Unilateral bamboo node of the vocal fold associated with anti-SS-A and anti-SS-B antibody.

Voice disorder is occasionally associated with systemic autoimmune diseases. Bamboo nodes of the vocal fold have a characteristic bamboo-shaped appearance and strongly indicate the presence of an underlying autoimmune disorder. Both mechanical and immunologic mechanisms are assumed to be involved in the pathogenesis of vocal disorder. We present a 27-year-old woman with hoarseness, sore throat, and a unilateral bamboo node of the vocal fold. Serum anti-SS-A and -SS-B antibodies were positive, but she had no systemic signs or symptoms suggestive of Sjögren's syndrome. Oral systemic glucocorticoid treatment was not effective, but surgical resection improved her hoarseness. Histopathologic findings of the resected vocal node revealed fibrosis with hyaline degeneration. Thereafter, she had no recurrence of hoarseness for 2 years. Bamboo nodes of the vocal fold may occur without definitive autoimmune diseases, although immunologic abnormalities such as autoantibody-positivity may occur.

Bamboo Nodes on a Series of 15 Patients: Vocal Fold Lesion as a Sign of Autoimmune Disease and Microphonotrauma.

OBJECTIVES: Bamboo nodes are band-like submucosal deposits of the middle third of the vocal fold. They are often related to connective tissue disorders, but can also precede them. The aim of this study was to report our experience with conservative treatment of those rare lesions. METHODS: This is a retrospective series of 15 patients consulting for hoarseness and presenting bamboo nodes from 2010 to 2016. RESULTS: All patients were women of mean age of 38 years with a moderate or high degree of daily vocal effort. Nine patients (60%) presented with known autoimmune disease at the phoniatric appointment. The other patients (40%) benefited from a systematic biological research for autoimmune disease, which retrieved two poorly symptomatic connective tissue disorders. Patients were clinically improved by speech therapy (53%) or by an optimization or introduction of immunosuppressive treatment (46%). A spontaneous improvement was observed for three patients after voice rest (one after retirement, one after professional change, and last one after resuming professional singing). In our series, no phonosurgery was performed. The vocal profile at last appointment found a moderate Voice Handicap Index at 35.3/120, a low maximum time of phonation at 13.6 seconds, and a high jitter at 1.4, sign of instability of the vibrator. CONCLUSION: This series emphasizes the importance of diagnosing bamboo nodes in middle-aged female presenting an autoimmune disease. Vice versa for each patient with bamboo nodes, a systematic autoimmune check-up has to be realized to detect a biological asymptomatic autoimmune disease.

Long-term control of laryngeal plasma cell mucositis with systemic immunosuppression.

Plasma cell mucositis (PCM) is a rare non-neoplastic plasma cell proliferative disorder of the mucous membranes, which typically presents as soft tissue lesions involving oral, upper airway or genital mucosa. Laryngeal involvement resulting in stridor has been reported in four other cases previously, with three requiring tracheostomy. We present a case of supraglottic stenosis in a 53-year-old woman presenting with dysphonia and stridor, requiring surgical resection on three occasions accompanied by tracheostomy on two occasions; biopsy was consistent with PCM. Due to relapsing disease activity, high-dose prednisolone and mycophenolate mofetil were commenced with prednisolone eventually being ceased. After 2 years of mycophenolate mofetil therapy, the patient's disease has been controlled without need for further surgical intervention. This is the first reported case of prolonged symptomatic improvement with the use of systemic immunosuppressive therapy with mycophenolate mofetil in PCM.

Bamboo Nodes of Vocal Folds: A Description of 10 Cases and Review of the Literature.

OBJECTIVE: Bamboo nodes are vocal fold lesions, mostly associated with autoimmune diseases. PATIENTS AND METHODS: This is a retrospective clinical study including 10 patients with bamboo nodes. Data were collected regarding associated autoimmune disorder and type of treatment. A systematic review of the literature was conducted. RESULTS: All patients were women, with hoarseness as the most frequent symptom. There was in most cases an associated autoimmune disease: 3 patients with systemic lupus erythematosus; 3 with rheumatoid arthritis; 1 with Sjögren syndrome; 1 with Hashimoto disease; and 1 with mixed connective tissue disease. Four patients were treated with speech therapy, 3 with oral steroids, 1 with speech therapy and oral steroids combined, 1 with oral steroids and laryngeal steroid injections, and 1 had oral steroids, surgery, and speech therapy. Speech therapy was the first-line treatment. CONCLUSION: Bamboo nodes should be looked for in every patient with a diagnosis of autoimmune disease complaining of dysphonia.

Ear, nose and throat involvement in granulomatosis with polyangiitis: how it presents and how it determines disease severity and long-term outcomes.

Ear, nose and throat (ENT) manifestations in granulomatosis with polyangiitis (GPA) represent the most frequent symptoms at disease onset. The aim of the study was to analyse ENT involvement at diagnosis, as well as how it could influence relapse rate, mortality and disease severity. A retrospective non-controlled cohort study was performed including all consecutive diagnosed GPA from 1996 to 2016 in two rheumatology centres of Northern Italy, focusing particularly on ENT presenting signs and symptoms at baseline. Eighty-nine patients (48.3% females) with new onset GPA were evaluated. They were mostly Caucasian (97.7%), middle aged (mean 54.5 years) and more frequently anti-neutrophil cytoplasmic antibodies (ANCA) positive (78.6%) with PR3 specificity (81.4%). At diagnosis, ENT involvement was reported in 71.9% patients, second only to systemic symptoms. These patients were significantly younger at disease onset (0.013), with less frequent renal involvement (0.014) irrespectively to ANCA status, but with significantly higher Vasculitis Damage Index (VDI) (0.001). The most frequent ENT manifestation was sinonasal involvement (58.4%, 73% of which with nasal inflammation/chronic sinusitis and 48% with nasal crusting), while otologic involvement (mainly otitis media/otomastoiditis) was observed in 34.8%. ENT-GPA patients presented a higher survival rate at 5 years (98.1 vs 77.7%, 0.049), and ENT involvement resulted to be an independent predictor of better outcome (OR 0.37, 95% CI 0.2-0.8, 0.019). Our data confirms that ENT involvement is not only one of the key clinical features of GPA, but also could point out a milder GPA subset with lower renal involvement and lower mortality rate, irrespectively to ANCA status.

Laryngeal abscess formation in an immunosuppressed patient: A case report.

Prior to the onset of the antibiotic era, laryngeal perichondritis and abscess formation were more frequent complications of systemic infections. We report a case of 54-year-old male who was medically immunosuppressed after kidney transplantation and developed multiple pseudomonas abscesses of his larynx. After failing initial treatment and with worsening signs and symptoms, the patient eventually was treated with a prolonged course of intravenous and oral antibiotics, with resolution of his symptoms and clinical findings. Although this pathophysiology remains uncommon, laryngeal abscess formation should remain in the differential for persistent symptoms, especially in cases of patients on immunosuppression. Laryngoscope, 127:2827-2829, 2017.

Generalized Leishmanides in an Immunodeprived Man.

Leishmanides have been rarely reported in the literature. In this study, the authors present a case of a 50-year-old HIV-positive man who developed a generalized cutaneous eruption of papules and plaques in which no microorganism was demonstrated by culture, microscopical examination, immunohistochemistry, or polymerase chain reaction. The patient was eventually diagnosed with laryngeal leishmaniasis, and when treated, the cutaneous lesions greatly improved.

Chronic Cough as a Female Gender Issue.

Cough accompanying acute respiratory tract disorders is a self-limiting phenomenon, and it usually does not require sophisticated management. Chronic cough, in contrast, is a bothersome problem, considerably influencing the quality of life of affected individuals. Specialized cough clinics report that substantial proportion of their patients are middle aged-to-postmenopausal females who cough for years in response to otherwise non-tussigenic stimuli, without a clear underlying disease reason. A newly established entity - 'cough hypersensitivity syndrome' explains pathogenesis of this problem. However, the syndrome has not been generally accepted, and the guidelines regarding the diagnostic protocols and treatment are not yet available. The reason why females cough more than males do is unclear, but the analysis of literature and experience with the chronic cough patients allows selecting three main targets of hormonal background which can contribute to the enhanced coughing in females. They are as follows: increased activity of transient receptor potential (TRP) channels expressed on vagal C-fibers mediating cough, laryngeal hypersensitivity and laryngeal dysfunction with paradoxical vocal cord movement, and mast cells which are known to express receptors for female sexual hormones and are frequently found in the bronchoalveolar lavage in chronic cough patients. In this review we analyze the potential contribution of the factors above outlined to excessive cough in female subjects.

Significant laryngeal destruction in a northern European cohort of Behçet's disease patients.

OBJECTIVES: Behçet's disease (BD) is a multisystem autoimmune disease of unknown origin typically affecting the triad of oral and genital mucosa and the eye. Limited data are available in the literature regarding the otolaryngology-related manifestations of BD, particularly in northern Europeans. This is a novel study detailing surprising and significant laryngeal structural changes in a northern European cohort of BD. METHODS: Patients meeting the International Study Group for Behçet's Disease (ISGBD) and the International Criteria for Behçet's Disease (ICBD) criteria for diagnosis were identified from an institutional database. Patients underwent examination with an otolaryngologist, including flexible laryngoscopy. Intra-oral, pharyngeal and laryngeal manifestations of BD were documented and characterised. Patients underwent hearing assessment with pure-tone audiometry. RESULTS: Fifteen patients with BD were identified (4 male, 11 female; median age 36 years). 60% (n=9) showed evidence of disease on examination and flexible laryngoscopy. 33% (n=5) showed laryngeal changes related to BD. 13% (n=2) demonstrated bilateral sensorineural hearing loss. The 5 cases demonstrating laryngeal manifestations of disease are described in detail with photographic records. CONCLUSIONS: Limited data has been published regarding the laryngeal manifestations of BD, particularly in a northern European population. Our cohort of BD patients demonstrate significant laryngeal structural changes. It would appear that these clinically relevant changes may be more common than was previously thought. Raised awareness of the risk of laryngeal pathology in BD patients, often in the absence of overt clinical symptomatology, may result in earlier diagnosis and treatment. Rheumatologists and otolaryngologists should consider closer multi-disciplinary co-operation in the management and follow up of patients with BD.

Work-associated irritable larynx syndrome.

PURPOSE OF REVIEW: The purpose of this study is to review the relevant literature concerning work-associated irritable larynx syndrome (WILS), a hyperkinetic laryngeal disorder associated with occupational irritant exposure. Clinical symptoms are variable and include dysphonia, cough, dyspnoea and globus pharyngeus. WILS is a clinical diagnosis and can be difficult to differentiate from asthma. Treatment options for WILS include medical and behavioural therapy. RECENT FINDINGS: Laryngeal-centred upper airway symptoms secondary to airborne irritants have been documented in the literature under a variety of diagnostic labels, including WILS, vocal cord dysfunction (VCD), laryngeal hypersensitivity and laryngeal neuropathy and many others. The underlying pathophysiology is as yet poorly understood; however, the clinical scenario suggests a multifactorial nature to the disorder. More recent literature indicates that central neuronal plasticity, inflammatory processes and psychological factors are all likely contributors. SUMMARY: Possible mechanisms for WILS include central neuronal network plasticity after noxious exposure and/or viral infection, inflammation (i.e. reflux disease) and intrinsic patient factors such a psychological state. Treatment is individualized and frequently includes one or more of the following: environmental changes in the workplace, GERD therapy, behavioural/speech therapy, psychotherapy counselling and neural modifiers.

Laryngeal actinomycosis in an immunocompromised patient.

Actinomycosis of the larynx represents an unusual presentation for a common bacterium comprising the oral and oropharyngeal florae. There are few cases reported in the literature of laryngeal actinomycosis occurring primarily in the immunocompromised population. Here, we present a case in a 74-year-old man that occurred in the setting of neutropenia as a result of chemotherapy. Once the diagnosis was made with biopsy of the larynx, the infection was resolved after a prolonged course of penicillin-based therapy.

Analysis of serum cytokine levels in larynx squamous cell carcinoma and dysplasia patients.

BACKGROUND: Although the imbalance of cytokines in Head and Neck Squamous Cell Carcinoma (HNSCC) is well known, there is scarce data regarding its occurrence during dysplasia, before the malignant transformation. OBJECTIVE: To determine whether laryngeal dysplasia patients show a different cytokine profile than patients with cancer and healthy controls. METHODS: Seventeen newly diagnosed, untreated larynx squamous cell carcinoma (SCC) and six laryngeal dysplasia patients as well as 22 healthy controls were analyzed for circulating cytokines. A flowcytometry Th1/Th2 cytokine array kit was used to quantitatively measure Interleukin-2 (IL-2), IL-4, IL-6, IL-10, Tumor Necrosis Factor-α (TNF-α) and Interferon-γ (IFN-γ) levels. Additionally, IL-8 levels were determined through ELISA. RESULTS: IL-6, IL-8 and IL-10 were determined to be statistically increased in SCC patients (p<0.05). IL-8 and IL-10 levels were also higher in SCC patients than dysplasia patients (p<0.05). Additionally, IL-6 and IL-10 were all found to be markedly increased in dysplasia patients compared with controls (p<0.05). CONCLUSION: Our results demonstrate an imbalance of IL-6 and IL-10 not only in HNSCC but also in laryngeal dysplasia.

Evidence of the local immune status in the human larynx.

We have studied the presence of local immunity in the larynx and its role and development of laryngeal glands in the human larynx. The local immune status in laryngeal secretion or related tissue specimens from the laryngeal ventricle was examined and the results were analyzed between individuals with or without head and neck cancer. Laryngeal secretions or mucosal tissue specimens were obtained during the microscopic laryngeal surgery or at the time of the surgery of the larynx. The laryngeal secretion contained immunological factors such as IgG, IgM, IgA or secretory IgA (SIgA). The mean level of SIgA of the mucosal tissue was low in patients with the benign laryngeal disease and considerably decreased in patients with previous radiation therapy. The level of SIgA in the laryngeal secretion closely correlated to the level of SIgA in the mucosal tissue. From the present study, we confirmed the actual presence of local immune function in the human larynx. Furthermore, the local immune status is affected by either the presence of malignancy or the treatment to the larynx such as radiation.

Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema.

INTRODUCTION: Hereditary angioedema (HAE) is a rare disorder characterized by C1 esterase inhibitor (C1-INH) deficiency, resulting in periodic attacks of acute edema that can be life-threatening if they occur in the laryngeal region. We assessed the efficacy of C1-INH concentrate in the emergency treatment of rarely occurring acute laryngeal HAE attacks in a prospective, open-label clinical study. METHODS: Acute laryngeal attacks were each treated with C1-INH concentrate (Berinert) at a single dose of 20 U/kg body weight. Efficacy endpoints included time to onset of symptom relief and time to complete resolution of all symptoms, each based on the patient's assessment. RESULTS: All 39 laryngeal attacks in 16 patients were treated successfully. The median time to onset of symptom relief was 15 min. The median time to complete resolution of all symptoms was 8.25 h. No treatment-related serious adverse events occurred, and the treatment was well tolerated. The administration of C1-INH concentrate was not associated with any viral infections. CONCLUSION: C1-INH concentrate is an effective and safe emergency treatment for providing reliable and rapid relief from the potentially life-threatening symptoms of laryngeal HAE attacks.

Immunological responses against human papilloma virus and human papilloma virus induced laryngeal cancer.

OBJECTIVE: This study aimed to clarify the local immune status in the larynx in the presence of infection or carcinogenesis associated with human papilloma virus. METHODS: Cytological samples (for human papilloma virus detection) and laryngeal secretions (for immunoglobulin assessment) were obtained from 31 patients with laryngeal disease, during microscopic laryngeal surgery. On histological examination, 12 patients had squamous cell carcinoma, four had laryngeal papilloma and 15 had other benign laryngeal disease. Cytological samples were tested for human papilloma virus DNA using the Hybrid Capture 2 assay. RESULTS: High risk human papilloma virus DNA was detected in 25 per cent of patients (three of 12) with laryngeal cancer. Low risk human papilloma virus DNA was detected only in three laryngeal papilloma patients. The mean laryngeal secretion concentrations of immunoglobulins M, G and A and secretory immunoglobulin A in human papilloma virus DNA positive patients were more than twice those in human papilloma virus DNA negative patients. A statistically significant difference was observed between the secretory immunoglobulin A concentrations in the two groups. Patients with laryngeal cancer had higher laryngeal secretion concentrations of each immunoglobulin type, compared with patients with benign laryngeal disease. The study assessed the mean laryngeal secretion concentrations of each immunoglobulin type in the 12 laryngeal cancer patients, comparing human papilloma virus DNA positive patients (n = 3) and human papilloma virus DNA negative patients (n = 9); the mean concentrations of immunoglobulins M, G and A and secretory immunoglobulin A tended to be greater in human papilloma virus DNA positive cancer patients, compared with human papilloma virus DNA negative cancer patients. CONCLUSION: These results suggest that the local laryngeal immune response is activated by infection or carcinogenesis due to human papilloma virus. The findings strongly suggest that secretory IgA has inhibitory activity against infection or carcinogenesis associated with human papilloma virus in the larynx.

Laryngopharyngeal reflux: diagnosis and treatment of a controversial disease.

PURPOSE OF REVIEW: Laryngopharyngeal reflux is a well-recognized and widely used term in ear, nose and throat practice. However, the symptoms and signs attributed to laryngopharyngeal reflux are non-specific and treatment is usually empirical. This review discusses current knowledge on diagnosis and treatment of laryngopharyngeal reflux. RECENT FINDINGS: Information is evolving regarding the implications of laryngopharyngeal reflux in the development of pathological conditions affecting the upper aerodigestive tract epithelium such as chronic laryngitis, otitis media with effusion and chronic sinusitis. However, there is still much to learn about the pathophysiologic mechanisms of laryngopharyngeal reflux and their role in its related disease conditions and there is still considerable controversy on diagnostic as well as therapeutic parameters for this condition. There is no consensus on the diagnosis and treatment of laryngopharyngeal reflux and the majority of clinicians depend mainly on clinical findings and empirical therapeutic tests rather than more specific investigations. SUMMARY: The concept of laryngopharyngeal reflux is still controversial. The current practice of empirical treatment with proton-pump inhibitors is based on weak evidence. However, this practice seems to be widely accepted and will not change until further clinical and laboratory studies improve our understanding of this common and well-recognized condition.