Sinonasal Manifestations Research Articles

Cystic fibrosis-related chronic rhinosinusitis: the key role of a comprehensive evaluation in the era of highly effective modulator therapy.

Chronic rhinosinusitis (CRS) is prevalent in cystic fibrosis (CF), significantly affecting quality of life. The introduction of CFTR modulators, including elexacaftor-tezacaftor-ivacaftor (ETI), offers promise for improving sinonasal outcomes. We conducted a retrospective cohort multicenter study analyzing electronic medical records of 45 adult CF patients with CRS, predominantly heterozygous for the ΔF508 mutation, treated with ETI between January 2018 and December 2023. Assessments included Sinonasal Outcome Test 22 (SNOT-22), Nasal Polyp Score (NPS), modified Lund-Kennedy Score (mLKS), Lund-Mackay Score (LMS), and olfactory function using smell loss visual analog scale (VAS) and Sniffin' Sticks identification test (SSIT). After 12 months of ETI therapy, significant improvements were observed in pulmonary function parameters (FEV1, FVC), CRS severity scores (SNOT-22, NPS, mLKS), radiological findings (LMS), and olfactory function. Subgroup analysis suggested enhanced efficacy in patients with prior endoscopic sinonasal surgery. ETI therapy demonstrates comprehensive improvements in CRS and olfactory function in CF patients, highlighting the potential of CFTR modulators in managing sinonasal manifestations.

IgG4-related disease: an update on pathology and diagnostic criteria with a focus on salivary gland manifestations.

Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder characterized by a highly variable clinical presentation depending on the affected organ/s, extent of tumefactive fibroinflammatory lesions, and associated functional impairment. The disease pursues a chronic, relapsing, often asymptomatic course and hence may pose a significant diagnostic challenge. Diagnostic delay can lead to progressive fibrosis and irreversible organ damage resulting into significant morbidity and even mortality. Given its broad clinical spectrum, physicians of all specialties may be the first clinicians facing this diagnostic challenge. Outside the pancreatobiliary system, the head and neck represents the major site of IgG4-RD with variable organ-specific diffuse or mass-forming lesions. In up to 75% of cases, elevated serum IgG4 levels are observed, but this figure possibly underestimates the fraction of seronegative cases, as the disease manifestations may present metachronously with significant intervals. Together with negative serology, this can lead to misdiagnosis of seronegative cases. A standardized nomenclature and diagnostic criteria for IgG4-RD were established in 2012 and revised in 2020 facilitating scientific research and expanding the range of diseases associated with IgG4 abnormalities. In addition to orbital pseudotumor, dacryoadenitis, Riedel thyroiditis, sinonasal manifestations, and rare miscellaneous conditions, IgG4-related sialadenitis is one of the most frequent presentations in the head and neck region. However, controversy still exists regarding the relationship between sialadenitis and IgG4-RD. This review focuses on the clinicopathological features of IgG4-related sialadenitis and its contemporary diagnostic criteria.

Efficacy of triple targeted therapy against chronic rhinosinusitis in children with cystic fibrosis (case series)

Introduction. Chronic rhinosinusitis (CRS) with underlying cystic fibrosis (CF) is one of the most challenging inflammatory diseases of the paranasal sinuses (PNS). The treatment of CF in general and CF-associated CRS in particular keeps developing exponentially: special drug delivery systems have been invented, drugs purpose-developed for the treatment of patients with CF (dornase alfa) are used, and radicality of surgical intervention is increasing. In recent years, CF innovative targeted therapy has been introduced. It aims to improve the course of the bronchopulmonary infectious and inflammatory process, nutritional status and pancreatic function, but its efficacy in relation to the PNS condition is still insufficiently studied.Aim. To evaluate the efficacy of ivacaftor/elexacaftor/tezacaftor therapy for CF sinonasal manifestations in children and to determine possible parallels of the CRS course vs other characteristics of the CF course.Materials and methods. A total of 15 children with CF receiving triple targeted therapy were included in the first series of observations. Computed tomography (CT) scans were assessed using the Lund-Mackay scores (LMS) before and 7.6 ± 3.0 months after starting the therapy. In addition, the changes in sweat chloride levels and pulmonary function test results were taken into account. The control group included 11 patients with comparable initial radiographic data who did not receive such therapy.Results and discussion. The treatment group showed a significant improvement in the PNS condition in the form of a decrease in the LMS scores from 15.6 ± 4.5 to 2.1 ± 3.0 (p = 0.001) with inhomogeneous changes vs different PNS groups. A certain parallelism between changes in the CT view and the level of sweat chlorides and pulmonary function test results was reported. In the control group, a nonsignificant deterioration in the course of CRS was observed (from 12.3 ± 6.3 to 14.6 ± 4.9 scores according to LMS; p = 0.108).Conclusions. The study demonstrated high efficacy of CF triple targeted therapy with ivacaftor/elexacaftor/tezacaftor in relation to the severe CRS with a rapid onset of effect in the form of PNS pneumatization restoration according to CT findings.

Ear and upper airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review.

Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by pulmonary, otological and sino-nasal manifestations. Well-defined clinical outcome measures are needed in such rare diseases research to improve follow-up and treatments. Pulmonary outcome measures have recently been described. The aim of this study was to identify ear and upper airway outcome measures that could be used for longitudinal follow-up of individuals with PCD. A scoping review was performed by systematically searching MEDLINE, Embase and Cochrane Database of Systematic Reviews online databases for studies published from January 1996 to March 2022 that included at least 10 adult or paediatric PCD patients and reported ear and upper airway outcomes. 33 studies (1794 patients) were included. 10 ear and upper airway outcomes were reported. 17 studies reported audiometry, 16 reported otoscopic findings, and 13 reported rhinoscopic findings and sinus imaging. Health-related quality of life questionnaires were performed in seven studies. There was a high variability in definitions and measurement of outcomes between studies. This scoping review highlights the lack of data regarding ear and upper airway outcomes in PCD. It also reports a high heterogeneity in outcome definitions or measures. We provide well-founded specific suggestions to standardise ear and upper airway outcome definitions and reporting for future PCD research studies.

Ocular manifestations of ANCA-associated vasculitis.

ANCA-associated vasculitis (AAV) is a group of multisystem diseases that can have several ocular manifestations. There are published data on ocular manifestations of granulomatosis with polyangiitis (GPA), but few for eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyangiitis (MPA). There is little information concerning chronicity, complications, and association with other cranial manifestations of AAV. This study retrospectively analysed longitudinal multicentre cohorts of individuals with AAV followed between 2006 and 2022. Data included diagnosis, demographics, cranial manifestations of disease, presence of manifestations at onset of disease and/or follow-up, and ocular complications of disease. Univariate and multivariable logistic regression analysis assessed associations across disease manifestations. Data from 1441 patients were analysed, including 395 with EGPA, 876 with GPA, and 170 with MPA. Ocular manifestations were seen within 23.1% of patients: 39 (9.9%) with EGPA, 287 (32.7%) with GPA, and 12 (7.1%) with MPA at any time in the disease course. There were more ocular manifestations at onset (n = 224) than during follow-up (n = 120). The most common disease-related manifestations were conjunctivitis/episcleritis and scleritis. In multivariable analysis, dacryocystitis, lacrimal duct obstruction, and retro-orbital disease were associated with sinonasal manifestations of GPA; ocular manifestations were associated with hearing loss in MPA. The most common ocular complications and/or damage seen were cataracts (n = 168) and visual impairment (n = 195). Ocular manifestations occur in all forms of AAV, especially in GPA. Clinicians should be mindful of the wide spectrum of ocular disease in AAV, caused by active vasculitis, disease-associated damage, and toxicities of therapy.

Sinonasal Manifestations of Severe Cherubism: A Case with 11-year Follow-up.

Cherubism, a type of fibroosteodysplasia, is a rare hereditary disease that causes variable degrees of facial deformity in children. Hypertrophy of the mandible is the most common symptom, but in severe cases, the disease affects the eyes, teeth, and sinonasal cavity. There have been few reports regarding sinonasal complications and no standard treatment has been established. This paper reports long-term treatment of severe cherubism that invaded the sinonasal cavity treated with consecutive endoscopic sinonasal surgeries.

Sinonasal manifestations and dynamic profile of RT-PCR results for SARS-CoV-2 in COVID-19 patients.

Sinonasal symptoms were usually reported to appear initially, yielding the symptoms important for the early detection of coronavirus disease 2019 (COVID-19). This study was conducted retrospectively to investigate the detailed sinonasal manifestations and dynamic profile of real-time reverse transcription polymerase chain reaction (RT-PCR) results for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in COVID-19 patients longitudinally. This retrospective study included 11 consecutive patients. The prevalence, timing and severity of sinonasal manifestations were analyzed. Oropharyngeal, nasal, sputum and stool specimens were collected to detect RT-PCR for SARS-CoV-2 over COVID-19 period. Among the 11 patients, 6 (54.5%) were female, and the median age was 51 (IQR, 36-62) years. Seven patients (63.6%) experienced sinonasal symptoms, with 6 (54.5%) exhibiting sinonasal symptoms on the onset day. Seven patients (63.6%) demonstrated nasal obstruction, 5 (45.5%) had rhinorrhea, and 4 (36.4%) exhibited olfactory dysfunction. All six patients with sinonasal symptoms on the onset day had non-severe infections. Most patients (85.7%) with sinonasal symptoms had non-severe infections. Sinonasal symptoms commonly appeared early. The positive RT-PCR rate for SARS-CoV-2 in various specimens was highest in the first week (73.3%), then gradually decreased over the disease course, but 3 patients (27.3%) had experienced a long-lasting fluctuated positive RT-PCR results since 29 days of illness in both groups, especially for two patients with airway comorbidities. Sinonasal symptoms were more prevalent in patients with mild or moderate COVID-19 and usually appeared early. In addition, regular nucleic acid testing for SARS-CoV-2 should be considered for COVID-19 patients with certain airway comorbidities.

Ocular Manifestations in Granulomatosis with Polyangiitis Patients from Saudi Arabia

Eye and/or orbit involvement occurs in Granulomatosis with polyangiitis (GPA) patients frequently. The aim of our study was to describe the clinical manifestations, therapy and outcome of ocular involvement in our GPA patients. A retrospective study was conducted including patients with GPA who followed up in Rheumatology clinics during 1990-2016 at King Khalid University Hospital, Riyadh. Information relating to demographics, ocular manifestations, laboratory findings, therapy and outcome of GPA patients were noted. Ocular involvement was detected in 9 (39.1%) of the 23 GPA cases identified. The mean age of ocular GPA patients was 51.8 (range 27 - 62) years, the mean age at onset of disease was 39.6 (range 11 - 57) years and the mean duration of disease was 9.0 (range 2 - 19) years. Concomitant ear, nose, throat and sino-nasal manifestations occurred with ocular symptoms in 77.8% GPA patients. The most frequent manifestations were, eye pain (66.7%), scleritis/episcleritis (55.6%), eye redness and itching (55.6% each). Antineutrophil cytoplasmic antibodies (ANCA) were positive in 88.9% patients, 55.6% had c-ANCA and 33.3% had p-ANCA. Infections were observed in 22.2% of patients, which included pneumonia in one patient and esophageal candidiasis and bacterial meningitis in another. All patients received oral prednisolone, 44.4% received intravenous cyclophosphamide, 22.2% refractory cases received rituximab doses and the disease outcome was good. Comparison of ocular GPA with non-ocular GPA patients showed that 77.8% of ocular GPA patients had concomitant sino-nasal symptoms compared to 42.9% in non-ocular GPA patients and 22.2% of the ocular GPA patients had renal involvement compared to 64.3% in non-ocular GPA patients (p = 0.049). We found that the frequency of ocular manifestations in our GPA patients was similar to reports elsewhere, and the most frequent symptom was eye pain and scleritis/episcleritis.

Prevalence of Taste and Smell Dysfunction in Coronavirus Disease 2019

Early diagnosis of coronavirus disease 2019 (COVID-19) may help control the diffusion of the disease into the population. To investigate the presence of sinonasal manifestations at the onset of COVID-19 to achieve an earlier diagnosis. This retrospective telephone survey study investigated patients diagnosed with COVID-19 from March 5 to March 23, 2020, who were hospitalized or discharged from a single referral center. Patients who were unable to answer (intubated, receiving noninvasive ventilation, or deceased) or unreachable by telephone were excluded. Of 359 consecutive patients, 204 fulfilled the inclusion criteria; 76 were unable to answer, 76 were unreachable by telephone, and 3 refused. Sinonasal manifestations reported before COVID-19 diagnosis were studied with a validated questionnaire: Italian Sino-Nasal Outcome Test 22 (I-SNOT-22). If reduction of taste and/or smell was documented by item 5 of the I-SNOT-22, further inquiries were made to score them separately on a scale from 0 to 5, with 0 indicating no problem and 5 indicating problem as bad as it can be. The prevalence of sinonasal manifestations preceding COVID-19 diagnosis. Among the 204 patients enrolled (110 [53.9%] male; mean [SD] age, 52.6 [14.4] years), the median I-SNOT-22 total score was 21 (range, 0-73). I-SNOT-22 identified 116 patients (56.9%) with reduction of taste and/or smell, 113 (55.4%) with taste reduction (median score, 5; range, 2-5), and 85 (41.7%) with smell reduction (median score, 5; range, 1-5). Eighty-two patients (40.2%) reported both. Severe reduction of taste was present in 81 patients (39.7%), and severe reduction of smell was present in 72 patients (35.3%). Only 12 patients (14.8%) with severe taste reduction and 12 patients (16.7%) with severe smell reduction reported severe nasal obstruction. Severe reduction of taste and smell was more prevalent in female vs male patients (odds ratios, 3.16 [95% CI, 1.76-5.67] vs 2.58 [95% CI, 1.43-4.65]) and middle-aged vs younger patients (effect sizes, 0.50 [95% CI, 0.21-0.78] vs 0.85 [95% CI, 0.55-1.15]). No significant association was observed between smoking habits and severe reduction of taste (odds ratio, 0.95; 95% CI, 0.53-1.71) and/or smell (odds ratio, 0.65; 95% CI, 0.35-1.21). The findings of this telephone survey study suggest that reduction of taste and/or smell may be a frequent and early symptom of COVID-19. Nasal obstruction was not commonly present at the onset of the disease in this study. The general practitioner may play a pivotal role in identifying potential COVID-19 in patients at an early stage if taste and/or smell alterations manifest and in suggesting quarantine before confirmation or exclusion of the diagnosis.

Subjective and Objective Measurements of Sinonasal Manifestations in Patients With Autoimmune Disorders.

A number of autoimmune disorders (ADs) are associated with a spectrum of sinonasal manifestations comparable to chronic rhinosinusitis (CRS). Our objective was to study the subjective and objective measurements of sinonasal manifestations of ADs. Retrospective cohort study. All patients with ADs referred to our tertiary care rhinology clinic from 2008 to 2019 with sinonasal symptoms were compared to randomly selected cohorts of noneosinophilic CRS without nasal polyps (neCRSsNP) and eosinophilic CRSsNP (eCRSsNP). Demographic data, along with the 22-item Sino-Nasal Outcome Test (SNOT-22), Lund-Kennedy (LK) endoscopy score, Lund-Mackay (LM) computed tomography (CT) score, nasal crusting, and epistaxis were reviewed at presentation. Fifty-three patients with an AD (26 with sarcoidosis, 14 with systemic lupus erythematosus, 10 with granulomatosis with polyangiitis [GPA], and three with pemphigoid vulgaris) were identified, and compared to 75 randomly selected neCRSsNP patients and 75 eCRSsNP patients. Patients with an AD had an average SNOT-22 score of 44.4 (confidence interval [CI]: 34.6-51.2) compared to 25 (CI: 24.4-25.1) and 29.7 (CI: 20.3-29.7) for neCRSsNP and eCRSsNP patients, respectively (P < .0001), and an average LK endoscopy score of 5.3 (CI: 4.3-6.3), compared to 3.4 (P = .005, CI: 2.7-4) in neCRSsNP and 4.4 in eCRSsNP (P = .2, CI: 3.7-5). There was no significant difference in the CT score compared to both groups. Patients with an AD also scored significantly worse on all four SNOT-33 subdomains, nasal obstruction, nasal crusting, and epistaxis. Additionally, patients with GPA had the worst symptomatic and endoscopy scores. Patients with ADs presenting with sinonasal symptoms have a more severe subjective and objective presentation than patients with CRS without nasal polyps. 4 Laryngoscope, 131:255-259, 2021.

Odontogenic Maxillary Sinusopathies: a Radiological Classification.

Before implant surgery, a preoperative radiological evaluation is recommended for recognizing maxillary inflammatory conditions. In order to avoid a failure of the dental procedure and prevent medical-legal consequences, it is necessary to treat patients suffering of maxillary sinus pathologies. The classification proposed in our study aims to standardize the reference values for mucosal thickening and to verify their association with the odontogenic or disventilatory causes of sinus pathology. The maximum mucosal thickness was measured at the level of the maxillary sinus floor: mucosal thickness was present when greater than 1 mm and was classified according to its extension within the sinus cavity. Imaging data of 270 adult patients were included, performed for dental diagnosis and treatment planning, and they were divided into four main classes: Class I (85 pt.), mucosal thickness lesser than 2 mm, not pathological. Class II A (52 pt.), mucosal thickness between 2 and 5 mm, localized to the maxillary sinus floor: it is still considered non-pathological, and a "wait-and-see" approach is recommended. Class II B (46 pt.), mucosal thickness greater than 5 mm but localized at sinus floor: pathological mucosa with odontogenic etiology, requiring dental treatment. Class III A (39 pt.), mucosa thicker than 5 mm and concentric, most likely due to sinus ventilation disfunction: it requires maxillary antrostomy. Class III B (30 pt.), sinonasal manifestations such as nasal polyposis, retention cysts, mucocele, dental foreign body: pathological mucosa to be treated with ESS. Class IV (12 pt.), oroantral fistula: nasal endoscopic or oral approach. Our classification intends to suggest the better therapeutic option, in case of sinus pathology, according to the entity and pathogenesis of the mucosal thickening, in order to reduce complication and failure rate of dental surgery.

Evolution of sinonasal clinical features in children with cystic fibrosis

Objectiveto assess the evolution of sinonasal manifestations in children with cystic fibrosis, since the improvement of their prognosis over the last decades. Methodsan observational, monocentric study with a retrospective cohort. We included 173 children (from 4 to 18 years old) with cystic fibrosis followed at the pediatric cystic fibrosis center of lyon, france. We collected respiratory, infectious and nutritional data, sinonasal complaints and physical examination at the onset of sinonasal symptoms (t-0), at the most severe of evolution (t-max) and at the end of followup (t-end). Resultssinonasal symptomatology appeared early around 5.4 years old, then rapidly reached the maximum at 6.9 years and finally improved during childhood (p < 0.0001), reaching scores at t-end significantly better than at t-0 (p < 0.0001). This evolution was significant for nasal obstruction, rhinorrhea and snoring. The other symptoms were rarer, with no significant 38,7% at t-max (p < 0.0001), and 29,5% at t-end (p = 0.52). The lildholdt score, turbinate hypertrophy and medial bulging of medial wall of the maxillary sinus followed the same evolution (p < 0.003). There was no association between sinonasal evolution and cystic fibrosis disease at infectious, respiratory or nutritional level. Conclusionit is the only recent study evaluating the evolution of each sinonasal manifestations in children with cystic fibrosis. Rhinosinusitis improved during childhood, reaching better scores than at the beginning of management. This particular improvement may be related to good effectiveness to ent management, but also to a positive effect of nasal cavity growth, independently to extra-ent manifestations.

Sinonasal Manifestations in Children with Cystic Fibrosis

Background: Involvement of the upper respiratory tract and pathological changes in the nose and paranasal sinuses are common in patients with cystic fibrosis. Objectives: We aimed to identify sinonasal manifestations in Iranian children with cystic fibrosis. Methods: Forthy seven children with cystic fibrosis were enrolled and symptoms related to the nose and paranasal sinuses were recorded using a questionnaire. The patients’ nasal and sinus CT scan were investigated in terms of findings related to cystic fibrosis. Results: The most common complaint was nasal congestion (61.7%). Ground glass opacity in the nasal cavity was found in 63.8% of the patients. Postnasal drip was observed in 36.2% of the patients. In nasal endoscopic examination, 15 (31.9%) patients had clear polyps in the nasal cavity. Conclusions: History, CT scans, and initial clinical examination (rhinorrhea in anterior rhinoscopy) does not allow accurate diagnosis of chronic rhino sinusitis or nasal polyps in children with cystic fibrosis.

Rhinotillexomania in a Cystic Fibrosis Patient Resulting in Septal Perforation

Cystic fibrosis (CF) is a multisystem disease that can have significant sinonasal manifestations. Viscous secretions are one of several factors in CF that result in chronic sinonasal pathology, such as sinusitis, polyposis, congestion, and obstructive crusting. Persistent discomfort and nasal manifestations of this disease significantly affect quality of life.

Sinonasal quality of life outcomes following laser treatment of epistaxis related to hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) is an inherited vascular disorder which manifests as recurrent, episodic, and potentially debilitating epistaxis. In this study, we aim to (1) characterize baseline sinonasal symptoms for HHT patients and to (2) analyze changes in sinonasal symptoms before and after laser surgical treatment for HHT. We performed a retrospective chart review of sinonasal outcome test-22 (SNOT-22) scores before and after one or more laser surgical treatments for HHT-related epistaxis between January 1, 2010 and December 1, 2015 at a tertiary academic medical center with an HHT Foundation-approved Center of Excellence. Preoperative and all subsequent postoperative SNOT-22 scores (short-term, <45days and long-term, ≥45days) were compared. Twenty consecutive HHT patients underwent 51 laser surgeries for recurrent epistaxis. Mean preoperative, short-term postoperative, and long-term postoperative SNOT-22 scores were 34.6 ± 5.4, 33.9 ± 5.5, and 18.8 ± 4.6, respectively. When analyzing subcategory scores, there was a significant improvement in the rhinologic domain from short-term to long-term postoperatively (13.5 vs. 7.3; p = 0.004), in the non-rhinologic otolaryngic domain from short-term to long-term postoperatively (2.8 vs. 1.7; p = 0.014), and in the psychological domain from preoperative and short-term postoperative to long-term postoperatively (12.2 and 10.0 vs. 6.0; p = 0.015 and 0.01, respectively). Following laser surgery for HHT-related epistaxis, patients' rhinologic symptoms worsened on the short run postoperatively but improved over time. The main benefit of laser treatment appears to be long-term improvement in psychological factors. This study once again underscores the important role of the otolaryngologist in managing sinonasal manifestations of HHT.

Anti-IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss): Data on Seventeen Patients.

To describe the efficacy and safety of omalizumab, an anti-IgE monoclonal antibody, in patients with refractory and/or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). We conducted a nationwide retrospective study including EGPA patients who received omalizumab. Response was defined as the absence of asthma and/or sinonasal exacerbations with a prednisone dosage of ≤7.5 mg/day (complete response) or >7.5 mg/day (partial response). Seventeen patients (median age 45 years) received omalizumab for severe steroid-dependent asthma (88%) and/or sinonasal involvement (18%). After a median follow-up of 22 months, 6 patients (35%) achieved a complete response, 5 patients (30%) achieved a partial response, and 6 patients (35%) had no improvement. The median Birmingham Vasculitis Activity Score decreased from 2.5 at baseline to 0.5 at 12 months. The median number of exacerbations per month decreased from 1 at baseline to 0 at 12 months, and the median forced expiratory volume in 1 second increased from 63% of the percent predicted at baseline to 85% of the percent predicted at 12 months. The median prednisone dosage decreased from 16 mg/day at baseline to 11 mg/day at 6 months and 9 mg/day at 12 months. Omalizumab was discontinued in 8 patients (47%) during follow-up, because of remission (12.5%), adverse event despite disease remission (12.5%), refractory disease (25%), or relapse (50%). Relapses included retrobulbar optic neuritis attributable to EGPA in 2 patients and severe asthma flare in 2 others. The results of this study suggest that omalizumab may have a corticosteroid-sparing effect in EGPA patients with asthmatic and/or sinonasal manifestations, but reducing the corticosteroid dose may also increase the risk of severe EGPA flares, which raises the question of the safety of omalizumab in patients with EGPA.

Olfactory Dysfunction in IgG4-Related Disease.

IgG4-related disease is a newly recognized systemic disease, and its elucidation is progressing. However, little is known about its sinonasal manifestations. The aim of this study was to assess the olfaction of patients with IgG4-related disease. Twenty-five patients with IgG4-related disease underwent T&T olfactometry to measure olfactory function. We analyzed the clinical features, including serum IgG4 and IgE levels, involved organs, and sinonasal computed tomography scores to explore the etiology of olfactory dysfunction. Thirteen patients with IgG4-related disease were found to have moderate to severe olfactory dysfunction (52%). There were no differences in the clinical features between the olfactory dysfunction group and the normal group. In 7 patients, the inferior turbinate was biopsied to study the correlation between olfaction score and the number of IgG4-positive cells, but no such correlation was found. Six hyposmia patients recovered to a normal state. Five patients recovered after corticosteroid treatment and 1 recovered spontaneously. We found that the prevalence of olfactory dysfunction was high in patients with IgG4-related disease and that it could be reversed. Olfactory dysfunction appears to be a novel important manifestation of IgG4-related disease.

Higher antineutrophil cytoplasmic antibody (C-ANCA) titers are associated with increased overall healthcare use in patients with sinonasal manifestations of granulomatosis with polyangiitis (GPA).

Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous airway inflammation and vasculitis. Sinonasal involvement occurs in more than 80% cases, with antineutrophil cytoplasmic antibody (C-ANCA) titers used as a marker of disease severity. The purpose of this study was to determine whether C-ANCA levels impact radiographic findings and healthcare use in patients with sinonasal GPA. A retrospective review was performed on GPA patients evaluated in a multidisciplinary rheumatologic/otolaryngologic clinic from 2008 to 2013. Data were collected with respect to age, gender, clinical presentation, C-ANCA titers, Lund-Mackay (LM) scores, surgical interventions, and healthcare use, the latter of which were determined by assessing the number of rheumatology/otolaryngology clinic visits, computed tomography (CT) scans, and email/telephone encounters. A total of 44 patients were identified, 11 male and 33 female. Sinonasal manifestations were evident in 70.4%, with chronic rhinosinusitis (CRS) (41.9%), septal perforation (38.7%), and crusting (32.2%) the most common findings. No significant differences in number of CT scans (p = 0.10) or mean LM scores (p = 0.47) were found between patients with more than or equal to 1:80 and less than 1:80 C-ANCA titers, respectively. However, overall healthcare use was increased in the more than or equal to 1:80 C-ANCA group (n = 28) compared with less than 1:80 (n = 16), with a significantly greater number of rheumatologic/otolaryngologic encounters (mean 121 versus 69.2, p = 0.03) noted. When otolaryngologic healthcare use was specifically examined, the average number of encounters was also higher in more than or equal to 1:80 C-ANCA patients (31.9 versus 22.9), but this difference was not statistically significant (p = 0.16). Sinonasal GPA patients with presenting C-ANCA titers more than or equal to 1:80 demonstrated significantly greater overall healthcare use than their lower C-ANCA level counterparts (less than 1:80). However, no significant differences in otolaryngology resource use or LM scores were evident between the two titer groups.

A Rare Manifestation of Crohn's Disease: Sinonasal Granulomatosis. Report of a Case and Review of Literature

Crohn’s disease is a granulomatous inflammatory bowel disease. Its pathologic findings include non-contiguous chronic inflammation and non-caseating granulomas, sometimes with extra-intestinal localizations. Sinonasal manifestations of Crohn’s disease are quite rare and only a few cases have been reported up to date in the worldwide literature. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforation. We report a case of sinonasal granulomatosis revealing Crohn’s disease in a 22-year-old woman and go over the available literature on sinonasal involvement in Crohn’s disease.

Sinonasal Symptomatic Outcomes following Laser Treatment of Epistaxis Related to Hereditary Hemorrhagic Telangiectasias

Objectives: (1) Characterize baseline sinonasal symptoms for hereditary hemorrhagic telangiectasia (HHT) patients. (2) Analyze changes in sinonasal symptoms before and after laser surgical treatment for HHT. Methods: Retrospective chart review of sinonasal outcome test-22 (SNOT-22) scores before and after one or more laser surgical treatments for HHT-related epistaxis between January 1, 2010, and December 31, 2013, in a tertiary academic medical center with an HHT Foundation-approved Center of Excellence. All HHT patients who had undergone one or more laser surgical treatments by 1 of 2 otolaryngologists were included in the analysis. Preoperative and all subsequent postoperative SNOT-22 scores were compared using unpaired t tests. Subgroup analysis was performed for patients who underwent more than 1 laser surgery. Results: A total of 21 consecutive HHT patients underwent laser surgery for recurrent epistaxis. Compared with preoperative scores, patients reported significantly improved ( P &lt; .05) SNOT scores at 2 weeks postoperatively for dizziness, difficulty falling asleep, waking up at night, getting a good night’s sleep, waking up tired, reduced concentration, feeling of frustration, and sadness. The same cohort reported significantly worse nasal obstruction at 2 weeks postoperatively. Patients who underwent more than one surgery experienced overall symptomatic improvement after the second surgery. Conclusions: HHT patients experienced a short-term improvement in sinonasal symptoms, especially those producing social and emotional consequences, following laser surgery for HHT-related epistaxis. This study once again underscores the important role of the otolaryngologist in managing sinonasal manifestations of HHT.