The Autoimmune Registry
Immunoglobulin G4 related disease Graphs for this disease are generated based on data from the Autoimmune Registry Inc.
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  • Names
    • Immunoglobulin G4 related disease
    • Eosinophilic angiocentric fibrosis
    • Hyper-IgG4 disease
    • Idiopathic hypocomplementemic tubulointerstitial nephritis
    • Idiopathic Retroperitoneal Fibrosis
    • IgG4 deficiency (subtype)
    • IgG4-related diseases
    • IgG4-related retroperitoneal fibrosis
    • IgG4-related sclerosing cholangitis (subtype)
    • IgG4-related systemic disease
    • Inflammatory aortic aneurysm
    • Inflammatory pseudotumor
    • Küttner's tumor
    • Mediastinal fibrosis
    • Mikulicz's syndrome
    • Multifocal fibrosclerosis
    • Ormond's disease
    • Periaortitis
    • Periarteritis
    • Retroperitoneal fibrosis
    • Riedel's thyroiditis

    Category: Blood
    Prevalence 2,666 cases in the US (Oprita 2017). Female/Male ratio: 2.5x.
    Age of Onset Can occur at any age.
    Description Immunoglobulin G4 is a type of antibody normally found in humans. However, in some persons, the level of this antibody is elevated and it plays a role in the development of several autoimmune diseases, including myasthenia gravis, pemphigus, autoimmune thrombocytopenic purpura and autoimmune pancreatitis. IgG4-related diseases (IgG4-RD) are complex fibro-inflammatory disorder that can affect any organ. Medical practitioners assign a diagnosis based on symptoms and the organ affected. The primary clinical feature in IgG4-RD entails a tumor-like presentation coupled with tissue-destructive lesions.For more information, see the pages on specific diseases.

    At this time, not all IgG4 diseases are documented, and many are very rare. However, as this disease profile shows, IgG4-related disease has 19 synonyms and subtypes, so it may be more common as a class than thought.

    Evidence of autoimmunity: Antibody
    Patient Groups
    Bloggers
    Symptoms
    Resources Link to Clinical Trials
    Link to PubMed
    References Link to Mayo Clinic
    Link to Cleveland Clinic
    Link to Healthline
    Profile by: Julie Farkas
  • Profile updated September 2024
  • Charts generated Oct 12, 2024 at 12:54 PM ET
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    Immunoglobulin G4 related disease sex breakdown


    Immunoglobulin G4 related disease age breakdown

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    Graphs for this disease are generated based on data from the Autoimmune Registry Inc.

  • Immunoglobulin G4 related disease top comorbidities/symptoms
    Chart is omitted due to low report count.

    Immunoglobulin G4 related disease top autoimmune comorbidities


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    * Disease is suspected as autoimmune, but unconfirmed.
    Data Source: Graphs on this page represent data from the Autoimmune Registry Inc. here. To protect participant anonymity, results derived from fewer than 5 participants are not graphed. The Autoimmune Registry Inc is a 501(c)(3) non-profit supported by donors like you.

  • Anti-inflammatory / specialty medications and supplements

    Immunoglobulin G4 related disease top immunosuppressants / autoimmune therapies


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    * Biologic medications.
    Data Source: Graphs on this page represent data in electronic health records from the All of Us research database. As of July 2024, there are 451,000 sets of electronic health records collected from participants. A snapshot of the data can be found here. To protect participant anonymity, results derived from fewer than 20 participants are not graphed.
    The All of Us Research Program is supported by the National Institutes of Health, Office of the Director: Regional Medical Centers: 1 OT2 OD026549; 1 OT2 OD026554; 1 OT2 OD026557; 1 OT2 OD026556; 1 OT2 OD026550; 1 OT2 OD 026552; 1 OT2 OD026553; 1 OT2 OD026548; 1 OT2 OD026551; 1 OT2 OD026555; IAA #: AOD 16037; Federally Qualified Health Centers: HHSN 263201600085U; Data and Research Center: 5 U2C OD023196; Biobank: 1 U24 OD023121; The Participant Center: U24 OD023176; Participant Technology Systems Center: 1 U24 OD023163; Communications and Engagement: 3 OT2 OD023205; 3 OT2 OD023206; and Community Partners: 1 OT2 OD025277; 3 OT2 OD025315; 1 OT2 OD025337; 1 OT2 OD025276. In addition, the All of Us Research Program would not be possible without the partnership of its participants.

  • Quick links: 
    Quick Links

    Patient-reported 7-day average fatigue
    Chart is omitted due to low report count.


    General physical health
    Chart is omitted due to low report count.


    General mental health
    Chart is omitted due to low report count.


    Social and relationship satisfaction
    Chart is omitted due to low report count.


    General quality of life
    Chart is omitted due to low report count.


    Ability to carry out social roles and activities
    Chart is omitted due to low report count.


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    Data Source: Graphs on this page represent data in self-reported survey results from the All of Us research database. As of July 2024, there are 560,000 participants who have completed these surveys. This dataset, however, may not be representative of the US population. A snapshot of the data can be found here. To protect participant anonymity, results derived from fewer than 20 participants are not graphed.
    The All of Us Research Program is supported by the National Institutes of Health, Office of the Director: Regional Medical Centers: 1 OT2 OD026549; 1 OT2 OD026554; 1 OT2 OD026557; 1 OT2 OD026556; 1 OT2 OD026550; 1 OT2 OD 026552; 1 OT2 OD026553; 1 OT2 OD026548; 1 OT2 OD026551; 1 OT2 OD026555; IAA #: AOD 16037; Federally Qualified Health Centers: HHSN 263201600085U; Data and Research Center: 5 U2C OD023196; Biobank: 1 U24 OD023121; The Participant Center: U24 OD023176; Participant Technology Systems Center: 1 U24 OD023163; Communications and Engagement: 3 OT2 OD023205; 3 OT2 OD023206; and Community Partners: 1 OT2 OD025277; 3 OT2 OD025315; 1 OT2 OD025337; 1 OT2 OD025276. In addition, the All of Us Research Program would not be possible without the partnership of its participants.

  • Quick links: 
    Quick Links

    Concerns about ability to pay
    Chart is omitted due to low report count.


    Patient employment status
    Chart is omitted due to low report count.


    Patient insurance coverage
    Chart is omitted due to low report count.


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    Graphs for this disease are generated based on data from the Autoimmune Registry Inc.