“…On T2-weighted MRI imaging lesions demonstrated isointense to hypointense signal intensity, and low signal intensity on T1-weighted series. This review also makes note of adjacent hypertrophic pachymeningitis, which describes the reactive inflammatory thickening of the meninges as seen in our patient’s case 9. A macroscopic examination was only noted in one case report and described fleshy granulation tissue.…”
IgG4-related disease (IgG4-RD) is an inflammatory condition characterised by infiltration of tissue by IgG4-positive plasma cells. This is the seventh reported case of IgG4-RD affecting the mastoid and informs clinicians in diagnosing patients affected by this rare condition.A woman in her 20s presented with unilateral otalgia, hearing loss and vertigo. She deteriorated despite antibiotic therapy and cross-sectional imaging revealed a destructive extra-axial lesion of the mastoid cells. Biopsy confirmed a diagnosis of IgG4-RD. She was successfully treated with prednisolone and azathioprine.Inflammatory conditions should be considered in patients with persistent middle ear symptoms after infection and malignancy are excluded. Delays in diagnosis can lead to irreversible mass effects and may occur as current diagnostic criteria exclude mastoid-specific features.IgG4-RD remains a rare diagnosis. To avoid significant effects on a patients’ quality of life, prompt multidisciplinary treatment is vital alongside development of diagnostic criteria specific to otolaryngology.
“…On T2-weighted MRI imaging lesions demonstrated isointense to hypointense signal intensity, and low signal intensity on T1-weighted series. This review also makes note of adjacent hypertrophic pachymeningitis, which describes the reactive inflammatory thickening of the meninges as seen in our patient’s case 9. A macroscopic examination was only noted in one case report and described fleshy granulation tissue.…”
IgG4-related disease (IgG4-RD) is an inflammatory condition characterised by infiltration of tissue by IgG4-positive plasma cells. This is the seventh reported case of IgG4-RD affecting the mastoid and informs clinicians in diagnosing patients affected by this rare condition.A woman in her 20s presented with unilateral otalgia, hearing loss and vertigo. She deteriorated despite antibiotic therapy and cross-sectional imaging revealed a destructive extra-axial lesion of the mastoid cells. Biopsy confirmed a diagnosis of IgG4-RD. She was successfully treated with prednisolone and azathioprine.Inflammatory conditions should be considered in patients with persistent middle ear symptoms after infection and malignancy are excluded. Delays in diagnosis can lead to irreversible mass effects and may occur as current diagnostic criteria exclude mastoid-specific features.IgG4-RD remains a rare diagnosis. To avoid significant effects on a patients’ quality of life, prompt multidisciplinary treatment is vital alongside development of diagnostic criteria specific to otolaryngology.
“…Both share the common feature of dense lymphoplasmacytic infiltrates and a high proportion of IgG4-bearing plasma cells with a high serum IgG4 level. IgG4-RD shares similarities with Castleman disease, however, there are also differences: (i) Systemic symptoms: IgG4-RD commonly accumulates in multiple glandular organs such as the parotid, lacrimal and salivary glands and the pancreas, and it can occur alone or in association with IgG4-related disease in other organs [16][17][18]. iMCD patients have a number of systemic symptoms including anemia, weakness, several enlarged lymph nodes and weight loss.…”
Background
Castleman’s disease (CD) is a rare disease that has clinical and pathological similarities to lymphoma and is characterized by a high frequency of associated immunological dysfunction. ImmunoglobulinG4-related disease (IgG4-RD) is a collection of systemic disorders that affect numerous organs and are also referred to as IgG4-associated sclerosing diseases. CD and IgG4-RD are difficult to separate because they may manifest similar commin clinical features.
Case presentation
This case describes a 53-year-old female who, during routine medical check-up, exhibited a progressive increase in serum globulin levels and a simultaneous worsening of anemia symptoms, raising concern for a clonal plasma cell disease such as myeloma. However, bone marrow punctures did not reveal any abnormal plasma cells. Also, serum and urine immunofixation electrophoresis demonstrated no abnormal monoclonal protein bands. In addition, several laboratory findings excluded chronic liver disease, chronic infections caused by bacteria or viruses. Later, we found elevated serum IgG4 levels (10,700 mg/L), and identified multiple enlarged lymph nodes throughout the patient’s body. Axillary lymph node aspiration revealed no abnormal lymphocytes, ruling out the possibility of lymphoma. Pathological morphology of the axillary lymph revealed a large number of plasma cells in the lymphatic follicles. In addition, there was a reduction in lymphatic follicle size and apoptosis of the germinal centres. Immunohistochemistry revealed IgG4+/IgG + in > 40% of cells, and more than 100 IgG4 + cells per high powered field (HPF) of specimen. As of now, finding strongly suggested IgG4-RD. This patient was treated with glucocorticoids and various immunosuppressive drugs, such as prednisone, cyclosporine, methotrexate, cyclophosphamide, mycophenolate mofetil, azathioprine and hydroxychloroquine. Unfortunately, the patient did not recover. Ultimately, idiopathic multicentric Castleman disease (iMCD) was diagnosed in relation to the patient’s clinical presentation and laboratory tests, and after combination chemotherapy (VCD: Bortezomib, Cyclophosphamide and Dexamethasone), durable remission was achieved without serious adverse effects. During the follow-up period of one year and ten months, the patient remained stable.
Conclusion
The diagnosis of Castleman must be distinguished from other disorders such as IgG4-RD, malignant lymphoma, reactive hyperplasia of various lymph nodes (mostly caused by viral infections), plasmacytoma, advanced HIV and rheumatic diseases. Besides observing systemic symptoms, laboratory tests such as immunoglobulin levels, complement levels, interleukin levels, and C-reactive protein levels should also be performed in order to determine a diagnosis.
Autoimmune pancreatitis (AIP) is a rare disease. The diagnosis of AIP is difficult and should be made by a comprehensive evaluation of clinical, radiological, serological, and pathological findings. Two different types of AIP have been identified: autoimmune pancreatitis type 1 (AIP-1), which is considered a pancreatic manifestation of multiorgan disease related to IgG4, and autoimmune pancreatitis type 2 (AIP-2), which is considered a pancreas-specific disease not related to IgG4. Although the pathophysiological conditions seem to differ between type 1 and type 2 pancreatitis, both respond well to steroid medications. In this review, we focused on the pathogenesis of the disease to develop a tool that could facilitate diagnosis and lead to the discovery of new therapeutic strategies to combat autoimmune pancreatitis and its relapses. The standard therapy for AIP is oral administration of corticosteroids. Rituximab (RTX) has also been proposed for induction of remission and maintenance therapy in relapsing AIP-1. In selected patients, immunomodulators such as azathioprine are used to maintain remission. The strength of this review, compared with previous studies, is that it focuses on the clear difference between the two types of autoimmune pancreatitis with a clearly delineated and separate pathogenesis. In addition, the review also considers various therapeutic options, including biologic drugs, such as anti-tumor necrosis factor (TNF) therapy, a well-tolerated and effective second-line therapy for AIP type 2 relapses or steroid dependence. Other biologic therapies are also being explored that could provide a useful therapeutic alternative to corticosteroids and immunosuppressants, which are poorly tolerated due to significant side effects.
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