Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness

2014 ◽  
Vol 6 (3) ◽  
pp. 19-22
Author(s):  
Vivek Mathew ◽  
Gaurav Ashish ◽  
Amit Kumar Tyagi ◽  
Anand Kamalam Job ◽  
Ramanathan Chandrasekharan
Keyword(s):  
Differential Diagnosis ◽  
Optic Neuritis ◽  
Paranasal Sinuses ◽  
Sphenoid Sinus ◽  
The Body ◽  
Rare Entity ◽  
Pulse Methylprednisolone ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Almost All

ABSTRACT Introduction IgG4 related disease is a rare entity which can affect almost all parts of the body. The available literature of this entity related to paranasal sinuses is very limited. Presentation of case We present a case of isolated sphenoid sinus affliction by IgG4 related sclerosing disease manifesting as blindness, which recovered fully with pulsed steroid therapy. Discussion We present this case in view of developing insights toward diagnosis and management of IgG4 related sclerosing disease in paranasal sinuses, specifically the sphenoid sinus. Conclusion IgG4 disease involving paranasal sinuses (PNS) is rare entity and is managed with pulse methylprednisolone and immunomodulator drugs. Considering the limit of understanding of etiopathogenesis of this disease, surgeon needs to keep this differential diagnosis in mind. It may present atypically like isolated sinus involvement and optic neuritis.

scholarly journals Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness

2013 ◽  
Vol 5 (3) ◽  
pp. 178-181
Author(s):  
Vivek Mathew ◽  
Ramanathan Chandrasekharan ◽  
Gaurav Ashish ◽  
Amit Kumar Tyagi ◽  
Anand Kamalam Job
Keyword(s):  
Differential Diagnosis ◽  
Optic Neuritis ◽  
Paranasal Sinuses ◽  
Sphenoid Sinus ◽  
The Body ◽  
Rare Entity ◽  
Pulse Methylprednisolone ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Almost All

ABSTRACT Introduction IgG4 related disease is a rare entity which can affect almost all parts of the body. The available literature of this entity related to paranasal sinuses is very limited. Presentation of case We present a case of isolated sphenoid sinus affliction by IgG4 related sclerosing disease manifesting as blindness, which recovered fully with pulsed steroid therapy. Discussion We present this case in view of developing insights toward diagnosis and management of IgG4 related sclerosing disease in paranasal sinuses, specifically the sphenoid sinus. Conclusion IgG4 disease involving paranasal sinuses (PNS) is rare entity and is managed with pulse methylprednisolone and immunomodulator drugs. Considering the limit of understanding of etiopathogenesis of this disease, surgeon needs to keep this differential diagnosis in mind. It may present atypically like isolated sinus involvement and optic neuritis. How to cite this article Chandrasekharan R, Mathew V, Ashish G, Tyagi AK, Job AK. Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness. Int J Otorhinolaryngol Clin 2013;5(3):178-181.

Serum IgG4 levels outperform IgG4/IgG RNA ratio in differential diagnosis of IgG4-related disease

2020 ◽  
Author(s):  
L Schulte ◽  
F Arnold ◽  
F Siegel ◽  
J Backhus ◽  
L Perkhofer ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

Establishment of a serum IgG4 cut-off value for the differential diagnosis of IgG4-related disease in Chinese population

2015 ◽  
Vol 26 (4) ◽  
pp. 583-587 ◽  
Author(s):  
Ping Li ◽  
Hua Chen ◽  
Chuiwen Deng ◽  
Ziyan Wu ◽  
Wei Lin ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Chinese Population ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

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.

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 Adult Onset Asthma and Periocular Xanthogranuloma (AAPOX), a Rare Entity With a Strong Link to IgG4-Related Disease

Medicine ◽  
2015 ◽  
Vol 94 (43) ◽  
pp. e1916 ◽  
Author(s):  
Jonathan London ◽  
Antoine Martin ◽  
Michael Soussan ◽  
Isabelle Badelon ◽  
Thomas Gille ◽  
...  
Keyword(s):  
Rare Entity ◽  
Adult Onset ◽  
Strong Link ◽  
Related Disease ◽  
Igg4 Related Disease

Clinical case of membranous nephropathy in a patient with IgG4-related disease

2020 ◽  
Vol 24 (4) ◽  
pp. 110-121
Author(s):  
M. O. Pyatchenkov ◽  
O. A. Vorobyeva ◽  
A. N. Belskykh ◽  
M. V. Zakharov ◽  
M. Y. Dendrikova
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
The Body ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pathologic Features ◽  
Wide Range ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) currently is considered as a chronic fibroinflammatory immune-mediated multisystemic condition of unidentified etiology, which can imitate a wide range of malignant, infectious, rheumatologic, and other diseases. It can affect almost any organ system in the body synchronously or sequentially, but the most often affected are the pancreas, hepatobiliary tract, periorbital structures, salivary glands, kidneys, and lymph nodes. The most frequent renal manifestations of IgG4-RD is IgG4-related tubulointerstitial nephritis. Membranous nephropathy is the most common glomerular disease accompanied by IgG4-RD. Regardless of the organ localization, patients with IgG4-RD are characterized by elevated serum IgG4, but this laboratory abnormality is not specific and can be changed in other diseases. In all suspected cases of IgG4-RD the diagnosis should be confirmed by histological examination. Characteristic pathologic features include diffuse or focal lymphoplasmacytic infiltration with prominent IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. Patients with IgG4- RD usually have an excellent clinical response to glucocorticoids, but relapse rates after steroid withdrawal are high, which may require additional use of immunosuppressants or rituximab. Due to the low prevalence and multitude of clinical manifestations the disease often remains underdiagnosed on time. This case report describes middle-aged patients with a history of chronic recurrent pancreatitis complicated by the nephrotic syndrome. Kidney biopsy showed membranous nephropathy and diagnosis IgG4-RD with multiorgan involvement was made. Partial remission was achieved on corticosteroid therapy. The presented case clearly demonstrates the difficulties of diagnosis and treatment of IgG4-RD. IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with nephrotic syndrome accompanied by multiorgan dysfunction.

Sign in / Sign up

Export Citation Format

Share Document