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IgG4 Syndrome

IgG4-Related Disease


Presentation

  • We report a case of IgG4-related disease presenting a soft tissue tumor affecting skeletal muscle. Case Report: A 32 - year - old man presented a soft tissue mass in his left arm.[scirp.org]
  • Haley VA Medical Center, Tampa, FL, USA This poster was presented at USF Research Day 2017 Poster Views: 1,050 View poster » Abstract: IgG4-RD is a rare syndrome with various presentations including eosinophilia, elevated serum IgG4, organ swelling and[eposters.net]
  • Here we present a case of IgG4 related disease in a 50 year old female, who initially presented with low grade fever, sicca symptoms, eyelid swelling with proptosis along with bilateral salivary gland enlargement.[casereports.in]
  • Presented at Annual European Congress of Rheumatology (EULAR 2017); Madrid, Spain, June 14-17, 2017. Abstract FR10588.[raredr.com]
  • These activated B cells and plasmablasts present antigen to CD4 cytotoxic T cells at sites of disease. Dr.[mdedge.com]
Mediastinal Lymphadenopathy
  • Extrapancreatic manifestations include: abdominal or mediastinal lymphadenopathy; the involvement of salivary glands and lacrimal glands, kidneys, lung, retroperitoneum.[eurekaselect.com]
  • We repeated the thoracic CT scan and confirmed the previously described pulmonary nodular images and mediastinal lymphadenopathies.[revistanefrologia.com]
Weight Gain
  • Prednisone has proven very effective in treating this condition but also leads to side effects such as weight gain, osteoporosis, high blood pressure and diabetes.[massgeneral.org]
Intravenous Administration
  • Intravenous administration of XmAb5871 (5 mg/kg once every 14 days) in patients with active IgG4-RD has been well tolerated.[raredr.com]
Soft Tissue Mass
  • Case Report: A 32 - year - old man presented a soft tissue mass in his left arm. Magnetic resonance imaging revealed a spindle like, peripheral mass, in the lateral head of the triceps of his left arm.[scirp.org]
Constitutional Symptom
  • This patient had proptosis with enlargement of both submandibular and sublingual glands, sicca symptoms and constitutional symptoms.[casereports.in]
Pharyngitis
  • "Reply to "IgG4-related pharyngitis-an addition to the nomenclature of IgG4-related disease: comment on the article by Stone et al. " ". Arthritis & Rheumatism. 65 (8): 2217–2218. doi : 10.1002/art.37998. PMID 23677887.[en.wikipedia.org]
Muscle Weakness
  • There was no extraoccular muscle weakness, restriction in movement of eyeball or any bruit. Visual acuity was normal bilaterally.[casereports.in]
  • Physical examination showed generalized muscle weakness. With primary diagnoses of sinusitis and ear infection, some paraclinical evaluations were requested.[archivesofrheumatology.org]
Subcutaneous Nodule
  • Cutaneous manifestations include erythematous plaques , papules , and subcutaneous nodules , often itchy, and often on the face or forearm. Most have inflammatory enlargement of lacrimal or salivary glands at some point.[dermnetnz.org]
Testicular Pain
  • pain [30] Individual organ manifestations [ edit ] IgG4-RD can involve one or multiple sites in the body.[en.wikipedia.org]

Workup

  • Workup IgG4-related disease is diagnosed on the basis of clinical, biochemical and histopathological findings. It is essential to exclude differential diagnoses, some of which are shown in box 2.[tidsskriftet.no]
Hyperviscosity
  • In rare cases, IgG4 related disease may be associated with hyperviscosity with polyclonal IgG4 predominant hypergammaglobulinemia [ 6 ].[avensonline.org]

Treatment

  • Treatment was started with steroids and azathioprine without relapses after 12 months follow-up.[ncbi.nlm.nih.gov]
  • Option Treatment for IgG4-RSD traditionally has involved prednisone, a steroid medication.[massgeneral.org]
  • It is also being developed for treatment of systemic lupus erythematosus. Dr.[mdedge.com]
  • The consensus statement offers clinicians seven key statements to help guide diagnosis and treatment of IgG4-related disease (see Table 1).[the-rheumatologist.org]
  • We have begun a clinical prospective study to establish a treatment strategy (Phase II prospective treatment study for IgG4-multiorgan lymphoproliferative syndrome: UMIN R000002311).[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis of IgG4-related disease is variable. It may spontaneously resolve or persist, with remitting and relapsing symptoms.[dermnetnz.org]
  • The question about the diagnostic value of limphoplazmatsitarny infiltrate detected in the salivary gland biopsy materials SD and its influence on the prognosis and course of pathological process remains open.[medlit.ru]
  • The reported cases of infantile Cronkhite-Canada syndrome are scant ( 10). [15] Prognosis Cronkhite-Canada syndrome (CCS) is considered a relentlessly progressive disease with a variable course and poor prognosis depending mainly on control of protein[emedicine.medscape.com]
  • Prognosis and outcome of non-Hodgkin lymphoma in primary Sjögren syndrome. Medicine (Baltimore) 2012; 91 :1–9 [ PubMed ] [ Google Scholar ] 7. Skopouli FN, Dafni U, Ioannidis JP, et al..[ncbi.nlm.nih.gov]

Etiology

  • Abstract: Background: IgG4-related disease is a rare, clinical and pathologic disease entity of unknown etiology.[eurekaselect.com]
  • The etiology of IgG4-RD is unknown and requires a tissue biopsy typically showing extensive lymphocyte and plasma cells infiltration, IgG4 immunoblasts, storiform fibrosis (fibrosis in a cartwheel distribution), eosinophil tissue infiltration and obliterative[eposters.net]
  • The underlying etiology is not known.[ajnrdigest.org]
  • IgG4 related disease is a syndrome of unknown etiology most often occurring in middle-aged and older men. Two major presentations of this condition are type 1 autoimmune pancreatitis and sclerosing sialadenitis.[clinlabnavigator.com]
  • IgG subclass 4-related disease is a recently recognized syndrome of unknown etiology most often occurring in middle-aged and older men.[mayomedicallaboratories.com]

Epidemiology

  • Clinical and epidemiological research IgG4 immune response in Churg–Strauss syndrome Augusto Vaglio 1 , Johanna D Strehl 2 , Bernhard Manger 3 , Federica Maritati 1 , Federico Alberici 1 , Christian Beyer 3 , Jürgen Rech 3 , Renato A Sinico 4 , Francesco[ard.bmj.com]
  • Almost all the IgG-positive plasma cells are also positive for IgG4 (brown colour) The epidemiology has been only partly determined, but prevalence is estimated to be 0.28 – 1.08 cases per 100 000 ( 2 ).[tidsskriftet.no]
  • Sarcoidosis: "systemic disease in which diverse organ manifestations are linked by the same histopathological considerations" (Stone 2012) Spectrum of IgG4-related disease with terms replaced by IgG4-RD Mickulicz's disease Kuttner's tumor Riedel's thyroiditis Epidemiology[medicine.uiowa.edu]
  • [ edit ] As recognition of IgG4-RD is relatively recent, there are limited studies on its epidemiology.[en.wikipedia.org]
  • Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan. Nippon Geka Hokan. 1995;64:3-14. Sweetser S, Alexander GL, Boardman LA. A case of Cronkhite-Canada syndrome presenting with adenomatous and inflammatory colon polyps.[rarediseases.org]
Sex distribution
Age distribution

Pathophysiology

  • The aim of this review was to describe the clinical, serological, histopathological and pathophysiological aspects of IgG4‐RS in the context of IgG4‐RD and highlight the differences between IgG4‐RS and SS.[ingentaconnect.com]
  • The success with rituximab is just one example of how improved understanding of the pathophysiology of IgG4-RD has opened the door to novel treatments. Dr.[mdedge.com]
  • “We now have a fairly coherent understanding of the pathophysiology of this disease. I actually think there are not too many rheumatologic conditions that we understand as well as we understand IgG4-related disease,” said Dr.[mdedge.com]
  • […] conditions confined to single organs are now part of the spectrum of IgG4-related disease like Kuttner’s tumour, Riedel’s thyroiditis and Mikulicz’s syndrome depending on the key pathological feature present and perhaps this provides more insight into the pathophysiology[casereports.in]
  • Useful For Suggests clinical disorders or settings where the test may be helpful Supporting the diagnosis of IgG4-related disease Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory[mayomedicallaboratories.com]

Prevention

  • The treatment of choice is glucocorticoids and it could be needed to add another immuno-suppressant agent as steroid sparing and to prevent relapses.[ncbi.nlm.nih.gov]
  • If IgG4-RSD is diagnosed early, we can usually control the inflammation within organs and prevent further damage.[massgeneral.org]
  • Serum immunoglobulin G4 is often elevated but this is not always the case. [ citation needed ] Treatment [ edit ] The goal of treatment is the induction and maintenance of remission so as to prevent progression of fibrosis and organ destruction in affected[en.wikipedia.org]
  • […] various clinical presentations based on involved organ such as sclerosing pancreatitis, sclerosing cholangitis, prostatitis, tubulointerstitial nephritis, interstitial pneumonia, and enlargement of salivary glands.( 3, 4 ) Early diagnosis is important to prevent[archivesofrheumatology.org]
  • Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs[emedicine.medscape.com]

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