scholarly journals MPO-ANCA-Positive Hypertrophic Pachymeningitis With Elevated Serum IgG4: A Case Report and Literature Review.

2021 ◽  
Author(s):  
Zhaoli Gao ◽  
Zhao Hu ◽  
Yanxia Gao
Keyword(s):  
Medical History ◽  
Elevated Serum ◽  
Progressive Increase ◽  
Pathological Examination ◽  
Hypertrophic Pachymeningitis ◽  
Case Presentation ◽  
Serum Igg4 ◽  
History Play ◽  
Previous Medical History ◽  
Igg4 Level

Abstract Background: We report a case of serum myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA)-positive hypertrophic pachymeningitis with elevated serum immunoglobulin G (IgG)4.Case presentation: He was a 67-year-old man who appeared headache and hearing loss 7 years ago. He was treated with methylprednisolone and mycophenolate when he was diagnosed with hypertrophic pachymeningitis 2 years ago. He went to the hospital this time because of fatigue and appetite, the results of his blood tests indicated a progressive increase in creatinine and an increase in MPO and IgG4. His kidney biopsy revealed very few infiltrating IgG4-positive cells. Instead, fibrinoid necrosis of arterioles was observed. Considering the clinical process and these findings, the disease was considered more likely to be ANCA-associated hypertrophic pachymeningitis with elevated serum IgG4 level.Conclusions: Our case highlights the importance of the pathological examination and the medical history. Pathological examination and previous medical history play an essential role in the diagnosis of the disease. Only when the disease is correctly diagnosed can it be further treated effectively and get a better prognosis.Data Sources: Data were collected from the patient’s electronic medical records and the hospital laboratory medicine database.

scholarly journals Sellar germinoma mimicking IgG4-related hypophysitis: a case report

2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Kang Chen ◽  
Yong Yao ◽  
Xinxin Mao ◽  
Hui You ◽  
Linjie Wang ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Inflammatory Diseases ◽  
Elevated Serum ◽  
Poor Response ◽  
Pathological Examination ◽  
Exclusion Criterion ◽  
Sellar Mass ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

Abstract Background The differential diagnosis of IgG4-related hypophysitis and other inflammatory diseases or tumors involving sellar region is challenging even after sellar biopsy. Sellar germinoma is usually infiltrated by lymphocytes or plasma cells, and may be confused with hypophysitis. Case presentation A 36-year-old man with diabetes insipidus, elevated serum IgG4 level (336 mg/dl), and sellar mass was suspected to have IgG4-related hypophysitis, and no other lesion of IgG4-related disease was detected. After treated by prednisone and mycophenolate mofetil, the serum IgG4 decreased to 214 mg/dl. However, after withdrawal of the drugs, the IgG4 level increased to 308 mg/dl. Endocrine assessments revealed panhypopituitarism, and the sellar mass enlarged. Transsphenoidal sellar exploration and biopsy was conducted. Pathological examination showed that the lesion was germinoma with lymphocytes and plasma cells infiltration, and IgG4-staining was positive (70/HPF, IgG4/IgG ratio = 10%). The patient was then treated by cisplatin and etoposide. After four cycles of chemotherapy, the serum IgG4 was 201 mg/dl, and the sellar mass was invisible. Conclusion Sellar germinoma can mimic the clinical characteristics of IgG4-related hypophysitis. Poor response to glucocorticoids can be used as an exclusion criterion in the clinical diagnosis of IgG4-related hypophysitis.

scholarly journals Immunoglobulin G4-related hypertrophic pachymeningitis

2019 ◽  
Vol 6 (4) ◽  
pp. e568 ◽  
Author(s):  
Michaël Levraut ◽  
Mikaël Cohen ◽  
Saskia Bresch ◽  
Caroline Giordana ◽  
Fanny Burel-Vandenbos ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Case Reports ◽  
Treatment Algorithm ◽  
Elevated Serum ◽  
Case Series ◽  
Hypertrophic Pachymeningitis ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Meningeal Involvement ◽  
Serum Igg4

ObjectiveMeningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).MethodsTwo IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.ResultsForty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.Discussion/conclusionIgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.

scholarly journals IgG4-related disease in a patient with HIV infection

2019 ◽  
Vol 12 (4) ◽  
pp. e226809
Author(s):  
Raquel Ron ◽  
Ignacio Ruz-Caracuel ◽  
Eugenia García ◽  
María Luisa Montes-Ramírez
Keyword(s):  
Lymph Nodes ◽  
Elevated Serum ◽  
Lung Damage ◽  
Axillary Lymph Nodes ◽  
Hiv Positive ◽  
Axillary Lymph ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

A 47-year-old HIV-positive man with good immune and virological status presented with chronic multiple enlarged lymph nodes, lung disease and eosinophilia. Radiologic tests showed enlarged cervical, thoracic and axillary lymph nodes, with interstitial lung damage. After several non-specific histologic studies, an elevated serum IgG4 level led us to request immunohistochemistry of a lymph node sample. The test confirmed the diagnosis of IgG4-related disease.

scholarly journals Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Jay H. Ryu ◽  
Ryohei Horie ◽  
Hiroshi Sekiguchi ◽  
Tobias Peikert ◽  
Eunhee S. Yi
Keyword(s):  
Clinical Practice ◽  
Wide Spectrum ◽  
Systemic Vasculitis ◽  
Elevated Serum ◽  
Serum Igg4 Level ◽  
Igg4 Related Disease ◽  
Clinical Presentations ◽  
Serum Igg4 ◽  
Disease Associations ◽  
Igg4 Level

IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8%) had an elevated serum IgG4 level (>140 mg/dL). IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4%) had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

scholarly journals Clinical Characteristics of 76 Patients with IgG4-Related Hypophysitis: A Systematic Literature Review

2019 ◽  
Vol 2019 ◽  
pp. 1-10
Author(s):  
Yujuan Li ◽  
Hua Gao ◽  
Zhen Li ◽  
Xinxin Zhang ◽  
Yizhi Ding ◽  
...  
Keyword(s):  
Clinical Features ◽  
Case Reports ◽  
Disease Onset ◽  
Elevated Serum ◽  
Median Number ◽  
Normal Serum ◽  
Hormone Deficiency ◽  
Serum Igg4 Level ◽  
Serum Igg4 ◽  
Igg4 Level

Background. IgG4-related hypophysitis (IgG4-RH) is a rare disease, and its prevalence remains unclear. In recent years, an increasing number of cases have been reported because of the increasing recognition of this disease. We aimed to summarize case reports of IgG4-RH and outline the clinical features and outcomes. Methods. We performed PubMed search of articles using the search terms “hypophysitis [AND] IgG4.” Consequently, only 54 English articles (76 cases) met Leporati’s diagnostic criteria. Results. Of the 76 cases, the ratio of men to women was 1.5 : 1, and the age at diagnosis was 54.1 ± 17.8 years. The median IgG4 concentration was 405.0 mg/dl. Anterior hypopituitarism, isolated central diabetes insipidus, and panhypopituitarism were observed in 14 (18.4%), 12 (15.8%), and 44 (57.9%) cases, respectively. The sequence of anterior hormone deficiency was as follows: gonadotropin (68.4%), ACTH (63.2%), TSH (59.2%), GH (48.7%), and prolactin (42.1%). The median number of involved organs was 1.5, and the lung (18.4%), retroperitoneum (17.1%), kidney (15.8%), submandibular glands (14.5%), and pancreas (13.2%) were the common involved organs. Elevated IgG4 concentration and normal IgG4 level were in 42 (76.4%) and 13 (23.6%) cases, respectively. Patients with elevated serum IgG4 concentration were older (60.9 ± 14.3 vs 45.6 ± 17.4, p=0.001) and male-prone (78.6% vs 40.4%, p=0.003) and had a susceptibility of multiple organ involvement (78.6% vs 35.0%, p=0.001) compared to those with normal serum IgG4 levels. Males were older at disease onset (61.5 ± 12.6 vs 42.9 ± 18.8, p<0.001) and had a higher IgG4 concentration (425.0 vs 152.5, p=0.029) and a greater number of involved organs (2.0 vs 0.0, p=0.001), while isolated hypophysitis was more prominent in female (63.3% vs 26.1%, p=0.001). Conclusion. In this review, we found that there were different characteristics between different genders. Patients with elevated serum IgG4 level in terms of some clinical features were also different from those with normal serum IgG4 level. However, the data in this review were limited by bias and confounding. Further clinical studies with larger sample sizes are warranted.

Serum Immunoglobulin G Fraction 4 Levels in Pancreatic Cancer: Elevations Not Associated With Autoimmune Pancreatitis

2008 ◽  
Vol 132 (1) ◽  
pp. 48-53 ◽  
Author(s):  
Amit Raina ◽  
Alyssa M. Krasinskas ◽  
Julia B. Greer ◽  
Janette Lamb ◽  
Erin Fink ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Cancer Patients ◽  
Immunoglobulin G ◽  
Autoimmune Pancreatitis ◽  
Elevated Serum ◽  
Serum Immunoglobulin ◽  
Pancreatic Mass ◽  
Serum Igg4 Level ◽  
Serum Igg4 ◽  
Igg4 Level

Abstract Context.—Autoimmune pancreatitis is an uncommon, inflammatory disease of the pancreas that presents with clinical features, such as painless jaundice and a pancreatic mass, similar to those caused by pancreatic cancer. Patients with autoimmune pancreatitis frequently have elevated serum immunoglobulin G fraction 4 (IgG4) levels, and their pancreatic tissue may show IgG4-positive plasma cell infiltration. It is imperative to differentiate autoimmune pancreatitis from pancreatic cancer because autoimmune pancreatitis typically responds to corticosteroid treatment. A previous Japanese study reported that serum IgG4 greater than 135 mg/dL was 97% specific and 95% sensitive in predicting autoimmune pancreatitis. Objective.—To prospectively measure serum IgG4 levels in pancreatic cancer patients to ascertain whether increased levels might be present in this North American population. Design.—We collected blood samples and phenotypic information on 71 consecutive pancreatic cancer patients and 103 healthy controls who visited our clinics between October 2004 and April 2006. IgG4 levels were determined using a single radial immunodiffusion assay. A serum IgG4 level greater than 135 mg/dL was considered elevated. Results.—Five cancer patients had IgG4 elevation, with a mean serum IgG4 level of 160.8 mg/dL. None of our cancer patients with plasma IgG4 elevation demonstrated evidence of autoimmune pancreatitis. One control subject demonstrated elevated serum IgG4 unrelated to identified etiology. Conclusions.—As many as 7% of patients with pancreatic cancer have serum IgG4 levels above 135 mg/dL. In patients with pancreatic mass lesions and suspicion of cancer, an IgG4 level measuring between 135 and 200 mg/dL should be interpreted cautiously and not accepted as diagnostic of autoimmune pancreatitis without further evaluation.

scholarly journals Prevalence of Elevated Serum IgG4 Level among Patients Diagnosed or Suspected with Cardiovascular Disorders

2017 ◽  
Vol 57 (6) ◽  
pp. 91-98 ◽  
Author(s):  
Nobukazu Ishizaka ◽  
Yasunari Fujinaga ◽  
Ryoto Hourai ◽  
Tomohiro Fujisaka ◽  
Koichi Sohmiya
Keyword(s):  
Elevated Serum ◽  
Cardiovascular Disorders ◽  
Serum Igg4 Level ◽  
Serum Igg4 ◽  
Igg4 Level

scholarly journals A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Kazuhiko Higashioka ◽  
Kenji Yoshida ◽  
Kensuke Oryoji ◽  
Kazuo Kamada ◽  
Shinichi Mizuki ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Elevated Serum ◽  
Clinical Findings ◽  
Organ Involvement ◽  
Immunoglobulin G4 ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

We report a case of IgG4-related disease (IgG4-RD) with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

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