[Eosinophils in otorhinolaryngology: friend or foe?] / Eosinophile in der HNO-Heilkunde: Freund oder Feind?

This article summarizes the essential aspects of current knowledge about eosinophils, classifies eosinophilia in terms of the dimensions healthy or harmful reaction, takes a specific look at eosinophils in the field of otorhinolaryngology, and gives recommendations for diagnostic workup and therapeutic intervention in case of proven eosinophilia, particularly as an adverse drug reaction. The thoughts are inspired by communications at the 29th Congress of the European Rhinologic Society in Sofia, Bulgaria, 2023.

[Fundamentals and practice of the application of nasal packing in sinonasal surgery]. / Grundlagen und Praxis der Anwendung von Nasentamponaden in der endonasalen Chirurgie.

BACKGROUND AND OBJECTIVES: This paper presents an overview on nasal packing materials which are available in Germany. The current literature is analyzed whether there are robust criteria regarding use nasal packing after sinonasal surgery, whether there are fundamental and proven advantages or disadvantages of products, and what this means in clinical practice. MATERIALS AND METHODS: Selective literature analysis using the PubMed database (key words "nasal packing", "nasal tamponade", "nasal surgery", "sinonasal surgery", or "sinus surgery"), corresponding text books and resulting secondary literature. RESULTS AND CONCLUSIONS: Because of systematic methodological shortcomings, the literature does not help in the decision-making about which nasal packing should be used after which kind of sinonasal surgery. In fact, individual approaches for the many different clinical scenarios are recommended. In principle, nasal packing aims in hemostasis, should promote wound healing, and should not result in secondary morbidity. Nasal packing materials should be smooth (non-absorbable materials), inert (absorbable materials), and should not exert excessive pressure. Using non-absorbable packing entails the risk of potentially lethal aspiration and ingestion. For safety reasons inpatient control is recommended as long as this packing is in situ. With other, uncritical packing materials and in patients with special conditions, outpatient control could be justified.

[Balloon dilatation of subglottic stenoses, especially in granulomatosis with polyangiitis]. / Ballon-Dilatation subglottischer Stenosen insbesondere bei Granulomatose mit Polyangiitis.

INTRODUCTION: Subglottic stenoses (SGS) are constrictions in the region below the vocal folds, that can pose a life-threatening problem for those affected. The aim of this research project was to identify the patient group for which the treatment of SGS by balloon dilatation can lead to long-term success. METHODS: 14 patients with SGS were examined (before and up to12 months after intervention) using pulmonary function tests, laryngoscopies and two questionnaires (Clinical Chronic Obstructive Pulmonary Disease Questionnaire), (modified Medical Research Council Dyspnoea Scale) regarding the respiratory situation. Additionally, the number of necessary interventions, the degree of stenosis (according to Myer Cotton and measured in millimeters) were documented. RESULTS: Four patients who required only one intervention with the balloon showed better pre-interventional respiratory function and a higher subjective stress level. Lung function tests and evaluation of the questionnaires showed an improvement in the respiratory situation and state of health of all 14 patients after the initial intervention. The degree of stenosis measured in millimeters shows that patients with a stenosis diameter of less than 4 mm only required one intervention. All patients who received an intervention using a 12-mm-balloon had to be treated several times. CONCLUSIONS: Patients with a high degree of stenosis and a tendency towards greater subjective stress and less objective restriction of the respiratory function appear to have a higher probability of long-term successful treatment with the balloon. Furthermore, the choice of balloon size seems to be an additional decisive factor for the success of an intervention.

[Treatment of chronic rhinosinusitis with nasal polyps (CRSwNP) with monoclonal antibodies (biologics): S2k guideline of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery (DGHNO-KHC), and the German College of General Practitioners and Family Physicians (DEGAM)]. / Therapie der chronischen Rhinosinusitis mit Polyposis nasi (CRScNP) mit monoklonalen Antikörpern (Biologika): S2k-Leitlinie der Deutschen Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie (DGHNO-KHC) und der Deutschen Gesellschaft für Allgemeinmedizin und Familienmedizin (DEGAM).

Monoclonal antibodies (so-called biologics) can be prescribed for chronic rhinosinusitis with nasal polyps (CRSwNP) within the scope of their market authorization. However, their prescription is limited to severe CRSwNP without disease control, whereby certain requirements must be met. Dupilumab, omalizumab, and mepolizumab have currently gained market authorization, with adequate evidence for their efficacy and safety available in the literature. It can be assumed that other biologics will be approved for this indication in the future. The severity of disease and the efficacy of treatment should be assessed objectively and subjectively before treatment initiation and after an appropriate duration, respectively. The documentation sheet proposed in this guideline chapter can be used for the assessments. In the presence of relative contraindications, a treatment should only be initiated after differentiated consideration by an experienced physician in the sense of a case-by-case decision. In summary, this guideline chapter aims to contribute to high-quality care of adult patients with these therapies in view of the increasing evidence for treatment with these substances and the increasing number of market authorizations of different biologics.

[The impact of smoking habit alteration on prognosis of head and neck cancer patients]. / Eine Änderung des Rauchverhaltens beeinflusst das Überleben von HNSCC-Patienten.

INTRODUCTION: The influence of smoking on survival in patients with HNSCC is well documented in the literature. There is little data on changes in smoking habits after diagnosis. Here, the effect on survival of the reduction of smoking compared to full smoking cessation is investigated. PATIENTS AND METHODS: Patient records and tumor documentation of 643 consecutive HNSCC cases of the Head and Neck Tumor Center of the University Hospital Kiel are evaluated retrospectively: smoking habits before and after treatment and survival are evaluated. RESULTS: Change in smoking behavior at the initial diagnosis of HNSCC leads to a significant positive effect on the prognosis compared to continued smoking. There is no difference between smoke reduction and weaning. This effect is based solely on those patients who are treated exclusively by surgery. Lifelong non-smokers have a significant survival advantage over active and ex-smokers, with no difference between the latter two groups. CONCLUSIONS: The positive influence of changed smoking habits on the prognosis runs parallel to the negative direct effect of active smoking on therapy, which is attributed to peritumoral hypoxia with a negative effect on the effectiveness of R(C)T. The positive effect of the change in smoking behaviour during surgery alone is most likely due to reduced peri-operative complications. Patients should be encouraged to at least minimize smoking with the cancer diagnosis. In addition, former smokers should be considered active smokers for survival estimates and therapy planning.

[Smoking and co-morbidity - it's impact on dose achievement in radio(chemo)therapy for HNSCC]. / Rauchen und Komorbidität ohne Einfluss auf geplante Zieldosis der Radio(chemo)therapie.

Smoking worsens the prognosis of patients with HNSCC. Furthermore, smoking is associated with the prevalence of co- and multimorbidity, so that it is assumed that not smoking per se, but co-/multimorbidity worsens the prognosis due to lack of compliance to therapy, e. g. by reducing the dose of the planned radio(chemo)therapy (RCT). However, data on this topic are currently sparse and contradictory, especially for HNSCC.Patient records and tumor documentation of 643 consecutive cases of the Head and Neck Tumor Center of the University Hospital Kiel were retrospectively evaluated. Patient characteristics and smoking habits were recorded and correlated with co-/multimorbidity and treatment course.The 643 patient files examined show that 113 (17.6 %) patients did not smoke, 349 (54.3 %) were active and 180 (28 %) patients had previously smoked. 315 (49 %) are treated exclusively by surgery; 121 (18.8 %) by surgery + adjuvant RCT and 72 (11.2 %) by surgery + adjuvant RT. 111 (17.3 %) receive a primary RCT and 24 (3.7 %) a primary RT. 131 (20.4 %) show co-/multimorbidity and 512 (79.6 %) do not. Smoking (> 10 py) is significantly associated with comorbidity (p = 0.002). However, smoking and comorbidity, neither alone nor in combination, are correlated with the achievement of the target dose of RCT (p > 0.05).As expected, smoking is significantly linked to co-/multimorbidity. Dose reduction of R(C)T is just as frequent in active smokers and patients with co-/multimorbidity as in non-smokers and patients without co-/multimorbidity. Thus, smoking and co-/multimorbidity influence the prognosis in other ways than by interfering with planned therapy regimens.

Changes in the composition of the upper respiratory tract microbial community in granulomatosis with polyangiitis.

Dysbiosis¸ i.e. changes in microbial composition at a mucosal interface, is implicated in the pathogenesis of many chronic inflammatory and autoimmune diseases. To assess the composition of the microbial upper respiratory tract (URT) community in patients with granulomatosis with polyangiitis (GPA), we used culture-independent high-throughput methods. In this prospective clinical study, nasal swabs were collected from patients with GPA, patients with rheumatoid arthritis (RA, disease control), and healthy controls. Nasal bacterial taxa were assessed using V3-V4 region 16S rRNA amplicon sequencing. Staphylococcus aureus, Haemophilus influenza, and entero- and rhinoviruses were detected using qPCR. Unbiased metagenomic RNA sequencing (UMERS) was performed in a subset of samples to determine the relative abundance of bacterial, fungal, and viral species. A trend toward reduced microbiome diversity was detected in GPA samples compared with healthy controls. The abundance of bacterial taxa and microbial richness were significantly decreased in GPA samples compared with RA samples. The relative abundance of bacterial families shifted, with increased Planococcaceae and decreased Moraxellaceae, Tissierellaceae, Staphylococcaceae, and Propionibacteriaceae in GPA and RA. Further, decreased abundance of Corynebacteriaceae, and Aerococcaceae was observed in GPA samples. Significantly more colonization of S. aureus was seen in the nasal microbiome of GPA compared with RA and healthy control samples. H. influenzae colonization was also observed in GPA samples. UMERS detected the presence of rhinoviral sequences in some GPA samples. Thus, our study uncovered changes in the URT microbial composition in patients with GPA and RA, suggesting that both immunosuppression and disease background affect the URT microbiome. Complex alterations of host-microbiome interactions in the URT could influence chronic endonasal inflammation in GPA.

Manifestation of granulomatosis with polyangiitis in head and neck.

OBJECTIVES: Granulomatosis with polyangiitis is a rare autoimmune disease of the group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Involvement of the ear, nose and throat (ENT)-region is only described in a few case series and case reports. The objective of this study is to systematically characterise the ENT-involvement in a large series of GPA patients. METHODS: GPA patients examined in the Department of Otorhinolayngology of the Christian-Albrechts-University of Kiel between 1990 and 2012 were included. Diagnosis was based on histological, serological and clinical parameters. GPA patients were examined in a standardised way based on the Ear Nose and Throat Activity Score (ENTAS) or its precursor. Medical history, ENT examination, diagnostic findings (ear, nose) and cranial radiology were documented cumulatively. RESULTS: A total of 230 GPA patients were included in this study. Over 95% of them showed ENT-involvement. 59% of the patients showed nasal obstructions, 57% a loss of smell. A hearing loss was diagnosed in 23% of the patients, 50% involvement in MR or CT scans and 15% showed laryngeal involvement. CONCLUSIONS: The data of the largest monocentric study presented here demonstrate a frequent ENT-involvement in GPA patients. Rhinological and ontological manifestations are most common.

[Manifestation of eosinophilic granulomatosis with polyangiitis in the head and neck area over time taking systemic disease activity into consideration]. / Manifestation der eosinophilen Granulomatose mit Polyangiitis im Kopf-Hals-Bereich im zeitlichen Verlauf unter Berücksichtigung der systemischen Krankheitsaktivität.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare granulomatous vasculitis of the small and medium sized blood vessels of unknown etiology. A regular involvement of the head and neck area is known. Since it is not yet known whether the inflammatory processes in the head and neck area are parallel to the systemic disease activity, the course of systemic and local disease activity was investigated in a comparative study. MATERIAL AND METHODS: Between 1990 and 2010 the disease progression of 25 patients with a total of 90 consultations and an average observation period of 7.02 years was recorded. The anamnesis, clinical observation and testing were performed in order to describe ear nose and throat (ENT) manifestations. Moreover, systemic data, such as the Birmingham Vasculitis Activity Score (BVAS), the Vasculitis Damage Index (VDI) and the serological parameters C­reactive protein (CRP), eosinophilic cationic protein (ECP) and absolute eosinophil count (EOS) were collected. In order to define the activity in the head and neck area the existing manifestations were summarized and statistically compared to the systemic data using Spearman's rank correlation coefficient. RESULTS: Overall the statistical analysis showed a very weak correlation between the disease activity in the ENT area and systemic disease activity, which is not considered as significant. Comparing activity in the nose region to systemic activity, significant values were found in 22.9% of all consultations. Comparing activity in the ear region to systemic activity, 11.8% of all consultations showed significant values. CONCLUSION: The results indicate that the disease activity in the head and neck area is independent of the systemic activity. Moreover, they point out a potential independent autoimmune inflammatory process in the mentioned regions; therefore, treatment should include long-term follow-up and an interdisciplinary management.

[The new German guideline on rhinosinusitis - an abbreviated version]. / Die neue Leitlinie Rhinosinusitis ­ eine Miniaturversion.

At the beginning of this year, the new German guideline on rhinosinusitis was published as a joint guideline of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery and the German College of General Practitioners and Family Physicians. The guideline was designed for the treatment of adult patients with inflammatory diseases of the paranasal sinuses and is addressed to all medical specialties involved in the management of these diseases. The current challenge is the implementation of this guideline in the clinical daily routine. For this purpose, an abbreviated version (miniature) was designed.

Intra- and inter-rater reliability of the modified ENT assessment score (ENTAS 2) in granulomatosis with polyangiitis: a prospective randomised trial.

OBJECTIVES: Ears nose and throat (ENT) involvement is found on a substantial proportion of patients with granulomatosis with polyangiitis (GPA). Structured, reliable ENT assessment is essential in the management of GPA patients. It is the aim of this study to determine the repeatability (intra-rater reliability) and reproducibility (inter-rater reliability) of the ENT Assessment Score (ENTAS 2). METHODS: The ENTAS 2 built the fundament of the prospective randomized trial. Anamnestic, video endoscopic and diagnostic data of 47 patients were used. A single assessor reference was created. GPA/ENT activity and damage were evaluated by three physicians at two time points (T1/T2). GPA/ENT activity was evaluated in dichotomy (yes/no) and grading (none/mild/moderate/high) and GPA/ ENT damage in dichotomy. RESULTS: ENTAS 2 activity evaluations intra-rater reliability was 80.7% (κ=0.56) in dichotomy and 72.8% (κ=0.41) in grading. ENTAS 2 damage evaluations showed 87.8% (κ=0.74) intra-rater reliability. ENTAS 2 activity inter-rater reliability at T1 was 62.2% (κ=0.43) in dichotomy and 51.1% (κ=0.29) in grading, at T2 it was 68.2% (κ=0.48) in dichotomy and 55.32% (κ=0.33) in grading. Inter-rater reliability of ENTAS 2 damage evaluation was 84.4% (κ=0.79) at T1 and 72.5% (κ=0.64) at T2. CONCLUSIONS: ENTAS 2 intra-rater reliability was high in dichotomous and graded GPA/ENT activity and damage evaluations. Inter-rater reliability was high in dichotomous activity and damage evaluations, but low in graded activity evaluations. The data demonstrate that the ENTAS 2 is a reliable score-system considering GPA/ENT activity and damage evaluations.

Manifestation of eosinophilic granulomatosis with polyangiitis in head and neck.

INTRODUCTION: 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. METHOD: 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. RESULTS: 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. CONCLUSION: 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.

EGPA: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) as a special presentation of chronic rhinosinusitis with nasal polyps (CRSwNP).

INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) was formerly known as Churg-Strauss syndrome. The condition is characterized by disseminated necrotizing vasculitis with extravascular granulomas associated with hypereosinophilia. The vasculitides affect small vessels and are associated with antineutrophil cytoplasmic antibodies (ANCAs) detectable in the blood. Distinguishing between type 2-mediated chronic airway inflammation such as chronic rhinosinusitis with nasal polyps (CRSwNP) without vasculitis can be clinically challenging and should be considered. MATERIALS AND METHODS: Immunological background, diagnosis, and therapy of EGPA were identified through literature searches in Medline, PubMed, as well as national and international studies (ClinicalTrials.gov) and the Cochrane Library. Human studies published up to and including 10/2023 on the topic were considered. RESULTS: In cases of deteriorating general health with previously known eosinophilic inflammation of the upper and lower airways, EGPA and its interdisciplinary investigation should be considered. Various types of eosinophilic inflammation and syndromes must be considered differentially. CONCLUSION: Characterization of mucosal airway inflammation through biomarker determination is meaningful and occasionally makes the difference for targeted therapy.

Monitoring mepolizumab treatment in chronic rhinosinusitis with nasal polyps (CRSwNP): Discontinue, change, continue therapy?

BACKGROUND: Chronic rhinosinusitis with nasal polyps (CRSwNP) is a multifactorial inflammatory disease of the mucous membranes of the nose and sinuses. Eosinophilic inflammation is described as a common endotype. The anti-IL-5 antibody mepolizumab was approved in November 2021 as an add-on therapy to intranasal glucocorticosteroids for the treatment of adults with severe chronic rhinosinusitis with nasal polyps when systemic glucocorticosteroids or surgery do not provide adequate disease control. While national and international recommendations exist for the use of mepolizumab in CRSwNP, it has not yet been adequately specified how this therapy should be monitored, what follow-up documentation is necessary, and when it should be discontinued if necessary. MATERIALS AND METHODS: A literature search was performed to analyze previous data on the treatment of CRSwNP with mepolizumab and to determine the available evidence by searching Medline, Pubmed, the national and international trial and guideline registries, and the Cochrane Library. Human studies published in the period up to and including 10/2022 were considered. RESULTS: Based on the international literature and previous experience by an expert panel, recommendations for follow-up, adherence to therapy intervals, and possible therapy breaks as well as discontinuation of therapy when using mepolizumab for the indication CRSwNP in the German healthcare system are given on the basis of a documentation sheet. CONCLUSION: Understanding the immunological basis of CRSwNP opens up new non-surgical therapeutic approaches with biologics for patients with severe, uncontrolled courses. Here, we provide recommendations for follow-up, adherence to therapy intervals, possible therapy pauses, or discontinuation of therapy when mepolizumab is used as add-on therapy with intranasal glucocorticosteroids to treat adult patients with severe CRSwNP that cannot be adequately controlled with systemic glucocorticosteroids and/or surgical intervention.

A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients.

OBJECTIVE: To evaluate a vasculitis centre based management strategy for eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA). METHODS: A retrospective cohort study at a vasculitis referral centre was performed. All EGPA patients admitted from 1990 to 2009 were included. A structured interdisciplinary work-up for proof of diagnosis, Disease Extent Index and Birmingham Vasculitis Activity Score was performed. Immunosuppressive therapy was initiated and regularly adapted. Treatment targets were induction and maintenance of remission according to definitions given by the European League Against Rheumatism and the European Vasculitis Study Group. Outcomes were mortality, rate of remission, relapses, adverse events and prednisolone-dose. RESULTS: Out of 269 patients with suspected EGPA 150 fulfilled the inclusion criteria. Of those, 104 had more than one follow-up visit resulting in a mean follow up of 53±4.9 months. By using additional data sources the follow-up concerning survival was extended to 92±5 month. Severe organ manifestations occurred at heart (46%), kidney (18%) and lungs (10%). Cyclophosphamide was used in 107 patients (71%). The prednisolone-doses of all patients were within the targeted range (i.e. ≤7.5 mg) in 69% of the total follow-up time; the median dose at end of follow-up was 5mg/d. The 10-year survival rate was 89% resulting in mortality comparable to the general population (SMR 1.29). Only patients with cardiac failure associated with EGPA had an increased mortality (SMR 3.06). CONCLUSIONS: Regular re-evaluation and target-orientated adaption of therapy may lead to normalization of life expectancy and attenuation of disease progression. Continued centre based interdisciplinary treatment should be standard of care.

Cartilage destruction in granulomatosis with polyangiitis (Wegener's granulomatosis) is mediated by human fibroblasts after transplantation into immunodeficient mice.

A key feature of granulomatosis with polyangiitis (GPA; or Wegener's granulomatosis) is the granulomatous inflammation of the upper respiratory tract, which leads to the subsequent destruction of adjacent tissues. The aim of our work was to study the histopathological and cellular components of tissue destruction of human GPA tissue transplanted into immunodeficient mice. Biopsy specimens from patients with active GPA (n = 10) or sinusitis (controls, n = 6) were s.c. co-implanted with healthy allogeneic human nasal cartilage into immunodeficient pfp/rag2(-/-) mice. Transplants were examined for their destructive capability of the allografted human cartilage. In addition, nasal fibroblasts from patients with GPA (n = 8) and control healthy nasal fibroblasts (n = 5) were cultured, and cell proliferation and apoptosis were quantified. mRNA and protein levels of matrix metalloproteinases and cytokines were evaluated at baseline and after proinflammatory stimulation. GPA implants showed massive destruction of the co-implanted human cartilage, whereas cartilage destruction was only marginal in control samples. Destruction was mediated by human fibroblasts and could be inhibited by corticoid treatment. The up-regulated production of matrix metalloproteinases 1, 3, and 13 and cytokines IL-6 and IL-8 was found in vivo and in vitro. Although proliferation of isolated fibroblasts was comparable between GPA and controls, GPA samples showed a significant delay of apoptosis. The destruction of nasal cartilage in GPA is mainly mediated by fibroblasts that can be blocked by corticosteroids, and this tissue destruction is not dependent on the influx of leukocytes.

Orbital masses in granulomatosis with polyangiitis are associated with a refractory course and a high burden of local damage.

OBJECTIVES: To identify and characterize patients with orbital masses in a monocentric cohort of 1142 GPA patients followed up from 1990 until the end of 2010 with regard to disease stage, local orbital inflammation, course of disease and outcome and to assess the efficacy of immunosuppressive treatment. METHODS: All GPA patients fulfilling ACR criteria or Chapel Hill Consensus Conference definitions or who had localized GPA and who developed orbital masses were evaluated regarding the course and outcome of the orbital masses (assessed by MRI, ophthalmologist and ENT specialist), all other clinical manifestations, disease stages, ANCA status, immunosuppression and its side effects and surgical procedures. RESULTS: Of 1142 GPA patients 58 developed orbital masses during a median follow-up of 101.5 months (range 23-255 months). Forty patients fulfilled the inclusion criteria and had complete clinical assessments [44% females, median age 43 (20-74) years, 85% ANCA positive]. Seventy-five per cent (29/40) had systemic disease when orbital masses occurred; both orbits were affected in 30%. Seventy-two per cent had evidence of infiltration from paranasal sinuses. Under highly potent immunosuppression (mostly CYC and glucocorticoids), 41% were refractory, 24% had unchanged activity, 24% showed a response and 8.1% had complete remission. Forty-four per cent had relapses of orbital masses. Seventy-two per cent developed visual impairment, 19% suffered blindness. Blindness was associated with a longer time to remission and a relapsing and refractory course. CONCLUSION: Orbital masses are a rare manifestation of GPA and are characterized by a refractory course and by a high rate of local damage. Patients with a refractory or relapsing course are at higher risk of developing blindness.

Magnetic resonance imaging in patients with granulomatosis with polyangiitis (Wegener's) and subglottic stenosis.

OBJECTIVE: To evaluate the ability of MRI to detect subglottic stenosis and to differentiate between active and inactive subglottic inflammation in patients with granulomatosis with polyangiitis (GPA). MATERIALS AND METHODS: MRI studies of the larynx of 18 GPA patients with suspected SGS were included. The MRI protocol included T1- and T2-weighted and STIR-sequences, dynamic contrast enhancement (DCE) and diffusion weighted imaging (DWI). Two independent observers reviewed the MR images. SGS were identified and quantified, inflammatory activity was assessed using edema imaging, DCE and DWI. Final MRI diagnoses were compared to the clinical, laryngoscopic and histopathologic results. RESULTS: MRI confirmed SGS in all GPA patients with significant narrowing of the airway lumen and thickening of subglottic wall. Assessing the subglottic inflammatory activity, MRI showed a sensitivity of 87.5 % and a specificity of 60.0 %. Interrater agreement was κ = 0.769. Of the different MR technical approaches tested, edema imaging was most sensitive and specific. DWI led to significant differences in the apparent diffusion coefficient between active and inactive subglottic inflammation. No significant differences were found with DCE imaging. CONCLUSION: MR imaging has shown the ability to detect and grade SGS in patients with GPA. It non-invasively assesses the status of inflammatory activity utilizing edema sensitive sequences and DWI.