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Journal of Otolaryngology - Head & Neck... Jun 2015Immunoglobulin G4 related disease (IgG4-RD) is a poorly understood chronic inflammatory disorder affecting the middle-aged and elderly that can present to the... (Review)
Review
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.
Topics: Adrenal Cortex Hormones; Autoimmune Diseases; Chronic Disease; Diagnosis, Differential; Humans; Immunoglobulin G; Inflammation; Otorhinolaryngologic Diseases
PubMed: 26092582
DOI: 10.1186/s40463-015-0071-9 -
Medicine Mar 2015This article aims to perform a meta-analysis to evaluate the diagnostic value of the immunoglobulin G (IgG)4/IgG ratio of plasmacytic infiltration for IgG4-related... (Meta-Analysis)
Meta-Analysis Review
This article aims to perform a meta-analysis to evaluate the diagnostic value of the immunoglobulin G (IgG)4/IgG ratio of plasmacytic infiltration for IgG4-related diseases.Four databases-EMBASE, ISI Web of Knowledge, PubMed, and the Cochrane Library-were systematically searched. Approximately 200 participants from several studies were included in this research. STATA 11.2 software (Stata Corporation, College Station, TX) and Meta-DiSc 1.4 (Unit of Clinical Biostatistics, Ramon y Cajal Hospital, Madrid, Spain) were used to perform the meta-analysis.Nine studies were included in the meta-analysis. The pooled diagnostic odds ratio was 18.94 [95% confidence interval (CI), 2.89-124.30]. The sensitivity was 58.80% (95% CI, 50.90-66.30) and the specificity was 90.20% (95% CI, 81.20-95.80). The positive and negative likelihood ratios were 3.12 (95% CI, 1.07-9.16) and 0.26 (95% CI, 0.09-0.70), respectively. The area under the curve of the summary receiver-operating characteristic was 0.88.To conclude, the IgG4/IgG ratio of plasmacytic infiltration is modestly effective in diagnosing IgG-related disease.
Topics: Biomarkers; Cholangitis, Sclerosing; Humans; Immunoglobulin G; Mikulicz' Disease; Nephritis, Interstitial; Odds Ratio; ROC Curve; Skin Diseases; Spain
PubMed: 25738476
DOI: 10.1097/MD.0000000000000579