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ObjectiveIgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes.MethodsWe used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis.ResultsIn the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1–3 (316, 178 and 445 mg/dL, respectively, p<0.001).ConclusionWe identified four distinctive IgG4-RD phenotypes according to organ involvement. Being Asian or female may predispose individuals to head and neck-limited disease. These phenotypes serve as a framework for identifying IgG4-RD and studying its aetiology and optimal treatment.

The human microbiome is increasingly recognized as a key factor in immune development and disease susceptibility, especially in early life. Nasal microbiome has emerged as a critical element in upper airway health, yet its role in pediatric otorhinolaryngological conditions remains underexplored. This narrative review synthesizes current evidence on the microbial nasopharyngeal patterns in healthy children compared with children suffering from chronic ENT conditions such as otitis media, allergic rhinitis, chronic rhinosinusitis, adenoid and tonsillar hypertrophy associated with obstructive sleep apnea. A structured search of Web of Science, PubMed, Google Scholar and CrossRef databases was conducted for peer-reviewed articles published in the past ten years. Nasal microbiota of healthy children was proved to be dominated by commensal protective taxa such as Dolosigranulum and Corynebacterium which contribute to mucosal immune stability. In contrast, patients with chronic ENT pathologies exhibited reduced diversity and increased prevalence of potential pathogens microbial species such as Haemophilus , Streptococcus and Staphylococcus. Several extrinsic factors appear to play an important role in modulating the nasal microbiota such as environmental exposure, delivery mode, feeding practices and antibiotic treatment. Growing evidence supports the predictive and modulatory potential of the nasal microbiome, however methodological variability, limited pediatric-specific studies and unclear causal relationships remain challenging components. This review highlights key microbial patterns, outlines the limitations of current research and suggests future directions for clinical integration of nasal microbiome analysis in pediatric ENT standard of care as it may hold promising utilisation of biomarkers for disease risk stratification and targeted therapeutic or preventative interventions in early life.

The study population comprised 29 patients with EGPA, evaluated during active and inactive disease (patients’ characteristics are reported in the online supplemental table 1), 30 patients with atopic asthma, 31 with active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (20 with granulomatosis with polyangiitis and 11 with microscopic polyangiitis) and 30 healthy controls (online supplemental methods). (B) Reactivity for the different allergen families (food allergens, panel a; respiratory allergens, panel b; other allergens, panel c) in active and inactive EGPA patients (rose and blue colour, respectively) and in the control groups (orange for asthmatic patients, violet for healthy controls and green for AAV patients). Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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.

Background: The global HIV/AIDS epidemic, with its significant presence in Vietnam by the early 2000s, often linked to injecting drug use, prompted concern at the National Otorhinolaryngology Hospital due to a rising prevalence of HIV among ENT patients. This highlighted a need to understand their specific clinical and laboratory profiles. Objective: This study aimed to systematically evaluate selected biochemical, hematological, and immunological parameters in these HIV-positive ENT patients to characterize their infection and identify significant laboratory alterations. Methods: This retrospective-prospective cohort study included 104 HIV-positive ENT patients from January 2010 to August 2014. HIV diagnoses were confirmed via a multi-test approach. Demographic, risk behavior, and ENT diagnosis data were collected from medical records. Biochemical, hematological, and immunological (CD4, CD8, CD4/CD8 ratio) parameters were analyzed. Results: The cohort was predominantly young adult males (85.55%), with injecting drug use as the main risk factor (79.04%). Over half (53.60%) had HIV-related ENT conditions; 46.40% were incidentally diagnosed. Biochemical analysis showed significantly elevated total protein/globulin and reduced albumin/A/G ratio (p < 0.05). Hematologically, leukopenia (7.4% males) and mild/moderate anemia (20.4% males, 33.3% females) were observed. Immunological profiling revealed significantly decreased CD4 counts (264±89.57 cells/mm³) and CD4/CD8 ratios, alongside increased CD8 counts (all p < 0.05). Conclusion: HIV-positive ENT patients in Vietnam exhibit typical demographic/risk profiles and frequent HIV-related ENT manifestations. Significant biochemical, hematological, and profound immunological abnormalities underscore HIV’s systemic impact. ENT clinics are crucial for early HIV detection, necessitating comprehensive laboratory monitoring for effective disease management.

Immunoglobulin G4 related disease is an inflammatory condition characterised by the presence of fibrotic lesions infiltrated by immunoglobulin G4 positive plasma cells. It can arise from almost any region of the body and it is being increasingly recognised in the head and neck. Regardless of the site of involvement, the histopathological resemblance is remarkable. Dense lymphoplasmacytic infiltration, overabundance of immunoglobulin G4 bearing plasma cells and presence of storiform fibrosis are typical findings. This paper presents two cases of immunoglobulin G4 related disease in which there was involvement of the orbit, the infraorbital nerve and the infratemporal fossa. Diagnosis was established in both cases by biopsying radiologically abnormal tissue in the infratemporal fossa. An awareness of this condition is required to establish the diagnosis and initiate appropriate therapy. Glucocorticoids are the mainstay of initial treatment. The effectiveness of B-lymphocyte depletion with rituximab has also been reported. Correct diagnosis may spare patients from unnecessarily radical surgery.

The origins of immunology and allergy are founded upon the early 19th century microbiological studies of Jenner and Pasteur. It was discovered that the immune system could cause harm. The subspecialty of allergy began with the coining of the term by Von Pirquet in 1906 to describe disorders resulting from hyper-reaction to normally innocuous environmental agents. Understanding the scientific basis of the immune system and allergy allowed Noon and Freeman, and later Cooke, to develop allergen immunotherapy. Initially the technique was crude, but with the subsequent key discovery of IgE, more accurate methods of diagnosis (such as the radioallergosorbent test (RAST)) and treatment ensued. The efficacy of specific immunotherapy has been demonstrated by many double-blind trials culminating in the WHO position paper. DNA recombinant technology has provided detailed molecular understanding of allergic disorders, which has resulted in several novel methods of immunotherapy that are potentially safer and more effective. Use of recombinant allergens, T-cell peptides, DNA vaccination with CpG motifs or plasmid vectors and anti-IgE strategies with monoclonal antibodies are showing promise.

Psycho-neuroimmunology depicts a conceptual frame in which possible interactions between psychic and physical processes can be examined. It could be very significant in the field of psychosomatics when the courses of psychic and somatic processes are examined. However, the research results from this field of study are varied and only for a few parameters of immunity is it possible to prove correlations with psychic variables. Many of the studies that have been conducted up to date were construed as cross-section studies and possibly therefore are not very suitable for depicting the probably very complicated forms of interactions between psychic and somatic levels in an adequate manner. In the framework of stationary psychosomatic psychotherapy two single case studies were carried out in order to examine temporal connections between psychic and immunological course parameters. Both single case studies are to be viewed as explorative attempts of examining questions of examination design and organization which are highly resolved regarding time. Furthermore we report several interesting individual results which emphasize in general the correlation between psychic and somatic parameters also in the course of time. However the limits of such studies regarding the significance of the individual immunological parameters, using time serial analytical methods as well as constructing models in the field of psycho-neuroimmunology are discussed.

In patients with chronic severe rhinosinusitis and nasal polyps, tezepelumab therapy led to greater reductions in polyp size and nasal congestion and less use of surgery and glucocorticoids than placebo.