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203 results on '"igg4-rd"'

201. IgG4-related disease: a novel, important but easily missed condition.

Author

Lees JS, Church N, Langdale-Brown B, Bellamy C, Gibson P, and Watson S

Subjects
Adrenal Cortex Hormones therapeutic use, Aged, Autoimmune Diseases drug therapy, Autoimmune Diseases immunology, Autoimmune Diseases pathology, Female, Fibrosis immunology, Humans, Inflammation blood, Inflammation diagnosis, Inflammation drug therapy, Japan, Kidney Diseases blood, Kidney Diseases drug therapy, Kidney Diseases pathology, Male, Middle Aged, Pancreatitis blood, Pancreatitis drug therapy, Pancreatitis pathology, Plasma Cells pathology, Retroperitoneal Fibrosis blood, Retroperitoneal Fibrosis drug therapy, Salivary Gland Diseases blood, Salivary Gland Diseases drug therapy, Salivary Gland Diseases pathology, Immunoglobulin G blood, Inflammation immunology, Kidney Diseases immunology, Pancreatitis immunology, Plasma Cells immunology, Retroperitoneal Fibrosis immunology, Salivary Gland Diseases immunology
Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a multisystem, fibroinflammatory condition unrecognised in medical science until the last decade. It is characterised by progressive scarring and dysfunction of affected organs and tissues including the pancreas, hepatobiliary tree, kidneys, salivary glands, retroperitoneum and lungs. The diagnosis is made with the presence of numerous IgG4 positive plasma cells within a histologically-distinct chronic inflammatory process; most patients also have elevated serum IgG4. Though early cases were all identified in Japan, subsequent reports clearly demonstrate that IgG4-RD exists worldwide. There are no data confirming the prevalence of IgG4-RD in the West but it is thought to be very rare. Limited awareness of the condition and its heterogeneous presentation frequently results in misdiagnosis. Prompt and correct diagnosis is critical, as a rapid reversal of even advanced disease is often seen with corticosteroid therapy. We present three cases that illustrate some of the typical features of this condition.

Published
2013
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203. Immunoglobulin G4-related diseases in the head and neck: a systematic review

Author

David W. J. Côté, Caroline C. Jeffery, Paras Satija, and Graeme B. Mulholland

Subjects
medicine.medical_specialty, Disease, Salivary glands, Autoimmune Diseases, Diagnosis, Differential, Head and neck, Adrenal Cortex Hormones, parasitic diseases, Eosinophilic, Humans, Medicine, Original Research Article, Inflammation, business.industry, Dacryoadenitis, Lymphandenopathy, Lacrimal glands, medicine.disease, Dermatology, Sialadenitis, Surgery, Otorhinolaryngologic Diseases, Otorhinolaryngology, Immunoglobulin G, Chronic Disease, IgG4-RD, Systematic review, Oral and maxillofacial surgery, IgG4-related disease, Differential diagnosis, business
Abstract

Background Immunoglobulin G4 related disease (IgG4-RD) is a poorly understood chronic inflammatory disorder affecting the middle-aged and elderly that can present to the otolaryngologist. We aim to summarize the current literature regarding the manifestations and management of IgG4-RD in the head and neck. Methods Pubmed and EMBASE were searched using the term relevant search algorithm utilizing keywords such as: IgG4 related disease, head and neck, orbit, salivary glands, sialadenitis, Kuttner, angiocentric eosinophilic fibrosis, submandibular, lacrimal, thyroid, dacryoadenitis, nasal, sinus, and Mikulicz’s. Reference lists were searched for identification of relevant studies. Case reports, original research and review articles published in English from 1964 to 2014 whose major topic was IgG4-RD affecting the head and neck were included. Data regarding patient demographics, presentation, histopathology, management and treatment outcomes of IgG4-RD were extracted. Level of evidence was also assessed and data were pooled where possible. Three independent reviewers screened eligible studies; extracted relevant data and discrepancies were resolved by consensus, where applicable. Descriptive and comparative statistics were performed. Results Fourty-three articles met our inclusion criteria. IgG4-RD most often presents as a mass lesion in the head and neck region. Common diagnostic features include: 1) elevated serum IgG4 level, 2) marked infiltration of exocrine glands by IgG4-positive plasma cells with fibrosis, and 3) marked improvement with corticosteroid therapy and additional immunosuppressive therapy in corticosteroid refractory cases. Early diagnosis and involvement of rheumatology is important in management. Conclusions IgG4-RD is a challenging non-surgical disease that has multiple manifestations in the head and neck. It must be distinguished from various mimics including malignancy, systemic diseases, and infectious. Otolaryngology-Head and Neck surgeons should be aware of this condition and its management.

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