Causes
Inflammation and fibrosis are caused by plasma cells that are positive for IgG4 infiltrating various organs in IgG4-related disease (IgG4-RD). The exact cause is unknown, but it is believed to involve an abnormal immune response. Genetic factors, environmental triggers, and infections may contribute to the onset and progression of the disease.
Symptoms
Depending on the organs affected, IgG4-RD can cause a wide range of symptoms:
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An autoimmune pancreatitis can cause abdominal pain, weight loss, and jaundice.
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Dry mouth, dry eyes are common symptoms of swelling of the salivary and lacrimal glands (which can lead to conditions like Mikulicz's disease).
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Enlargement of lymph nodes without pain.
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Symptoms of renal dysfunction include proteinuria and renal insufficiency.
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Fibrosis of the retroperitoneum can cause abdominal or back pain.
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Aneurysms or dissections of the aorta can result from aortitis.
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The thyroid gland is swollen or dysfunctional (Riedel's thyroiditis).
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It can also affect the lungs, liver, and central nervous system, resulting in organ-specific symptoms.
Diagnosis
IgG4-RD is diagnosed through a combination of clinical evaluations, laboratory tests, imaging studies, and histopathological examinations:
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Assessing symptoms and medical history to identify potential organ involvement.
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Elevated serum IgG4 levels may suggest IgG4-RD, but they are not definitive.
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CT scans, MRIs, or PET scans can detect organ enlargements, masses, and fibrotic changes.
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For a diagnosis, a biopsy of the affected organ must show lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis.
Treatment
IgG4-RD is treated to reduce inflammation, manage symptoms, and prevent organ damage:
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Prednisone is commonly used to reduce inflammation. Initial doses are tapered down over time.
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In cases where steroids are contraindicated, immunosuppressive agents such as azathioprine, methotrexate, or mycophenolate mofetil may be used.
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Rituximab, a monoclonal antibody, can be effective in refractory cases.
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Symptomatic treatment: Treatment for specific organ dysfunctions, such as pancreatic enzyme supplements for pancreatic insufficiency or hypertension due to renal impairment.
Prevention
The exact cause of IgG4-RD is unknown, so there are no specific preventive measures. However, early detection and management are crucial:
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In individuals with known risk factors or those already diagnosed, regular follow-up with healthcare providers is recommended to monitor disease progression and treatment response.
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By educating patients about the symptoms and potential complications, early diagnosis and treatment can be ensured.
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Maintaining a healthy lifestyle, including a balanced diet and regular exercise, can enhance immune function.
Conclusion
Multiple organs are affected by IgG4-related disease, which has a variety of symptoms. Understanding its presentation and effective management strategies are essential to improving patient outcomes, even though the exact cause is unclear. The disease can be managed and severe complications can be avoided by early diagnosis, appropriate treatment, and regular monitoring.
