EP.TU.182Surgical Serendipity: The Accident that Exposed an Autoimmune disease

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Emiko Sultana
Keyword(s):  
Rare Condition ◽  
High Serum ◽  
Middle Lobe ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Close Relationship ◽  
Bronchoscopic Biopsy ◽  
The Right ◽  
Car Accident ◽  
Relationship Of

Abstract Objectives IgG4 related disease is an autoimmune condition that often leads to diagnostic challenges which can often delay timely treatment. It rarely presents with pulmonary findings which can radiologically mimic cancer. We describe a unique case of an incidentally diagnosed IgG4- related lung disease (IgG4-RLD). Case description A 32-year-old healthy female suffered a car accident and had a CT Trauma. This revealed an indeterminate soft tissue lesion in the middle lobe of the right lung with an endobronchial component of uncertain histology which had central extension into the right inferior hilum. She had a bronchoscopic biopsy revealing that the mass was an Inflammatory Myofibroblastic Tumour (IMT). Due to the close relationship of this tumour with the root of her right lung, she was planned for a right pneumonectomy. However, it was agreed for that she underwent another bronchoscopy with biopsy to obtain more histology before proceeding with a pneumonectomy for the uncertainty about the diagnosis of an IMT and due to the absence of distinct clinical features from this mass. This revealed that the mass was highly suggestive of IgG4-RLD. Her blood tests revealed high serum IgG4 levels which confirmed the diagnosis of IgG4-RLD. Conclusion IgG4-RLD is a rare condition which can seldom be diagnosed incidentally. Patients with biopsies suggestive of IMT should undergo a repeat biopsy before proceeding to surgical resection of the tumour. Alternatively, a serum IgG4 level can also be deemed a useful tool for screening such patients.

scholarly journals High Serum IgG4 Concentrations in Patients with Hashimoto’s Thyroiditis

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Anna Popławska-Kita ◽  
Maria Kościuszko-Zdrodowska ◽  
Katarzyna Siewko ◽  
Beata Telejko ◽  
Justyna Hryniewicka ◽  
...  
Keyword(s):  
Fas Ligand ◽  
Hashimoto Thyroiditis ◽  
High Serum ◽  
Composition Analysis ◽  
Rapid Progression ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Receptor Antibodies ◽  
Higher Doses ◽  
Igg4 Level

Purpose. Since recent reports suggest that Hashimoto thyroiditis (HT) may be associated with IgG4-related disease, we aimed to find out whether the measurement of serum IgG4 allows for the identification of distinct types of HT, with different clinical, sonographic, and serologic characteristics.Methods. The group studied consisted of 53 patients with HT and 28 healthy individuals who underwent thyroid ultrasonography and body composition analysis. Serum concentrations of IgG4, TSH, anti-peroxidase antibodies (TPOAb), anti-TSH receptor antibodies, TNF-α, TGF-β1, Fas Ligand, TRAIL, and chemokines (CXCL9, CXCL11, and CXCL10) were measured by ELISA or radioimmunoassay.Results. The group with IgG4 level >135 IU/ml accounted for 32.5% of the patients. The signs of fibrosis were present in 27.0% of the high-IgG4 patients and in 9.1% of the normal-IgG4 group. The patients with elevated IgG4 required higher doses of L-thyroxine and had significantly lower level of TPOAb (P=0.02) than the non-IgG4-HT individuals and higher TNF-αlevel in comparison with the controls (P=0.01).Conclusions. Our results suggest that the measurement of serum IgG4 allows for an identification of patients with more rapid progression of HT, requiring higher doses of L-thyroxine. Low TPOAb level and the absence of coexisting autoimmune diseases may suggest distinct pathomechanism of this type of thyroiditis.

scholarly journals Immunoglobulin G4-related disease - diagnostic dilemma and importance of clinical judgement: a case report

2020 ◽  
Vol 8 (11) ◽  
pp. 4144
Author(s):  
Jagadeesh Chandrasekaran ◽  
Phani Krishna Machiraju
Keyword(s):  
Early Recognition ◽  
Elevated Serum ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Inflammatory Disorder ◽  
Imaging Features ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
The Right

Immunoglobulin G4 (IgG4)-related disease is a multi-organ, immune-mediated, fibro-inflammatory disorder characterized by tumefactive masses in the affected organs. Incidence and prevalence of IgG4-related disease (RD) are not clearly known and have slight male preponderance. It often involves multiple organs at the time of presentation or over the course of disease mimicking malignancy, Sjogren's syndrome, antineutrophil cytoplasmic antibodies associated vasculitis, infections. A thorough workup is needed to rule out these mimickers. A 33-year-old gentleman presented to us with history of progressive swelling in the right peri-orbital region for four years. On evaluation, abdominal imaging was notable for the sausage-shaped pancreas and hypoenchancing nodules in bilateral kidneys. Histological examination of right lacrimal gland revealed lymphoplasmacytic infiltrate and storiform fibrosis. Serum IgG4 levels were normal, and immunostaining was negative. A diagnosis of IgG4-RD was suggested because of multi-organ involvement, classical radiological and histopathological features. Awareness about IgG4-RD, an under-recognized entity is essential, as it is treatable, and early recognition may help in a favourable outcome. Appropriate use of clinicopathological, serological and imaging features in the right clinical context may help in accurate diagnosis. Elevated serum IgG4 levels and biopsy are not mandatory for the diagnosis.

scholarly journals IgG4-Related Disease without Overexpression of IgG4: Pathogenesis Implications

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Naoshi Nishina ◽  
Yuko Kaneko ◽  
Masataka Kuwana ◽  
Hironari Hanaoka ◽  
Hideto Kameda ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Histopathological Examination ◽  
High Serum ◽  
Cell Infiltration ◽  
Pituitary Mass ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Plasma Cell Infiltration ◽  
Serum Igg4 ◽  
Systemic Lymphadenopathy

IgG4-related disease is a new disease group that affects multiple organs. It is characterized by high serum IgG4 and abundant infiltration of IgG4-bearing plasma cells in the affected organ. Here, we describe an intriguing case that suggested that IgG4-related disease might present without IgG4 overexpression or infiltration, at least during a relapse. A 47-year-old man had been diagnosed with systemic lupus erythematosus 15 years. He was admitted due to a pituitary mass, systemic lymphadenopathy, and multiple nodules in the lungs and kidneys. The serum IgG4 level was normal and histopathological examination of the pituitary mass showed abundant lymphocyte and plasma cell infiltration with very few IgG4-positive cells. When we examined specimens preserved from 15 years ago, we found high serum IgG4 levels and IgG4-bearing plasma cell infiltration. This resulted in a diagnosis of IgG4-related disease, and we considered the current episode to be a relapse without IgG4 overexpression. This case indicated that, to clarify the pathogenesis of IgG4-related disease, current cases should repeat specimen evaluations over the course of IgG4-related disease to define diagnostic markers.

scholarly journals Acute Suppurative Sialadenitis Secondary to Excisional Ranula Biopsy in Child

2016 ◽  
Vol 5 (2) ◽  
pp. 123-126
Author(s):  
Tiago N Pinheiro ◽  
Juliana Arid ◽  
Aloizio P Maciel ◽  
André FM Machado ◽  
Marleno L Monteiro ◽  
...  
Keyword(s):  
Opportunistic Infections ◽  
Mandibular Gland ◽  
Excisional Biopsy ◽  
Preoperative Care ◽  
Antibacterial Prophylaxis ◽  
Biopsy Site ◽  
Close Relationship ◽  
The Right ◽  
Relationship Of

ABSTRACT Introduction Ranulas are lesions that affect the salivary glands, and they are generally treated by excision; however, they can recur in some cases where the gland is not removed and they may progress to acute suppurative sialadenitis, yet such cases are rare, especially in children. Aims To report clinical management of occurrence of acute suppurative sialadenitis secondary to excisional biopsy of ranula in a child. Case report An 11-year-old female patient was diagnosed with ranula where excisional biopsy was performed. Subsequently, the patient developed infection, suggesting Ludwig's angina, after reevaluation, she was diagnosed with acute suppurative sialadenitis in the right mandibular gland; during the follow-up period, antibiotic therapy was introduced. Conclusion The close relationship of the biopsy site with the submandibular space and other structures of the neck required the completion of antibacterial prophylaxis associated with preoperative care, transoperative care, and postoperative care to combat opportunistic infections and their subsequent complications. Clinical Significance Suppurative sialadenitis and its subsequent complications may be considered, although rare, as a complication in cases of ranula. How to cite this article Pinheiro TN, Arid J, Maciel AP, Machado AFM, Monteiro ML, de Oliveira Daltoé M, de Carvalho FK. Acute Suppurative Sialadenitis Secondary to Excisional Ranula Biopsy in Child. Int J Experiment Dent Sci 2016;5(2):123-126.

scholarly journals Right-sided lateralization of skin temperature in healthy young persons

2016 ◽  
Vol 39 (6) ◽  
pp. 121 ◽  
Author(s):  
Havva Demirel ◽  
Arzu Bahar ◽  
Nurcan Gokgoz ◽  
Muzeyyen Arslan ◽  
Senol Dane
Keyword(s):  
Lymph Node ◽  
Immune System ◽  
Skin Temperature ◽  
Healthy Subjects ◽  
The Body ◽  
Node Distribution ◽  
Significant Difference ◽  
Close Relationship ◽  
The Right ◽  
Relationship Of

Purpose: Previous studies reported a peripheral immune asymmetry and lateralization of different paired organ cancers. Because of the close relationship of immune system with body temperature, the aim of the study was to test the possible lateralization of skin temperature. Materials and Methods: Four hundred sixty three healthy subjects (325 women, 138 men) with an average age of 23.62 years (SD = 8.65) participated in this study. All skin temperature measurements were done 8:00-9:00 a.m. Skin temperature was assessed for three different points in skin: axillary, thumb and external auditory. Results: Skin temperature was statistically significantly higher on the right side of the body than on the left side, when measured by axillary and thumb temperatures. But there was no statistically significant difference in external auditory temperature. Conclusion: The asymmetry of skin temperature may result from the peripheral immune asymmetry or the asymmetric lymph node distribution.

scholarly journals IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-16
Author(s):  
Sylvia Drazilova ◽  
Eduard Veseliny ◽  
Patricia Denisa Lenartova ◽  
Dagmar Drazilova ◽  
Jakub Gazda ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Histological Finding ◽  
Good Prognosis ◽  
High Serum ◽  
Obliterative Phlebitis ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis

IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis. It presents with signs of cholestasis and during differential diagnosis it should be distinguished from cholangiocarcinoma or from other forms of sclerosing cholangitis (primary and secondary sclerosing cholangitis). Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. The diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram. Increased serum IgG4 levels are nonspecific but are indeed a part of the diagnostic criteria proposed by the Japan Biliary Association and the HISORt criteria for IgG4-SC. High serum IgG4 retains clinical utility depending on the magnitude of elevation. Approximately 90% of patients have concomitant autoimmune pancreatitis, while 10% present with isolated biliary involvement only. About 26% of patients have other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, or IgG4-related renal lesions. A full-blown histological finding characterized by IgG4-enriched lymphoplasmacytic infiltrates, obliterative phlebitis, and storiform fibrosis is difficult to capture in practice because of its subepithelial localization. However, the histological yield is increased by immunohistochemistry, with evidence of IgG4-positive plasma cells. Based on a cholangiogram, IgG-4 related sclerosing cholangitis is classified into four subtypes according to the localization of stenoses. The first-line treatment is corticosteroids. The aim of the initial treatment is to induce clinical and laboratory remission and cholangiogram normalization. Even though 30% of patients have a recurrent course, in the literature data, there is no consensus on chronic immunosuppressive maintenance therapy. The disease has a good prognosis when diagnosed early.

scholarly journals QUANTUM KALEIDOSCOPES AND BELL'S THEOREM

2006 ◽  
Vol 20 (11n13) ◽  
pp. 1711-1729 ◽  
Author(s):  
P. K. ARAVIND
Keyword(s):  
State Estimation ◽  
Quantum State ◽  
Geometrical Structure ◽  
Quantum Tomography ◽  
Bell's Theorem ◽  
Quantum State Estimation ◽  
Close Relationship ◽  
Bell Nonlocality ◽  
The Right ◽  
Relationship Of

A quantum kaleidoscope is defined as a set of observables, or states, consisting of many different subsets that provide closely related proofs of the Bell-Kochen-Specker (BKS) and Bell nonlocality theorems. The kaleidoscopes prove the BKS theorem through a simple parity argument, which also doubles as a proof of Bell's nonlocality theorem if use is made of the right sort of entanglement. Three closely related kaleidoscopes are introduced and discussed in this paper: a 15-observable kaleidoscope, a 24-state kaleidoscope and a 60-state kaleidoscope. The close relationship of these kaleidoscopes to a configuration of 12 points and 16 lines known as Reye's configuration is pointed out. The "rotations" needed to make each kaleidoscope yield all its apparitions are laid out. The 60-state kaleidoscope, whose underlying geometrical structure is that of ten interlinked Reyes' configurations (together with their duals), possesses a total of 1120 apparitions that provide proofs of the two Bell theorems. Some applications of these kaleidoscopes to problems in quantum tomography and quantum state estimation are discussed.

scholarly journals The Utility of Serum IgG4 Concentrations as a Biomarker

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Shigeyuki Kawa ◽  
Tetsuya Ito ◽  
Takayuki Watanabe ◽  
Masahiro Maruyama ◽  
Hideaki Hamano ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
High Serum ◽  
Wall Thickening ◽  
Systemic Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Long Term Follow Up ◽  
Characteristic Features

IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation and having 6 characteristic features: (1) systemic involvement; (2) solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3) high serum IgG4 concentration >135 mg/dL; (4) abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5) a positive response to corticosteroid therapy; and (6) complications of other IgG4-related diseases. To date, most IgG4-related diseases have been recognized as extrapancreatic lesions of autoimmune pancreatitis. This paper will discuss the utility of IgG4 as a biomarker of IgG4-related diseases, including in the diagnosis of autoimmune pancreatitis and its differentiation from pancreatic cancer, in the prediction of relapse, in the long-term follow-up of patients with autoimmune pancreatitis and normal or elevated IgG4 concentrations, and in patients with autoimmune pancreatitis and extrapancreatic lesions, as well as the role of IgG4 in the pathogenesis of IgG4-related disease.

scholarly journals Tumor-induced osteomalacia: a case report

2022 ◽  
Vol 16 (1) ◽  
Author(s):  
Khalid Aligail ◽  
Joel A. Dave ◽  
Ian Louis Ross
Keyword(s):  
Bone Mineral Density ◽  
Bone Mineral ◽  
Serum Alkaline Phosphatase ◽  
Rare Condition ◽  
Fragility Fractures ◽  
High Serum ◽  
Mixed Ancestry ◽  
Mineral Density ◽  
Case Presentation ◽  
The Right

Abstract Background Tumor-induced osteomalacia is a rare, acquired paraneoplastic syndrome, including hypophosphatemia, high serum alkaline phosphatase, reduced active vitamin D, suboptimal bone mineral density, bone pain, fragility fractures, and muscle weakness. Case presentation We report a case of 74–year–old male of mixed ancestry with hypophosphatemia resistant to treatment despite optimal compliance, associated with profound reduction of bone mineral density and multiple nontraumatic fractures, including bilateral rib fractures, lower-thoracic (T11, T12) vertebrae, and two fractures involving the surgical and anatomical neck of the right humerus. We discuss an approach to identifying the underlying cause of hypophosphatemia associated with fragility fractures, and options for management of this rare condition. Conclusion Although rare, tumor-induced osteomalacia can be diagnosed if a logical stepwise approach is implemented. Surgery could be curative if the tumor is properly located and is resectable.

IgG4-related disease: an analysis of the clinicopathological spectrum: UK centre experience

2021 ◽  
pp. jclinpath-2021-207748
Author(s):  
Wajira Dassanayaka ◽  
Kanchana Sanjeewani Liyanaarachchi ◽  
Aftab Ala ◽  
Izhar N Bagwan
Keyword(s):  
Plasma Cells ◽  
High Serum ◽  
High Power Field ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Common Clinical Presentation ◽  
Characteristic Features

AimTo retrospectively evaluate the characteristic clinicopathological spectrum in patients with suspicion of IgG4-related disease (IgG4RD).MethodsWinpath histology database from January 2011 to April 2018 identified all suspected IgG4RD cases wherein IgG4 immunohistochemistry was performed. The histology slides were reviewed to categorise cases into Boston criteria groups—highly suggestive of IgG4RD, probable IgG4RD and insufficient evidence. Information regarding clinical data, treatment received, follow-up and serum IgG4 levels was obtained from medical records and AllScripts Patient Administration System (APAS) clinical database.ResultsThe study included 204 patients and the most common sites of biopsy/resection were pancreas and duodenum. The most common clinical presentation was fibroinflammatory lesion or mass/lump. On histology, 54/204 (26.47%) cases showed typical storiform fibrosis, 65/204 (32.64%) had >10 IgG4+ plasma cells per high power field and only one case showed thrombophlebitis (0.49%). There were 14/204 (6.78%) cases categorised as highly suggestive of IgG4RD; 8 of these showed high serum IgG4 levels and were managed clinically as true IgG4RD.ConclusionHistological diagnosis of IgG4RD remains challenging, as not all characteristic features are always present especially in small biopsies. Due to the novelty of its experience, fear of over diagnosis in the context of malignancy and features overlapping with diseases of similar clinical scenario, diagnosis of IgG4RD has become more puzzling. Further multicentre clinical trials/studies are advisable.

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