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IgG4-related disease

ICD-10 Codes

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Description

IgG4-related Disease: A Multi-Organ Fibro-Inflammatory Condition

IgG4-related disease (IgG4-RD) is a rare immune-mediated condition that affects multiple organs and tissues in the body. It is characterized by the formation of tumefactive lesions, which are large, painless masses that can cause organ enlargement.

Key Features:

  • Multi-organ involvement: IgG4-RD can affect nearly any organ system, including the pancreas, liver, biliary tract, retroperitoneum, head and neck, and other organs.
  • Fibro-inflammatory condition: The disease is characterized by fibrosis (scarring) and inflammation in affected organs.
  • Tumefactive lesions: IgG4-RD is typified by the formation of large, painless masses that can cause organ enlargement.

Common Presentations:

  • Major salivary and lacrimal gland involvement: The disease often affects the major salivary glands (such as the parotid gland) and lacrimal glands.
  • Pancreato-hepato-biliary disease: IgG4-RD can affect the pancreas, liver, and biliary tract, leading to symptoms such as abdominal pain and jaundice.

Other Organ Involvement:

  • Retroperitoneal fibrosis and/or aortitis: The disease can also affect the retroperitoneum (the area behind the abdominal organs) and cause inflammation of the aorta.
  • Head and neck disease: IgG4-RD can involve the head and neck, leading to symptoms such as facial swelling and difficulty swallowing.

References:

  • [1] by LYC Chen · 2019 · Cited by 165 — IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland ...
  • [3] by S Nambiar · 2023 · Cited by 15 — It is a multi-organ, fibro-inflammatory condition with tumefactive lesions of unknown etiology and characteristic histopathological features.
  • [7] by CA Perugino · 2020 · Cited by 300 — IgG4-related disease (IgG4-RD) is an insidiously progressive immune-mediated fibrotic disease typified by tumour-like mass formation in many ...
  • [9] by ZS Wallace · 2024 · Cited by 7 — IgG4-related disease (IgG4-RD) is an increasingly recognized cause of fibroinflammatory lesions in patients of diverse racial and ethnic backgrounds.

Additional Characteristics

  • Multi-organ involvement: IgG4-RD can affect nearly any organ system, including the pancreas, liver, biliary tract, retroperitoneum, head and neck, and other organs.
  • Fibro-inflammatory condition: The disease is characterized by fibrosis (scarring) and inflammation in affected organs.
  • Tumefactive lesions: IgG4-RD is typified by the formation of large, painless masses that can cause organ enlargement.
  • Major salivary and lacrimal gland involvement: The disease often affects the major salivary glands (such as the parotid gland) and lacrimal glands.
  • Pancreato-hepato-biliary disease: IgG4-RD can affect the pancreas, liver, and biliary tract, leading to symptoms such as abdominal pain and jaundice.
  • or aortitis: The disease can also affect the retroperitoneum (the area behind the abdominal organs) and cause inflammation of the aorta.
  • Head and neck disease: IgG4-RD can involve the head and neck, leading to symptoms such as facial swelling and difficulty swallowing.

Signs and Symptoms

Common Signs and Symptoms of IgG4-Related Disease

IgG4-related disease (IgG4-RD) is a systemic condition characterized by the presence of IgG4-positive plasma cells, which can lead to various symptoms. Here are some common signs and symptoms associated with IgG4-RD:

  • Weight loss: Weight loss is a particularly common symptom in patients with IgG4-RD, especially when multiple organ systems are affected [1][2].
  • Lymphadenopathy: Swollen lymph nodes are another common manifestation of IgG4-RD [3][4].
  • Autoimmune pancreatitis: This is a type of inflammation of the pancreas that can occur in patients with IgG4-RD [5].
  • Salivary gland disease: Enlargement or swelling of the salivary glands, lacrimal glands, and other organs can also be symptoms of IgG4-RD [6][7].
  • Tumor-like masses: Tumor-like masses in different regions, such as the pancreas, liver, or lymph nodes, can occur in patients with IgG4-RD [8].

Additionally, some patients may experience more general symptoms, including:

  • Fatigue
  • Loss of appetite
  • Joint and tendon pain

It's essential to note that these symptoms can vary widely among individuals, and not everyone will exhibit all of them. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper diagnosis and treatment.

References:

[1] Symptoms of IgG4-Related Disease. Common symptoms include swollen lymph nodes and weight loss. [2] Symptoms and Signs of IgG4-Related Disease ... Weight loss is a particularly common symptom when multiple organ systems are affected. [3] Common features include IgG4-related autoimmune pancreatitis, swelling of or within an organ system (an inflammatory pseudotumor), salivary gland disease (which ... [4] by LYC Chen · 2019 · Cited by 165 — Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis ... [5] Sometimes people have mild symptoms (such as tiredness, loss of appetite) for months or even years before seeking medical help. Other common symptoms include:. [6] by M Legatowicz-Koprowska · 2018 · Cited by 18 — Symptoms of IgG4-RD include diffuse pain of joints, tendons, with associated fatigue, especially when several systems/organs are already affected. There is no ... [7] Some common symptoms include: Tumor-like masses in different regions; Enlargement or swelling of organs; Sneezing, runny nose, or sinus complications; Eye ... [8] Sep 10, 2024 — Outline · Spectrum of disease · Autoimmune pancreatitis · IgG4-related sclerosing cholangitis · Lymphadenopathy · Salivary gland involvement ...

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for IgG4-related Disease

IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that can affect various organs, including the pancreas, kidneys, and lymph nodes. Diagnosing IgG4-RD requires a combination of clinical evaluation, laboratory tests, and imaging studies.

Clinical Evaluation The diagnosis of IgG4-RD often begins with a thorough clinical evaluation by a healthcare provider. This includes taking a detailed medical history, performing a physical examination, and assessing symptoms such as swelling, pain, or organ dysfunction [1].

Laboratory Tests

  • Serum Protein Electrophoresis: This test can help identify abnormal protein levels in the blood, which may indicate IgG4-RD [2].
  • IgG Subclasses: Measuring IgG subclasses can provide additional information to support a diagnosis of IgG4-RD [3].

Imaging Studies

  • Computed Tomography (CT) Scan: CT scans can help identify enlarged organs or lymph nodes, which may be indicative of IgG4-RD [6].
  • Magnetic Resonance Imaging (MRI): MRI can provide detailed images of affected organs and tissues, helping to confirm a diagnosis of IgG4-RD [6].

Tissue Biopsy A tissue biopsy is often necessary for a definitive diagnosis of IgG4-RD. The biopsy should be performed on the affected organ or lymph node and examined for characteristic histological features such as storiform fibrosis and obliterative phlebitis [5, 9].

Additional Tests

  • Urinalysis: This test can help assess kidney function and detect proteinuria, which may indicate IgG4-related kidney involvement [8].
  • Serum Creatinine: Measuring serum creatinine levels can provide information on kidney function and help monitor disease activity [8].

It's essential to note that a diagnosis of IgG4-RD requires a comprehensive evaluation by a healthcare provider, taking into account the results of these diagnostic tests.

References:

[1] by M Abraham · 2017 · Cited by 53 [2] by LYC Chen · 2019 · Cited by 165 [3] by H Umehara · 2021 · Cited by 431 [5] by H Umehara · 2021 · Cited by 431 [6] [8] [9]

Additional Diagnostic Tests

  • Magnetic Resonance Imaging (MRI)
  • Computed Tomography (CT) Scan
  • Urinalysis
  • Serum Protein Electrophoresis
  • IgG Subclasses
  • Tissue Biopsy
  • Serum Creatinine

Treatment

Treatment Options for IgG4-related Disease

IgG4-related disease (IgG4-RD) is a chronic inflammatory condition that can affect various organs, including the pancreas, kidneys, and lymph nodes. While there is no cure for IgG4-RD, several treatment options are available to manage the symptoms and prevent organ damage.

Glucocorticoids: The Cornerstone of Treatment

Glucocorticoids, such as prednisone, are considered the cornerstone of treatment for most patients with IgG4-RD [4][9]. These medications can reduce inflammation and induce remission in many patients. The initial recommended dose is 0.6 mg/kg/day, administered for 2-4 weeks, which is then gradually tapered to a maintenance dose [3].

Biological Agents: A Promising Alternative

Biological agents, such as rituximab and elotuzumab, have shown promise in treating active IgG4-RD [6][7]. Rituximab, in particular, has been found to be nearly universally effective in inducing remission in patients with IgG4-RD. Elotuzumab is currently being investigated as a potential treatment option for IgG4-RD in an NIH-funded clinical trial.

Other Treatment Options

In addition to glucocorticoids and biological agents, other treatment options are available for IgG4-RD. These include:

  • Inebilizumab: A monoclonal antibody that has been shown to reduce the risk of flares and increase the likelihood of flare-free complete remission [2].
  • Steroids: While steroids can cause side effects, they remain an effective treatment option for many patients with IgG4-RD [8].

Treatment Goals

The primary goal of treatment in IgG4-RD is to reduce inflammation and prevent organ damage. Treatment should be individualized based on the patient's specific needs and medical history.

References:

[1] HM Moutsopoulos, et al. (no context provided)

[2] JH Stone (2024) [2]

[3] T Kamisawa (2017) [3]

[4] Y Tanaka (2023) [4]

[5] CA Perugino (2016) [5]

[6] (no context provided)

[7] Jan 19, 2022 (no context provided)

[8] ZS Wallace (2024) [8]

[9] Oct 14, 2020 [9]

Recommended Medications

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Differential Diagnosis

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder characterized by elevated serum levels of IgG4, marked infiltration of IgG4-bearing plasma cells, and tumefaction in various organs. When considering the differential diagnosis of IgG4-RD, several conditions should be taken into account.

Common Mimickers

  • Autoimmune pancreatitis: This condition is often considered a variant of IgG4-RD, especially when it presents with similar clinical features such as pancreatic swelling and elevated serum levels of IgG4 [1].
  • Lymphadenopathy: Enlarged lymph nodes can be a feature of various conditions, including lymphoma, which should be ruled out in the differential diagnosis of IgG4-RD [2].
  • Multicentric Castleman disease: This is a rare disorder characterized by enlarged lymph nodes and systemic symptoms, which can mimic IgG4-RD [3].

Other Differential Diagnoses

  • IgG4-related sclerosing cholangitis: This condition involves inflammation of the bile ducts and can be associated with elevated serum levels of IgG4 [4].
  • Salivary gland involvement: Involvement of the salivary glands can occur in various conditions, including autoimmune pancreatitis and lymphadenopathy [5].

Laboratory Abnormalities

  • Peripheral eosinophilia: Elevated levels of eosinophils in the blood can be a feature of IgG4-RD, as well as other conditions such as hypereosinophilic syndromes [6].
  • Hypergammaglobulinemia: Elevated levels of gamma globulins in the blood can be associated with various conditions, including IgG4-RD and lymphoma [7].

Rare Differential Diagnoses

  • Lymphomatoid granulomatosis: This is a rare condition characterized by inflammation of the lymph nodes and other organs, which can mimic IgG4-RD [8].
  • Inflammatory myofibroblastic tumor (IMT): This is a rare tumor that can involve various organs, including the pancreas, and should be considered in the differential diagnosis of IgG4-RD [9].

References:

[1] S Nambiar · 2023 · Cited by 15 [2] Sep 10, 2024 [3] by M Yamamoto · 2022 · Cited by 3 [4] by LYC Chen · 2019 · Cited by 165 [5] Oct 2, 2024 [6] by JH Stone · 2015 · Cited by 214 [7] by A Satou · 2020 · Cited by 44 [8] IgG4-related disease is a systemic disorder characterised by diffuse or tumoural inflammatory lesions.

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