryoadenitis and sialoadenitis Takashi Maehara 1 , 2 , Hamid Mattoo 1 , Miho Ohta 2 , Vinay S Mahajan 1 , Masafumi Moriyama 2 , 3 , Masaki Yamauchi 2 ,[ard.bmj.com]
  • Epidemiology There is a little publication on epidemiology of IgG4-ROD mainly because of lacking awareness of IgG4-ROD before 2007. [11] By pooling 95 cases from seven studies of IgG4-ROD from Japan, [9] , [12] , [13] , [14] Korea, [15] America, [16][e-tjo.org]
Sex distribution
Age distribution

Pathophysiology

  • 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.[ncbi.nlm.nih.gov]
  • To determine the pathophysiologic features of IgG4-RD, we examined T follicular helper (Tfh) cells in lesions and blood from patients with IgG4-RD.[jimmunol.org]
  • Keywords: IgG4, autoimmune pancreatitis, cytokine, regulatory T cells, pathology, Systemic lupus erythematosus, Hypophysitis, Chronic sclerosing dacryoadenitis, Chronic sclerosing sialadenitis, Inflammatory pseudotumor, Sclerosing cholangitis Title: Pathophysiology[eurekaselect.com]

Prevention

  • Additional studies are required to compare different strategies to prevent and treat IgG4-RD and maintain disease remission.[academic.oup.com]
  • Also, therapies are directed at improving symptoms, preventing the complications (such as dental caries, oral candida, or corneal damage) and preventing disease progression. A.[dry.org]
  • Fc-Fc binding could potentially prevent immune responses by blocking interactions with complement proteins and Fc receptors.[kjpbt.org]
  • Blind biopsies of parotid masses should be avoided to prevent injury to the facial nerve and avoid seeding of malignant cells. Diagnosis was established in all from surgical excision.[musculoskeletalkey.com]

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