scholarly journals Chronic sclerosing sialadenitis IgG4-related in a boy with recurrent parotitis: a case report

2021 ◽  
Author(s):  
Fabio Stefano Timeus ◽  
Mario Michele Calvo ◽  
Anna Maria Caci ◽  
Giogio Oliviero Gallone ◽  
Federico Vittone
Keyword(s):  
Plasma Cells ◽  
White Blood Cells ◽  
Anaerobic Bacteria ◽  
Final Diagnosis ◽  
Low Grade ◽  
Obliterative Phlebitis ◽  
Cervical Lymph Nodes ◽  
Chronic Sclerosing Sialadenitis ◽  
Igg4 Related Disease ◽  
Serum Igg4

Abstract BackgroundIgG4-related diseases are a group of immune-mediated diseases characterized by a lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. We report here a case of an unexpected diagnosis of IgG4-related sialoadenitis in a boy previously diagnosed as affected by juvenile recurrent parotitis.Case presentationA six-year old boy presented to our centre with left parotid swelling about 4x3 cm, tender, soft in consistency, with the overlying skin red and warm. He had low-grade fever and a mild enlargement of left cervical lymph nodes. In the last two years he had five episodes of parotitis and a diagnosis of juvenile recurrent parotitis.. The general conditions were good, and the remainder of the physical examination was normal. At the ultrasonography left parotid was enlarged, inhomogeneous, with a colliquated intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests were normal, except for an increase of white blood cells and positivity for IgM and IgG anti-VCA, with anti-EBNA e anti-EA I negative. The patient was treated with cephalosporins and azitromycin, but after 10 days the parotid became fluctuating and the patient underwent to drainage and biopsy of the gland. He was discharged after 3 days of observation, without any complications, and treated with a further course of cefpodoxime and betametasone, with a good clinical response.Investigations for aerobic and anaerobic bacteria, mycobacteria and Bartonella on the pus were negative. Histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltration. The plasma cells were polyclonal and mostly expressed IgG4 subclass immunoglobulins. The final diagnosis was chronic sclerosing sialadenitis IgG4-related in recurrent parotitis and recent EBV infection. ConclusionsThe interest of this case comes from the rarity of IgG4-related sialadenitis in children. It is not possible to argue with certainty that the previous episodes of parotitis were manifestations of a IgG4-related disease, due to the lack of histological data. Serum IgG4 in our patient were normal, as described in literature in 30% of cases.

scholarly journals IgG4-related chronic sclerosing sialadenitis in a child with recurrent parotitis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Fabio Timeus ◽  
Mario Michele Calvo ◽  
Anna Maria Caci ◽  
Giorgio Oliviero Gallone ◽  
Federico Vittone
Keyword(s):  
Plasma Cells ◽  
White Blood Cells ◽  
Normal Serum ◽  
Oral Antibiotic ◽  
Obliterative Phlebitis ◽  
Surgical Biopsy ◽  
Cervical Lymph Nodes ◽  
Juvenile Recurrent Parotitis ◽  
Chronic Sialadenitis ◽  
Serum Igg4

Abstract Background IgG4-related disease (IgG4-RD) includes a group of immune-mediated diseases histologically characterized by lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. Here we report the case of IgG4-related sialadenitis in a six-year-old patient previously diagnosed as juvenile recurrent parotitis. Case presentation A six-year-old patient was referred to our Centre for left parotid swelling of 4 × 3 cm, that was tender, soft in consistency, with overlying red and warm skin. His general condition was good but he was subfebrile; general examination revealed mild enlargement of left cervical lymph nodes. In the last 2 years he had had five episodes of parotitis, diagnosed by another pediatric Center as juvenile recurrent parotitis. On ultrasound examination the left parotid gland appeared enlarged, inhomogeneous, with a colliquative intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests showed an increase of white blood cells and anti-VCA IgM and IgG positivity, with anti-EBNA e anti-EA I negativity. The patient was initially treated with oral antibiotics, but after 10 days the parotid became fluctuating, requiring surgical biopsy and drainage. Postoperative course was regular, with complete remission under oral antibiotic and steroid therapy. Microbiological tests, including cultures for aerobic and anaerobic bacteria, mycobacteria and Bartonella, were negative. Surprisingly, histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltrate with polyclonal plasma cells mostly expressing IgG4 immunoglobulins. Thus, the diagnosis of IgG4 related chronic sialadenitis in recurrent parotitis and recent EBV infection was made. Conclusions IgG4-related sialadenitis is very unusual in children. Histology plays a key role in diagnosis, considering that up to 30% of patients have normal serum IgG4 levels, as shown in our case. The lack of previous histological data makes it impossible to attribute our patient’s previous episodes of parotitis to IgG4-RD, though it is a very consistent possibility.

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 IgG4-Related Disease in a Urachal Tumor

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Travis W. Dum ◽  
Da Zhang ◽  
Eugene K. Lee
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Needle Aspiration ◽  
Lung Lesion ◽  
High Rate ◽  
Mediastinal Lymphadenopathy ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease is a newly recognized fibroinflammatory disorder that has the ability to affect nearly every organ system. It is characterized by tumefactive lesions and fibrosis and closely mimics neoplasms. Only one case of IgG4-related bladder mass has been reported in the literature, but there are no reports of IgG4-related disease in a urachal mass. Herein, we report a 26-year-old male who initially presented with symptoms of recurrent UTI. Work-up revealed a 6 cm urachal tumor, a 1.4 cm pulmonary lesion, and mediastinal lymphadenopathy; all metabolically active on PET scan and suspicious for urachal adenocarcinoma. Lung lesion fine needle aspiration and TURBT pathology revealed inflammation but no evidence of malignancy. The patient underwent a partial cystectomy and umbilectomy with pathology demonstrating dense plasmacytic cells, a high rate of immunohistochemistry staining positive for IgG4 plasma cells, a storiform pattern of fibrosis, and an obliterative phlebitis. Furthermore, the patient had an elevated serum IgG4 level of 227 mg/dL (range 2.4–121 mg/dL). IgG4-related disease is a newly recognized fibroinflammatory disorder that can mimic neoplastic processes and a high index of suspicion and accurate tissue pathology is necessary for an accurate diagnosis.

scholarly journals The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease

2020 ◽  
Vol 21 (14) ◽  
pp. 5082
Author(s):  
Song-Chou Hsieh ◽  
Chieh-Yu Shen ◽  
Hsien-Tzung Liao ◽  
Ming-Han Chen ◽  
Cheng-Han Wu ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Malignant Neoplasms ◽  
Th2 Response ◽  
Obliterative Phlebitis ◽  
Inflammatory Reactions ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) is a spectrum of complex fibroinflammatory disorder with protean manifestations mimicking malignant neoplasms, infectious or non-infectious inflammatory process. The histopathologic features of IgG4-RD include lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis together with increased in situ infiltration of IgG4 bearing-plasma cells which account for more than 40% of all IgG-producing B cells. IgG4-RD can also be diagnosed based on an elevated serum IgG4 level of more than 110 mg/dL (normal < 86.5 mg/mL in adult) in conjunction with protean clinical manifestations in various organs such as pancreato–hepatobiliary inflammation with/without salivary/lacrimal gland enlargement. In the present review, we briefly discuss the role of genetic predisposition, environmental factors and candidate autoantibodies in the pathogenesis of IgG4-RD. Then, we discuss in detail the immunological paradox of IgG4 antibody, the mechanism of modified Th2 response for IgG4 rather than IgE antibody production and the controversial issues in the allergic reactions of IgG4-RD. Finally, we extensively review the implications of different immune-related cells, cytokines/chemokines/growth factors and Toll-like as well as NOD-like receptors in the pathogenesis of tissue fibro-inflammatory reactions. Our proposals for the future investigations and prospective therapeutic strategies for IgG4-RD are shown in the last part.

scholarly journals Needle biopsy compared with surgical biopsy: pitfalls of small biopsy in histologial diagnosis of IgG4-related disease

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Yanying Liu ◽  
Fei Yang ◽  
Xiying Chi ◽  
Yuxin Zhang ◽  
Jiangnan Fu ◽  
...  
Keyword(s):  
Needle Biopsy ◽  
Plasma Cells ◽  
Classification Criteria ◽  
Histological Diagnosis ◽  
Obliterative Phlebitis ◽  
Surgical Biopsy ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Significant Difference ◽  
Storiform Fibrosis

Abstract Objective The growing utilization of needle biopsy has challenged the current pathology consensus of IgG4-related disease (IgG4-RD). The aims of this study were to identify the histological characteristics of needle biopsy and surgical specimens and evaluate the ability of needle biopsy in histological diagnosis of IgG4-RD. Methods Biopsies from patients who were referred to as IgG4-RD by the 2019 ACR/EULAR IgG4-RD classification criteria in Peking University People’s Hospital from 2012 to 2019 were re-evaluated. Typical histological features and diagnostic categories were compared between needle biopsy and surgical biopsy. Results In total, 69 patients met the 2019 ACR/EULAR classification criteria and 72 biopsies of them were re-evaluated. All cases showed lymphoplasmacytic infiltrate, while storiform fibrosis and obliterative phlebitis were only present in 35 (48.6%) and 23 (31.9%) specimens, respectively. Storiform fibrosis was more likely to be seen in retroperitoneum lesion (P = 0.033). Surgical biopsy showed significantly higher IgG4+ plasma cells/high-power field (IgG4/HPF) count (P < 0.01) and higher proportion of IgG4/HPF > 10 (P < 0.01). No significant difference was observed with regard to the ratio of IgG4+ plasma cells/IgG+ plasma cells (IgG4/IgG) (P = 0.399), storiform fibrosis (P = 0.739), and obliterative phletibis (P = 0.153). According to the 2011 comprehensive diagnostic criteria, patients who performed a needle biopsy were less likely to be probable IgG4-RD (P = 0.045). Based on the 2011 pathology consensus, needle biopsy was less likely to be diagnosed as IgG4-RD (P < 0.01), especially to be highly suggestive IgG4-RD (P < 0.01). Only 1/18 (5.6%) needle salivary specimens fulfilled the cutoff of IgG4/HPF > 100, which was significantly less than 15/23 (65.2%) of surgical ones (P < 0.01). Conclusions Needle biopsy shows an inferiority in detecting IgG4/HPF count but not in IgG4/IgG ratio, storiform fibrosis, and obliterative phlebitis. Compared with surgical samples, needle biopsy is less likely to obtain a histological diagnosis of IgG4-RD. A different IgG4/HPF threshold for needle biopsy of the salivary glands may be considered.

scholarly journals Ossifying fibrous epulis as an IgG4-related disease of the oral cavity: a case report and literature review

2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Yoshiko Ike ◽  
Takahiro Shimizu ◽  
Masaru Ogawa ◽  
Takahiro Yamaguchi ◽  
Keisuke Suzuki ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Lymph Nodes ◽  
Plasma Cells ◽  
Maxillofacial Surgery ◽  
The Body ◽  
Pathological Diagnosis ◽  
Cervical Lymph Nodes ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Organ Manifestations

Abstract Background Fibrous sclerosing tumours and hypertrophic lesions in IgG4-related disease (IgG4-RD) are formed in various organs throughout the body, but disease in the oral region is not included among individual organ manifestations. We report a case of ossifying fibrous epulis that developed from the gingiva, as an instance of IgG4-RD. Case presentation A 60-year-old Japanese man visited the Department of Oral and Maxillofacial Surgery, Gunma University Hospital, with a chief complaint of swelling of the left mandibular gingiva. A 65 mm × 45 mm pedunculated tumour was observed. The bilateral submandibular lymph nodes were enlarged. The intraoperative pathological diagnosis of the enlarged cervical lymph nodes was inflammation. Based on this diagnosis, surgical excision was limited to the intraoral tumour, which was subsequently pathologically diagnosed as ossifying fibrous epulis. Histopathologically, the ossifying fibrous epulis exhibited increased levels of fibroblasts and collagen fibres, as well as infiltration by numerous plasma cells. The IgG4/IgG cell ratio was > 40%. Serologic analysis revealed hyper-IgG4-emia (> 135 mg/dL). The patient met the comprehensive clinical diagnosis criteria and the American College of Rheumatology and European League Against Rheumatism classification criteria for IgG4-RD. Based on these criteria, we diagnosed the ossifying fibrous epulis in our patient as an IgG4-related disease. A pathological diagnosis of IgG4-related lymphadenopathy was established for the cervical lymph nodes. Concomitant clinical findings were consistent with type II IgG4-related lymphadenopathy. Conclusions A routine serological test may be needed in cases with marked fibrous changes (such as epulis) in the oral cavity and plasma cells, accompanied by tumour formation, to determine the possibility of individual-organ manifestations of IgG4-related disease.

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

Thoracic Involvement in IgG4-Related Disease

2020 ◽  
Vol 41 (02) ◽  
pp. 202-213 ◽  
Author(s):  
Marta Casal Moura ◽  
Ria Gripaldo ◽  
Misbah Baqir ◽  
Jay H. Ryu
Keyword(s):  
Clinical Laboratory ◽  
Wide Spectrum ◽  
The Body ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
First Line Therapy ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Histopathologic Features

AbstractImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that has been recognized to involve virtually any organ in the body and typically manifests mass-like lesions (tumefactive). Although initial reports of this disease (autoimmune pancreatitis [AIP]) were described in the Japanese population, it has since been reported worldwide. It is most commonly seen in adults of middle age or older, more often men than women. The pathogenesis of IgG4-RD is largely unknown, but genetic factors, microorganisms, and autoimmunity are thought to play important roles. Serum IgG4 concentration is elevated in the majority of patients with IgG4-RD but is a nonspecific finding. Characteristic histopathologic features include dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis. Lung involvement in IgG4-RD was first reported in 2004 in two patients with AIP and coexisting interstitial lung disease. Since then, a wide spectrum of intrathoracic involvement has been reported and includes not only parenchymal lung diseases but also pleural, airway, vascular, and mediastinal lesions. Thoracic involvement in IgG4-RD is often found incidentally during the workup of extrathoracic lesions but can sometimes be the presenting abnormality. The diagnosis of IgG4-RD requires correlation of clinical, laboratory, imaging, and histopathologic features. Glucocorticoids are the first-line therapy but other options including B cell depletion are being investigated. IgG4-RD is generally associated with an indolent clinical course and most patients improve with glucocorticoid therapy.

scholarly journals A case report of Rosai Dorfman disease

2018 ◽  
Vol 4 (3) ◽  
pp. 868
Author(s):  
Jyotika Waghray ◽  
Pradyut Waghray
Keyword(s):  
Lymph Node ◽  
Plasma Cells ◽  
Final Diagnosis ◽  
Needle Aspiration ◽  
Lymph Node Involvement ◽  
Unknown Etiology ◽  
Low Grade ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Node Involvement

<p>Rosai-Dorfman’s disease also known as sinus histiocytosis with massive lymphadenopathy (SHML) is characterized by distorted lymph node architecture with marked dilation of lymphatic sinuses occupied by numerous lymphocytes, as well as histiocytes with vesicular nucleus and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as ‘emperipolesis’. This disease of unknown etiology progresses with a benign prognosis strictly and only when an early diagnosis and treatment is made. A late diagnosis and a generalized lymph node involvement contribute to a poor prognosis. We reported a case of a 29-year-old Indian female with a 4-month history of painful unilateral cervical mass and low-grade fever with the final diagnosis of Rosai-Dorfman disease. The final diagnosis was made by fine needle aspiration (FNA) biopsy of the cervical lymph node. In conclusion, FNA biopsy can be enough to make the diagnosis in most cases due to the distinct cytological features of SHML, thereby avoiding more invasive approaches that potentially are unnecessary.</p>

scholarly journals Urologic manifestations of IgG4-related disease

2020 ◽  
Vol 80 (5) ◽  
pp. 1-10
Author(s):  
Benjamin Enrique Montaño Roca ◽  
Davide Vanacore ◽  
Gustavo Gallegos Sánchez ◽  
César Eduardo Rosales Velázquez ◽  
Guillermo Enrique Ruvalcaba Oceguera ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Aged Patients ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Urinary Tract Symptoms ◽  
Serum Igg4 ◽  
Inflammatory Pseudotumors ◽  
Clinically Significant

IgG4-related disease (IgG4-RD) is a clinical entity characterized by elevated serum IgG4 and tumor-like inflammation, with tissue infiltration by IgG4 and plasma cells. IgG4-RD is rare, but clinically significant, and its urologic manifestations have been reported in the literature. The present review covers a broad spectrum, describing the pathologies related to the area of urology. In 2003, Terumi Kamisawa was the first to recognize IgG4-RD, characterized by multiorgan lesions in patients with autoimmune pancreatitis and classified as an inflammatory and fibrotic entity with a dense lymphoplasmacytic infiltrate, positive for  IgG4.(1–3) It presents in middle-aged patients, between 59-68 years of age, with no clear distribution by sex, (4–6) and has different clinical presentations. The main urologic manifestations are inflammatory pseudotumors and lower urinary tract symptoms. The present article offers a clear, general overview of the disease, encompassing its pathophysiology, diagnosis, and treatment, from the perspective of urology. 

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