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Interdisciplinary Approach Is Essential for Sufficient Diagnosis and Therapy in Igg4-Related Disease Involving Multiple Organs
United European Gastroenterology Journal ; 10(Supplement 8):934-935, 2022.
Article in English | EMBASE | ID: covidwho-2114143
ABSTRACT

Introduction:

IgG4-related disease (IgG4-RD) is a systemic, chronic inflammatory syndrome, with enlargement of involved organs, elevated serum levels of IgG4, dense lymphoplasmocytic infiltrates, rich in IgG4-positive plasma cells, and fibrosis in involved organs. The most frequently involved organs are the pancreato-hepatobiliary tract, salivary and lacrimal glands, the retroperitoneum, kidneys, lungs, and aorta. Often multiple organ systems are involved. As an initial treatment, glucocorticoids are recommended. In patients with relapse along with glucocorticoid dose reduction, various immunosuppressive agents have been reported. Aims &

Methods:

We reviewed 98 patients (2019-now) who were treated in the special outpatient unit for IgG4-RD at the University Hospital of Essen and identified 10 patients with IgG4-RD involving multiple organ systems. Result(s) The first patient is a 65-year-old male diagnosed with an IgG4- RD involving parotitis, lymphadenitis, sialadenitis with orbitopathy and elevated IgG4 serum level (7400 mg/l). Clinically response to therapy with steroids was documented. The second patient is a 63-year-old man with an IgG4-positive pancreatitis, sialadentis, lymphadenopathy, and elevated IgG4 serum level (3960 mg/l). Immunosuppression with tacrolimus leaded to clinical benefit. As the third patient, we report a 48-year-old man with IgG4-related inflammatory condition in pancreas and kidneys with high IgG4 serum levels. The patient was successfully treated with azathioprine and prednisolone. As the fourth patient, we demonstrate a 34-year-old man with IgG4-related autoimmune hepatitis, lymphadenitis, and pancreatitis. After treatment with tacrolimus in combination with rituximab, a significant decrease of IgG4-level was detected. The fifth patient, a 65-year-old man, was diagnosed with IgG4-related fibro- inflammatory pseudotumors in the liver, esophagitis, and lymphadenopathy combined with high serum levels of IgG4 (12000 mg/l). Clinically response to therapy with steroids and azathioprine was reported. As the sixth patient we demonstrate a 29-year-old male with IgG4-related lymphadenopathy, recurrent myocarditis, and pancreatitis. The patient has symptom-free episodes under low-dose prednisolone. We also found an IgG4-RD with multiple organ involvement in our seventh patient. A 54-year-old man with IgG4-related cholangitis, pancreatitis, prostatitis, and very high serum level of IgG4 (26700 mg/l) were treated with steroids and azathioprine. As our eight case, we present a 23-year-old man with congenital hepatic fibrosis, after living-donor liver transplantation, who developed an IgG4- related disease with high IgG4 serum levels (45300 mg/l) after infection with SARS-CoV-2. Pathologically enlarged lymph nodes were detected. In a biopsy of retroperitoneal lymph nodes, IgG4-positive plasma cells were detectable. Intestinal biopsies have shown numerous positive plasma cells in the IgG4-staining (40 IgG4 positive plasma cells/HPF). Treatment with rituximab is planned. The ninth patient is a 56-year-old woman with lymphadenitis and cholangitis, who clinically responded to a treatment with budesonide. As the last patient we present a 59-year-old man with an IgG4-related aortitis, cardiac fibrosis, cholangitis, hepatitis, exocrine pancreatic insufficiency and Hashimoto's thyroiditis responding very sufficient to rituximab. Conclusion(s) An interdisciplinary approach is essential for a sufficient diagnosis and therapy in IgG4-RD involving multiple organs. This collective is extremely heterogeneous, and treatment is often based on individual concepts.
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Full text: Available Collection: Databases of international organizations Database: EMBASE Language: English Journal: United European Gastroenterology Journal Year: 2022 Document Type: Article

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Full text: Available Collection: Databases of international organizations Database: EMBASE Language: English Journal: United European Gastroenterology Journal Year: 2022 Document Type: Article