Ear, Nose And Throat Involvement Research Articles

Early Ear, Nose and Throat Manifestations in Eosinophilic Granulomatosis with Poliangioitis: Results from Our Cohort Group and Literature Review.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic necrotizing vasculitis affecting small-to-medium-sized vessels. EGPA's clinical manifestations are heterogeneous, affecting different organs and systems, and the upper respiratory tract can be affected by ear, nose and throat (ENT) involvement. The aim of our study was to assess type manifestations at the time of diagnosis in a cohort of EGPA patients and correlate findings with baseline variables (sex, age, antineutrophil cytoplasmic antibodies-ANCA-status) and literature reports. The main ENT manifestations in our patients at the time of diagnosis were: chronic rhinosinusitis with nasal polyposis (CRSwNP) (52%), turbinate hypertrophy (48%), nasal swelling (40%), rhinorrhea (40%), chronic rhinosinusitis without nasal polyposis (CRSsNP) (32%), nasal bone deformities (32%), nasal crusts (20%), nasal mucosal ulcers (12%), corditis (12%), hoarseness/dysphonia (12%), hearing loss (12%), mucoceles (4%) and eosinophilic rhinitis (4%). No correlations were found between sex, age, ANCA status and ENT clinical manifestations. A polymorphic ENT involvement is often observed in the early stages of EGPA. The presence of nasal, sinus, ear and/or laryngeal manifestations in patients with asthma and hypereosinophilia, independently of sex, age or ANCA status, should raise an alert for further investigation and differential diagnosis for EGPA. ENT specialists should be aware of their leading position in this diagnostic race.

Sinonasal and ear manifestations of Erdheim-Chester disease.

To calculate the prevalence of sinonasal and ear involvement in an Erdheim-Chester disease (ECD) population, to describe the different ear, nose and throat (ENT) manifestations and to study the association between ENT involvement, other organ involvement, and BRAF mutations. We led a retrospective monocentric study in the national referral center for ECD. One hundred and sixty-two patients with ECD and ENT data were included between January 1, 1980 and December 31, 2020. Ear and nose clinical and radiological findings were noted. We described and studied the prevalence of ENT involvement in ECD population. The association between sinonasal and ear involvement, other organ involvement, and BRAF mutations was calculated. The prevalence of ENT manifestations is around 45%. No clinical rhinologic or otologic signs were specific to ECD. Sinus imaging was abnormal in 70% of cases. A bilateral maxillary sinus frame osteosclerosis was highly specific of ECD. Associations were found between the sinus MRI imaging type and BRAF status, central nervous system involvement, cerebellum involvement and xanthelasma. Sinonasal or ear involvement is frequent in ECD and has specific imaging features for sinuses. Trial registration: #2011-A00447-34.

Systemic and Local Medical or Surgical Therapies for Ear, Nose and/or Throat Manifestations in ANCA-Associated Vasculitis: A Systematic Literature Review.

Ear, nose and throat (ENT) manifestations are common in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), yet how to treat these manifestations remains controversial. Therefore, we systematically reviewed the literature on the efficacy of therapies on ENT manifestations in AAV. A systematic review was conducted in accordance with the PRISMA guidelines, searching Medline, Embase and Cochrane libraries, including clinical studies between January 2005 and January 2022, in adults with AAV and ENT involvement, reporting on the effects of local and systemic therapy. The critical appraisal was performed using tools provided by the Cochrane Library and the level of evidence (LoE) was scored according to the Oxford Centre for Evidence-based Medicine. After screening 5609 identified studies, 136 full-text articles were assessed. Finally, 31 articles were included for critical appraisal and data-extraction. Nearly all studies (n = 29) were retrospective and scored low on LoE. The included studies evaluated local interventions (n = 11), glucocorticoids combined with conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) (n = 8), rituximab (n = 6), or mepolizumab (n = 6). Due to heterogeneity across studies meta-analysis was not performed. Four studies on mepolizumab for sinonasal symptoms (n = 92) showed response in 33-100% and relapse in 35%. Local therapy for subglottic stenosis was effective in 80-100% of patients in 11 studies (n = 157), but relapses were common (up to 83%). In five studies, hearing improvement was observed in 56-100%, with better outcomes when glucocorticoids were combined with csDMARDs compared to glucocorticoids only. Response rates of ENT manifestations varied widely in studies and relapses were observed frequently. Heterogeneity among studies impaired comparison.

The effect of nasal Staphylococcus aureus colonization and antibiotic treatment on disease activity in ANCA-associated vasculitis: a retrospective cohort study in the Netherlands

The aim of this study was to identify the role of nasal Staphylococcus aureus (S. aureus) colonization and the effect of systemic or local antibiotic treatment on disease activity in patients with antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis and ear nose and throat (ENT) involvement. Clinical, laboratory and histological data from all patients with ANCA-associated vasculitis and ENT involvement, who were diagnosed in two medical centres in The Netherlands between 1981 and 2020, were retrospectively collected. Nasal S. aureus colonization was defined as at least one positive nasal swab during follow-up. Data on systemic (cotrimoxazole and azithromycin) and local (mupirocin) antibiotic use were collected. Disease activity was divided into systemic and local disease activity. Univariate analyses and regression analyses (negative binomial Poisson and binary regression) were used. Two-hundred and thirteen patients were available for analysis. Median follow-up time was 8 (IQR 3–17) years. S. aureus colonization was tested in 100 (46.9%) cases of whom 44 patients (44%) tested positive. In these 100 patients, systemic and local disease activity at baseline and at last visit were comparable between patients with and without S. aureus colonization. Twenty-eight of the 44 S. aureus positive patients received antibiotics aimed at eradication of S. aureus. No statistically significant difference was found between the treated versus non-treated group with regard to systemic and local disease activity. Nasal S. aureus colonization does not influence systemic or local disease activity. Antibiotic treatment aimed at eradication did not modify disease activity.

MO324A RARE CASE OF GRANULOMATOSIS WITH POLYANGIITIS ASSOCIATED WITH FACTOR II DEFICIENCY

Abstract Background and Aims Granulomatosis with polyangiitis (GPA) (formerly called Wegener's) is a systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins. The kidney damage is severe. We report an observation of a young patient whose exploration of rapidly progressive renal failure revealed GPA with an original association with factor II deficiency. Method This a case report of a patient who was admitted to the nephrology, dialysis and transplantation in the sahloul university hospital in Tunisia. She was transferred from the regional hospital of sidi bouzid. Results This a case of a 19-year-old patient. She had the only a history of allergic rhinitis. She presented recently epistaxis and headache. The explorations reported a kidney failure with level of creatinine at 5.6 mg/dl. On examination: The blood pressure was normal (120/70 mmHg), she had signs of acute sinusitis and edema in both lower limbs and she developed anuria. For the biology: creatinine = 11.3 mg/dl (clearance = 4.6 ml/min), urea = 50 mmol/l, Proteinuria = 2.5 g/24h and Hematuria = 100/mm3. The cANCA type PR3 was positive. The diagnosis of rapidly progressive glomerulonephritis (RPGN) was suspected. She had microcytic hypochromic anemia of 6.9 g/dL and a low Prothrombin time (PT) of 54%. The exploration concluded to an isolated Factor II deficiency (59%). The Ear, Nose and throat (ENT) examination revealed nasal inflammation, sinusitis and nasal crusting. The kidney ultrasound showed two kidneys of normal size. The computed tomography showed bilateral maxillary sinusitis and there were no intra-alveolar hemorrhage. The Kidney biopsy could not be performed initially due to the risk of bleeding. On hospitalization, the patient presented a generalized tonic clonic seizures. The exploration by a cerebral Magnetic resonance imaging (MRI) showed signs of cerebral vasculitis and the electroencephalography (EEG) showed no focal abnormalities. The association of ENT involvement, the cerebrovascular disease, the rapid evolution of the creatinine, the positivity of the cANCA, the diagnosis of granulomatosis with polyangiitis was established. The therapeutic management was to start symptomatic treatment: diuretics, antibiotic therapy, transfusions, hemodialysis initially and antiepileptic therapy. The immunosuppressive treatment was based on pulses of methylprednisolone followed by full dose corticosteroid therapy, cyclophosphamide intravenous and sessions of plasma exchange. The evolution was characterized by clinical and biological improvement with creatinine = 1.3 mg/dl, a PT at 100% and a factor II at 70 % stable after stopping the plasma exchange in favor of the immunological cause of this deficit. Conclusion The early diagnosis and treatment of RPGN is important even if the biopsy cannot be performed. We reported a rare association between granulomatosis with polyangiitis and factor II deficiency. The normalization of the factor II after the treatment is in favor of the immunological cause of this deficit.

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

Granulomatosis with polyangiitis (GPA) frequently affects ear, nose and throat (ENT) at disease onset. Our aim was to report on our experience with the ENT manifestations in GPA patients, therapy and outcome. A retrospective study of GPA patients was performed who followed up in Rheumatology clinics at King Khalid University hospital, Riyadh during the period 1990-2016. Demographics, different ENT manifestations, laboratory and diagnostic features, therapy and outcome of GPA patients were retrieved from their medical charts. ENT involvement was observed in 15 (65.2%) of the 23 GPA cases identified. Otologic symptoms were noted in 3 (13.0%), nose and sinus symptoms in 13 (56.5%) and throat symptoms in 3 (13.0%) GPA patients. Epistaxis (39.1%) was the most prevalent nose and sinus symptom followed by sinusitis (30.4%), otitis media and hearing loss were the frequent otologic symptoms, oral ulcers and hoarseness of voice constituted frequent throat symptoms in GPA patients. Of the 15 ENT-GPA patients, 9 were males and 6 were females (male: female; 1.5: 1). Their mean age at disease onset was 33.8 ± 18.3 (range 11-57) years and mean duration of disease was 10.1 ± 5.9 (range 1-20) years. ANCA was positive in 93.3% ENT-GPA patients, 73.3% had c-ANCA and 20.0% had p-ANCA. Infections were noted in 33.3% ENT-GPA patients that included pneumonia, septicemia, esophageal candidiasis, bacterial meningitis and herpes zoster. All patients received oral prednisolone, 60.0% received intravenous cyclophosphamide, 20.0% refractory cases received rituximab doses, and the disease outcome was good. Comparison of ENT- GPA with non- ENT GPA cohort showed that 26.7% ENT-GPA patients had renal involvement compared to 87.5% non ENT-GPA patients (p = 0.009). Our study showed that the frequency of ENT symptoms in our GPA patients was less compared to other studies, and the disease outcome was good. Renal involvement was significantly less frequent in ENT-GPA cohort compared to non ENT-GPA cohort.

Ear Nose and Throat (ENT) Manifestations in Granulomatosis with Polyangiitis in Patients from Saudi Arabia

Granulomatosis with polyangiitis (GPA) frequently affects ear, nose and throat (ENT) at disease onset. Our aim was to report on our experience with the ENT manifestations in GPA patients, therapy and outcome. A retrospective study of GPA patients was performed who followed up in Rheumatology clinics at King Khalid University hospital, Riyadh during the period 1990-2016. Demographics, different ENT manifestations, laboratory and diagnostic features, therapy and outcome of GPA patients were retrieved from their medical charts. ENT involvement was observed in 15 (65.2%) of the 23 GPA cases identified. Otologic symptoms were noted in 3 (13.0%), nose and sinus symptoms in 13 (56.5%) and throat symptoms in 3 (13.0%) GPA patients. Epistaxis (39.1%) was the most prevalent nose and sinus symptom followed by sinusitis (30.4%), otitis media and hearing loss were the frequent otologic symptoms, oral ulcers and hoarseness of voice constituted frequent throat symptoms in GPA patients. Of the 15 ENT-GPA patients, 9 were males and 6 were females (male: female; 1.5: 1). Their mean age at disease onset was 33.8 ± 18.3 (range 11-57) years and mean duration of disease was 10.1 ± 5.9 (range 1-20) years. ANCA was positive in 93.3% ENT-GPA patients, 73.3% had c-ANCA and 20.0% had p-ANCA. Infections were noted in 33.3% ENT-GPA patients that included pneumonia, septicemia, esophageal candidiasis, bacterial meningitis and herpes zoster. All patients received oral prednisolone, 60.0% received intravenous cyclophosphamide, 20.0% refractory cases received rituximab doses, and the disease outcome was good. Comparison of ENT- GPA with non- ENT GPA cohort showed that 26.7% ENT-GPA patients had renal involvement compared to 87.5% non ENT-GPA patients (p = 0.009). Our study showed that the frequency of ENT symptoms in our GPA patients was less compared to other studies, and the disease outcome was good. Renal involvement was significantly less frequent in ENT-GPA cohort compared to non ENT-GPA cohort.

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 54.5years) 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 5years (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.

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Mexican Population: Clinical Phenotype, Autoantibody Profile and Renal Characteristics

Objective: To describe the clinical, serological characteristics, and renal histology in Mexican patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Methods: Forty patients with diagnosis of AAV and renal biopsy, followed at a tertiary care center in Mexico City from 2002-2012 were retrospectively studied. Demographics, comorbidities, clinical, laboratory variables, disease activity, mortality, renal variables and histological classes were analyzed. Additionally, 50 patients with AAV, and a control group (other systemic vasculitis, systemic lupus erythematosus, infections, healthy subjects) were studied transversally to assess the prevalence of specific antibodies (ANCA, MPO, PR3, cathepsin G, lysozyme, lactoferrin, BPI and elastase), and their association with clinical manifestations. Results: Granulomatosis with polyangiitis (GPA) was the predominant phenotype, and median age at diagnosis was 53 years. High disease activity (BVAS/GPA=24), general symptoms (98%), renal (80%), pulmonary (63%), and ear, nose and throat (ENT) (58%) involvement were frequent at diagnosis. Renal damage was present at diagnosis (serum creatinine 3.9 mg/dl, estimated glomerular filtration rate 16 ml/min, 24 hr proteinuria 2.5 g, 15% on dialysis). The most prevalent renal histological class was sclerotic (58%), followed by mixed (32%), focal (8%), and crescentic (2%). No significant differences were found between histological classes in comorbidities, clinical, renal and laboratory variables, or survival. The main ANCA immunofluorescence pattern was C-ANCA (68%), followed by XANCA (22%), and P-ANCA (18%). PR3-ANCA (high sensitive) was the most frequent antibody, positive in 86%, while positivity for MPO-ANCA was observed in 18%. ANCA positivity was more frequent in patients with AAV than the control group (p<0.001). Antibodies directed to minor targets were infrequent in patients with AAV (2%). No significant correlations were identified between autoantibodies and clinical manifestations. Conclusion: GPA phenotype, C-ANCA and PR3-ANCA positivity, high disease activity, chronic renal damage, general symptoms, ENT and lung involvement at diagnosis distinguish our population with AAV.

Manifestation of eosinophilic granulomatosis with polyangiitis in head and neck.

Besides an obvious clinical involvement of the ear, nose and throat (ENT)-region in Eosinophilic Granulomatosis with Polyangiitis (EGPA), systematic data is sparse. Only a few case series and case reports are available that particularly describe rhinological, otological or other manifestations of EGPA in the ENT-region. Therefore, the objective of this study is to systematically describe data on ENT-region involvement in a large series of EGPA patients. EGPA patients examined in the Department of Otorhinolaryngology of the Christian-Albrechts-University of Kiel between 1990 and 2010 were included in the study. Criteria for ENT-manifestation were assigned to five subgroups (history, ENT examination, audiological and rhinological diagnostic findings and cranial MRI) and documented cumulatively. EGPA patients were examined in a standardized way based on the validated Ear Nose and Throat Activity Score (ENTAS) or its precursor, including audiological and rhinological diagnostic findings. MRI scans were analysed to further evaluate ENT involvement. A total of 95 EGPA patients were included in the study. In approximately 80% of them, ENT-involvement was documented and the assumption of a frequent rhinological manifestation in patients with EGPA was confirmed. Moreover, the data reveals remarkable evidence for an otological manifestation. A missing correlation between the rhinological and the otological manifestation indicates an independent autoimmune-inflammatory process for this manifestation. The data of the largest monocentric study presented here confirms the hypothesis of a frequent ENT involvement in EGPA patients, in whom rhinological and otological manifestations are most common. Therefore, treatment should include long term follow-up and should be managed interdisciplinary.

Manifestation of eosinophilic granulomatosis with polyangiitis in head and neck

Besides an obvious clinical involvement of the ear, nose and throat (ENT)-region in Eosinophilic Granulomatosis with Polyangiitis (EGPA), systematic data is sparse. Only a few case series and case reports are available that particularly describe rhinological, otological or other manifestations of EGPA in the ENT-region. Therefore, the objective of this study is to systematically describe data on ENT-region involvement in a large series of EGPA patients. EGPA patients examined in the Department of Otorhinolaryngology of the Christian-Albrechts-University of Kiel between 1990 and 2010 were included in the study. Criteria for ENT-manifestation were assigned to five subgroups (history, ENT examination, audiological and rhinological diagnostic findings and cranial MRI) and documented cumulatively. EGPA patients were examined in a standardized way based on the validated Ear Nose and Throat Activity Score (ENTAS) or its precursor, including audiological and rhinological diagnostic findings. MRI scans were analysed to further evaluate ENT involvement. A total of 95 EGPA patients were included in the study. In approximately 80% of them, ENT-involvement was documented and the assumption of a frequent rhinological manifestation in patients with EGPA was confirmed. Moreover, the data reveals remarkable evidence for an otological manifestation. A missing correlation between the rhinological and the otological manifestation indicates an independent autoimmune-inflammatory process for this manifestation. The data of the largest monocentric study presented here confirms the hypothesis of a frequent ENT involvement in EGPA patients, in whom rhinological and otological manifestations are most common. Therefore, treatment should include long term follow-up and should be managed interdisciplinary.

THU0226 Clinical features and mortality causes in a large cohort of spanish patients with anca associated vasculitides

BackgroundAntineutrophil cytoplasmic autoantibody (ANCA) associated vasculitides (AAV) are uncommon chronic and relapsing diseases characterized by inflammatory cell infiltration and necrosis of blood vessel walls. They often have overlapping clinical and...

Manifestations ORL et maladies systémiques

Ear nose and throat (ENT) manifestations are amongst the most common clinical features of systemic and auto-immune disorders. In some of them including relapsing polychondritis, granulomatosis with polyangeitis (Wegener's) or Churg-Strauss syndrome, ENT involvement is part of the disease. In many others, ENT manifestations are less common but may be the presenting features and represent a diagnostic challenge. Collaboration between ENT specialists, rheumatologists and general internists is essential to avoid diagnostic delay and improve patients’ care.

The multidisciplinary management of non‐melanoma conchal bowl skin cancer

Mohs micrographic surgery (MMS) has been shown to achieve high cure rates and reduce recurrence rates of skin cancer in auricular and preauricular regions. Non-melanoma skin cancers of the conchal bowl are difficult to treat due to the challenge of accessing them and their close association with surrounding structures that may be inadvertently involved. Treatment goals in this anatomical area include total tumour removal and maximal tissue conservation to provide the best functional and aesthetic result for the patient. We present two patients with conchal bowl basal cell carcinoma treated with MMS and found to have extensive disease that benefited from collaborative management with an ear, nose and throat (ENT) surgeon. Where extensive subclinical spread is encountered or complex reconstruction is required it is useful to adopt a multidisciplinary approach to achieve total tumour control and maximal tissue conservation. Currently preoperative assessment of tumour margins is difficult and limited data are available on what cases should be co-managed. Our recommendation is that collaborative ENT involvement be reserved for cases where a long history is involved, a history of recurrence, previous failed excisions, close proximity to difficult to access areas, histological subtypes of skin carcinoma that are known to be aggressive or recurrent or when there is suspicion of subclinical spread based on other grounds.

Ear, nose and throat involvement in patients with pemphigus vulgaris: correlation with severity, phenotype and disease activity

Pemphigus vulgaris (PV) is the most common clinical form of pemphigus that is characterized by easily ruptured or loose bulla formation on skin and/or mucosa. The frequency of the ear, nose and throat involvement of PV is not clearly defined. To evaluate the frequency of ear, nose and throat (ENT) involvement in patients suffering from PV who had been recently diagnosed or exacerbated under follow up and to determine the association with ENT symptoms, clinical involvement, severity and duration of pemphigus vulgaris. The study group included a total of 38 PV patients comprising 24 new patients and 14 patients who showed exacerbations while on complete remission or under treatment. All patients were asked about ENT symptoms and endoscopic examination were performed to evaluate the presence of nasal, pharynx, larynx and ear involvement. Of the 38 patients, 33 (87%) had active PV lesions on endoscopic evaluation. Twenty-five patients (66%) had lesions on pharynx, twenty-one (55%) on larynx, twenty-nine (76%) on nasal mucosa and four (10%) on the ear mucosa. ENT involvement was not associated with the severity and the clinical involvement of the disease. Pharyngeal and nasal involvement was significantly associated with symptoms, while laryngeal and ear involvement was not found to be significantly associated with symptoms. Nasal and ear involvement was not associated with the duration of the disease while pharyngeal and laryngeal involvement favored newly diagnosed patients. Our results revealed that high number of patients with PV may present with active ENT lesions, furthermore patients with ear, nose and throat involvement may be asymptomatic and active lesions may be found in patients without any ENT symptoms. Therefore it should be considered that to understand the real extent of PV involvement, endoscopic ENT evaluation should be performed in patients with or without symptoms.

Ear, nose and throat manifestations in pemphigus vulgaris

Pemphigus vulgaris (PV) is an autoimmune disease characterized by mucocutaneous intraepithelial blisters and pathogenic autoantibodies against desmoglein 3. There are two clinical forms: mucosal (MPV) and mucocutaneous (MCPV). The frequency of ear, nose and throat (ENT) involvement in PV is not clearly defined. Only a few isolated individual cases have been reported. The objective of our study was to determine the incidence of ENT involvement in patients with PV. We studied prospectively all 16 patients diagnosed with PV and treated in the Department of Dermatology of the University Clinic of Navarra between 2001 and 2005. They were 10 cases of MPV and six cases of MCPV. All patients were evaluated for ENT manifestations by endoscopic examination. Of the 16 patients, 13 presented with throat symptoms (81%), 12 pharyngeal (75%) and seven laryngeal symptoms (44%). Fourteen patients (88%) had active PV lesions on endoscopic evaluation (eight patients had active lesions on both pharyngeal and laryngeal mucosa, four had PV lesions only on laryngeal mucosa and two had PV lesions on pharyngeal mucosa). Laryngeal lesions were most commonly present in MPV patients. The frequency of nasal symptoms (38%) was lower than active PV lesions (62%) found on ENT examination. Oral symptoms and oral active PV lesions were the most frequent findings (94%). Only three patients with MCPV showed erosions on the external auditory canal. As ENT endoscopy allows more extensive areas of mucosa to be examined than simple visual inspection, we recommend that it be included in the examination of all patients with PV. By obtaining more complete information concerning the extent of the disease, a more accurate diagnosis can be made, better choice of drug and dose may be decided and, ultimately, response to treatment may be improved.

Ear, nose and throat manifestations of Churg-Strauss syndrome

Conclusion. Ear, nose and throat (ENT) involvement is common in Churg-Strauss syndrome (CSS), usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps. Otolaryngologists may play a pivotal role in making an early diagnosis of this disease. Objectives. CSS is a systemic vasculitic disorder that affects small to medium-sized blood vessels. Although the cause of CSS remains unknown, tissue damage seems more likely to be mediated by activated eosinophils. Patients affected by CSS frequently have ENT manifestations, which are often present at the time of disease onset and may represent relevant clues for the diagnosis. Thus, our objective was to present the ENT manifestations at the onset, at the diagnosis and at some point during the course of the disease in a series of patients with CSS collected at a single center. Materials and methods. Twenty-eight patients with CSS, as defined according to the 1990 American College of Rheumatology classification criteria, were identified. Twenty-one (75%) of these patients had ENT involvement. We evaluated the clinical course, laboratory data, histologic findings, treatment and outcomes. Results. Of the 21 patients, 13 (61.9%) had ENT involvement at asthma onset and 8 (38%) at diagnosis or during follow-up. The most common ENT manifestations were allergic rhinitis in 9 (42.8%) patients and nasal polyposis in 16 (76.1%). Three (14.2%) patients developed chronic rhinosinusitis without polyps, three (14.2%) had nasal crusting, one (4.7%) serous otitis media, one (4.7%) purulent otitis media, two (9.5%) progressive sensorineural hearing loss, and one (4.7%) unilateral facial palsy. Corticosteroid therapy associated with immunosuppressive drugs usually yielded improvement or stabilization.