Caterina Marinelli

Vogt–Koyanagi–Harada syndrome

OBJECTIVES The objectives of this study are to review our current knowledge of the aetiopathogenesis of Vogt-Koyanagi-Harada syndrome, including viral infection, genetic factors and immunomediated mechanisms, and to discuss pathogenesis and its relevance to pharmacotherapy. SYSTEMATIC REVIEW METHODOLOGY Relevant publications from 1965 to 2012 on the aetiopathogenesis and pharmacotherapy of VKHS were analysed. RESULTS AND CONCLUSION Vogt-Koyanagi-Harada syndrome (VKHS) is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. The disease is characterised by bilateral uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. The disease occurs more frequently among people with darker skin pigmentation. Asians, Native Americans, and Hispanics are most frequently affected. It predominates in patients aged between 20 and 50years, and females are affected more frequently, with a female:male ratio of 2:1. The classic clinical course is characterised by bilateral panuveitis, hypoacusis, and meningitis, in addition to cutaneous involvement with poliosis, vitiligo, and alopecia. Although the exact cause of VKH disease remains unknown, it is thought to be a T-cell-mediated autoimmune process directed against melanocytes. VKHS classically begins with vague systemic symptoms suggestive of a viral infection, although a clear association between a specific viral agent and the disease has not been established. Genetic factors may play an important role in the loss of self-tolerance in VKHS. The HLA-DRB1*0405 allele is the main susceptibility allele for VKHS. Early and aggressive systemic corticosteroids are still the primary initial therapy for VKHS. Ocular complications may require an intravitreous injection of corticosteroids. Despite proper treatment with steroids, a number of patients experience recurrent attacks or steroid-associated complications. Thus, non steroid immunomodulatory therapy (IMT) has become necessary for the treatment of VKHS.

Carotid stenosis after adjuvant cervical radiotherapy in patients with head and neck cancers: a prospective controlled study

Clin. Otolaryngol. 2012, 37, 376–381

Clinic manifestations in granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility. In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8–99 years). Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70–100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85–100%), whereas otological disease is found in approximately 35% (range, 19–61%) of cases. Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60–70% when there is no kidney involvement. The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase. A multidisciplinary approach, involving otorhinolaryngologists, oral and maxillofacial surgeons, oral physicians, rheumatologists, renal and respiratory physicians, and ophthalmologists, is necessary for the diagnosis and therapeutic treatment of GPA. ENT physicians have a determining role in recognising the early onset of the disease and starting an appropriate therapy.

Is vestibular neuritis an immune related vestibular neuropathy inducing vertigo

Objectives. To review the current knowledge of the aetiology of vestibular neuritis including viral infections, vascular occlusion, and immunomediated mechanisms and to discuss the pathogenesis with relevance to pharmacotherapy. Systematic Review Methodology. Relevant publications on the aetiology and treatment of vestibular neuritis from 1909 to 2013 were analysed. Results and Conclusions. Vestibular neuritis is the second most common cause of peripheral vestibular vertigo and is due to a sudden unilateral loss of vestibular function. Vestibular neuronitis is a disorder thought to represent the vestibular-nerve equivalent of sudden sensorineural hearing loss. Histopathological studies of patients who died from unrelated clinical problems have demonstrated degeneration of the superior vestibular nerve. The characteristic signs and symptoms include sudden and prolonged vertigo, the absence of auditory symptoms, and the absence of other neurological symptoms. The aetiology and pathogenesis of the condition remain unknown. Proposed theories of causation include viral infections, vascular occlusion, and immunomediated mechanisms. The management of vestibular neuritis involves symptomatic treatment with antivertiginous drugs, causal treatment with corticosteroids, and physical therapy. Antiviral agents did not improve the outcomes.

Cricoarytenoid joint involvement in rheumatoid arthritis: radiologic evaluation

Rheumatoid arthritis rarely involves the cricoarytenoid joint. The possible consequent symptom includes hoarseness, dysphagia, odynophagia, dysfunctional dysphonia, and acute dyspnea. Etiologic diagnosis is possible with high-resolution computed tomography, which can show spacing of the articular cartilage, density and volume alterations, and subluxation of the cartilage. However, these radiologic signs are not pathognomonic for rheumatoid arthritis, and they should be combined with anamnestic data.

Autologous serum skin test reactivity and basophil histamine release test in patients with nasal polyposis: preliminary results.

An eosinophilic inflammatory process is generally observed in patients suffering from nasal polyposis (NP), however its onset has not yet been defined. It has been suggested that immune activation of inflammatory cells may be the cause. The aim of this study is to verify whether autoantibodies and/or histamine-releasing factors are present in the serum of patients suffering from NP. In fact, we assume that autoantibodies and/or histamine-releasing factors, as already demonstrated in chronic idiopathic urticaria and asthma, may be involved in the pathogenesis of NP. In this case-control analytical study 40 patients with NP and 27 control subjects underwent the in vivo autologous serum skin test (ASST). The sera from 6 patients suffering from NP and 9 control group subjects, who had all been previously studied and randomly selected, underwent basophil histamine release assay from normal donor as a pilot study. The ASST showed positive results in 55% of patients suffering from NP versus 8% of the control group (p= .00006), the basophil histamine release test (BHRT) turned out positive in all patients tested and in 11% of the control group. We found a weak positive correlation between the percentage of histamine release and the wheal diameter. ASST reactivity is very frequent in patients suffering from NP, thus suggesting the presence of histamine-releasing factors in the blood stream. The BHRT was positive in the serum of all patients, thus suggesting the presence of anti-FcεRI, anti-IgE autoantibodies and/or other histamine-releasing factors, the presence of which can play a role in triggering and maintaining the eosinophilic inflammatory process in NP.

Rare and massive odontogenic parakeratotic cyst treated by endoscopic sinus surgery: a case report

IntroductionKeratocystic odontogenic tumors are benign neoplasms of odontogenic origin with a potential for aggressive and infiltrative behavior. Many different treatments for this type of lesion have been reported. However, no common consensus has emerged to date regarding the most effective therapeutic approach. Cases of maxillary sinus giant keratocystic odontogenic tumors completely excised by enucleation or marsupialization via endoscopic sinus surgery are extremely rare, and, to the best of our knowledge, only one case has been described in the literature since 2005.Case presentationWe report a case of a 24-year-old Italian man who came to our department with maxillary sinus region swelling, pain and left nasal obstruction. A massive keratocystic odontogenic tumor involving the right maxillary sinus and causing focal erosions of the bony walls was diagnosed. The keratocystic odontogenic tumor was removed as much as possible by a transnasal approach using endoscopic sinus surgery, which produced optimal surgical and prognostic outcomes. Follow-up is reported for an 8-year period.ConclusionConservative management in this case demonstrated good therapeutic efficacy with a low risk of recurrence. For injuries involving the maxillary sinus, the possibility of decompression or marsupialization by endoscopic sinus surgery should always be considered because it demonstrated the potential to lead to excellent results even after 8 years of follow-up in our patient. To our knowledge, no case report has described follow-up longer than 8 years for a maxillary sinus keratocystic odontogenic tumor treated with endoscopic sinus surgery.

How to classify the stylohyoid complex syndrome in the ICHD

We have read the International Classification of Headache Disorders, third edition (beta) (ICHD-3 beta) (1), and for the first time headaches are attributed to inflammation of the stylohyoid ligament (SL). It is included among the secondary headaches in ‘‘Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure.’’ This is positive news because stylohyoid complex syndrome (SHCS) is an uncommon disease and now plays a role in ICHD-3 beta, as suggested by Montalbetti et al. in 1995 in this same journal (2). Therefore, it is our opinion that SHCS is not fully understood in ICHD-3 beta. SHCS is an uncommon condition related to degeneration or anatomical malformation of the stylohyoid complex (SHC), composed of the styloid process (SP), the lesser cornu of the hyoid bone and the SL that connects them. Three pathological conditions can develop in the SHCS: elongated SP, ossification or inflammation of the SL and an elongated hyoid bone. In SHCS there are two clinical groups: the classic variety presents with chronic neck pain, odynophagia, otalgia, dysphagia and foreign body sensation; the second variety is uncommon, since patients have neck and face pain plus headaches that are not relieved by common analgesics. In the first group one of the three pathological conditions of SHCS can provoke inflammation of soft tissues and neck nerves, while in the second symptom group the impingement of the SP on carotid vessels produces inflammation of the sympathetic nerve plexus in the arterial sheath with functional deficit. The mechanism is similar to cluster headaches in which carotid vasodilation pushes against the sympathetic plexus, producing a sympathetic deficit with release of vasoconstrictor tone. In the English literature we have found seven cases of headaches caused by styloid process impingement on carotid vessels and in three cases three-dimensional (3D) computed tomography (CT) imaging (CT-3D) of the neck showed elongated SP and the diagnosis of SHCS was made (3). Koebke also reveals typical characteristics of early arteriosclerosis at the point of SP impingement on carotid vessels (4). We have been treating a 35-year-old man who has been suffering from headaches for 10 years and who reported pain in the left cervical region. The 3D-CT imaging revealed a left cervical carotid artery compression due to an elongated SP. This procedure facilitates visualization of the SL and the relationship between the SP and the carotid artery (Figure 1). In conclusion, we believe that ICHD-3 beta can be improved: Point 11.8 states ‘‘headache or facial pain attributed to inflammation of the stylohyoid ligament,’’ but this is just one of three conditions in the SHCS. The right title should be ‘‘headache or facial pain attributed to SHCS.’’ For this reason, we propose that diagnostic B criteria should include radiologic evidence of ‘‘inflammation of the SL and/or elongated SP and/or the horn of the elongated hyoid bone’’ and that the C2 criteria should include pain caused by or exacerbated by head turning, chewing and swallowing. It is our opinion that the second variety of SHCS in ICHD-3 beta should also be considered. We propose that the pressing of SP on the carotid artery can be classified as secondary headache at point

Evidence and role of autoantibodies in chronic rhinosinusitis with nasal polyps.

In this study, we review our current knowledge of the autoimmune etiopathogenesis of chronic rhinosinusitis with nasal polyps including bacterial infections, viral infections and immunomediated mechanisms and to discuss pathogenesis with relevance for pharmacotherapy. Relevant publications on the etiopathogenesis and treatment of chronic rhinosinusitis with nasal polyps (CRSwNP) from 1977 to 2013 were analyzed. The characteristic signs and symptoms include appearance of relapsing nasal polyps, with typical symptoms such as nasal obstruction, nasal discharge and, usually, loss of the sense of smell. The etiology and pathogenesis remain unknown. Proposed theories of causation include bacterial or viral infections and immunomediated mechanisms. The autoimmune aetiology of of unknown origin or failure to respond to classic pharmacological treatments with nasal and oral steroids is now suspected. At present, the nature of the antigen trigger, the exact role played by B/T cells and anti-dsDNA autoantibodies in the pathogenesis of nasal polyposis remains unclear. Corticosteroids and surgery are the first line of treatment in CRSwNP. In the case of corticosteroid treatment failure, other drugs can be used such as rituximab, belimumab or omalizumab which have demonstrated clinical efficacy in the treatment of nasal polyposis with comorbid asthma. Immunosuppressive drugs such as methotrexate, and cyclophosphamide have also been used with varying degrees of success.

Use of electrochemotherapy in a case of neck skin metastasis of oral squamous cell carcinoma: Case report and considerations

Squamous cell carcinoma (SCC) is the most common oral cavity malignant tumor. Surgery, radiotherapy, and chemotherapy have been the major options for its treatment. Electrochemotherapy (ECT) is a novel local treatment successfully used in secondary or primary skin or subcutaneous tumors. This new cancer treatment is a modality in which a locally applied electrical field enhances cell membrane permeability, thereby allowing greater intracellular accumulation of a chemotherapeutic agent.