Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

A Case of Cutaneous Malakoplakia in the Head and Neck Region and Review of the Literature.

Malakoplakia is a rare inflammatory condition that typically occurs in the urinary tract. The cutaneous form is less prevalent, and most commonly occurs in the perianal or genital regions. Here we present a 61 year old lady with cutaneous malakoplakia of the neck, which was successfully treated with surgical excision and a prolonged course of ciprofloxacin. We follow our case report with a discussion and literature review of all seventeen previously reported cutaneous head and neck malakoplakia cases from the literature. A diagnosis of cutaneous malakoplakia should be considered in nodular, ulcerated or discharging lesions that are refractory to treatment. Histology is essential, not only to diagnose malakoplakia, but also to exclude other important differential diagnoses, such as malignancy. Combined surgical excision and prolonged antibiotic courses appear to have the highest success rate. Antibiotics should be culture specific, but quinolones appear to be the best empirical choice.

Radiologically predicting when a sternotomy may be required in the management of retrosternal goiters.

OBJECTIVES: Surgery remains the most effective treatment for retrosternal goiters. These commonly present as asymptomatic lesions in elderly patients, but may also cause airway and esophageal compression and, less commonly, may also be malignant. Although the majority of these goiters are amenable to transcervical thyroidectomy, in a minority of patients sternotomy is required. The ability to predict the need for sternotomy before operation would allow for safer surgery and operative counseling, as well as improved logistical efficiency if coordination with thoracic surgeons is required. In this report, we assess the radiologic factors that might be predictive of the need for sternotomy. METHODS: We performed a retrospective review of 97 retrosternal goiters for which thyroidectomy was performed within the otolaryngology department at Addenbrooke's Hospital, Cambridge, between 2001 and 2011. There were a total of 80 cervical excisions and 17 cases in which sternotomy was required. A detailed computed tomographic analysis of these 17 cases was undertaken to assess the predictive factors for the requirement of sternotomy. The factors assessed included posterior mediastinal extension, presence of an ectopic nodule, extension below the carina, extension below the aortic arch, a "conical shape" of the goiter, and tracheal compression. These were compared to the same factors in the control group of 80 patients, and Fisher's exact test was used to determine statistical significance. RESULTS: The significant predictive factors for sternotomy, were posterior mediastinal extension, extension below the carina, and a "conical" goiter in which the thoracic inlet becomes a ring of constriction (all p < 0.05). CONCLUSIONS: Our results suggest that it is possible to predict on the basis of computed tomographic imaging the need for sternotomy in retrosternal goiters.

A disease activity score for ENT involvement in granulomatosis with polyangiitis (Wegener's).

OBJECTIVES/HYPOTHESIS: Accurate assessment of disease activity in patients with otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (ENT/GPA) is necessary for treatment decisions and clinical trials. We have designed a disease activity score (ENT/GPA DAS) for this purpose. STUDY DESIGN: A prospective cross-sectional study. METHODS: GPA patients seen in a tertiary center were systematically assessed for disease activity and/or infection in the ear, nose, and throat region using European Vasculitis Study Group guidelines. An ENT disease activity score was developed and validated, and compared to the ENT domain of the Birmingham Vasculitis Activity Score (ENT/BVAS). RESULTS: One hundred forty-four patients were studied, of whom 87% (125/144) had ENT involvement. ENT items of disease activity were correlated with expert clinical assessment. Discriminant correlation tests were performed to control for infection. Six items were retained to form the ENT/GPA DAS: reported bloody rhinorrhoea, granulation, ulcers and/or friable mucosa in the upper airway on endoscopic evaluation, objective stridor, sudden sensorineural hearing loss, and inflamed tympanic membrane/middle ear without infection. Nasal crusting was excluded. Individual items of ENT/GPA DAS had higher sensitivities and comparable specificities in predicting disease activity than ENT/BVAS items. Overall ENT/GPA DAS demonstrated higher sensitivity and lower specificity for disease activity in ENT/GPA when compared to overall ENT/BVAS. CONCLUSIONS: A tool to assess ENT disease activity in GPA has been developed. It is potentially superior to existing tools but requires further testing for intra- and interobserver reliability.