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Background: Granulomatosis with polyangiitis (Wegener's granulomatosis) causes progressive nasal collapse, nasal obstruction, and central face deformity. It is not known whether cartilaginous nasal reconstruction should be performed immediately or delayed until after disease "burnout.", Objectives: The aims of this research regarding nasal collapse due to Wegener's granulomatosis were to (1) assess the functional and aesthetic outcomes following immediate vs delayed nasal reconstruction; and (2) measure the impact of psychosocial well-being (anxiety, depression, social isolation) in immediate vs delayed nasal reconstruction., Methods: Wegener's patients with either immediate or delayed nasal surgery (n = 61) were compared. Functional and aesthetic severity were compared with the validated Standard Cosmesis and Health Nasal Outcome Survey (SCHNOS) score (t test). In addition, Patient-Reported Outcomes Measurement Information System (PROMIS) perioperative and 1-year follow-up surveys were analyzed., Results: At initial consultation, SCHNOS score severity types were similar for both groups (immediate vs delayed): mild 15% vs 15%; moderate 59% vs 60%, and severe 26% vs 25%. Over a 30 ± 4 month period, delayed surgery patients' conditions deteriorated, with a shift from mild to more severe SCHNOS scores, from 25% severe at initial consultation to 85% before surgery. PROMIS scores at presentation were high compared to the general public; by the time of delayed surgery, patients had significantly worsened: anxiety from 28 to 73; depression from 18 to 62; and social isolation from 20 to 80. Although both immediate and delayed groups improved after surgery in functional and psychosocial scores, the immediate surgery group's improvement was superior., Conclusions: Data showed superior functional and aesthetic scores and superior psychosocial indicators with immediate cartilaginous nasal reconstruction, compared with waiting until disease burnout to undergo surgery., (© The Author(s) 2024. Published by Oxford University Press on behalf of The Aesthetic Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Granulomatosis with polyangiitis (GPA) is a vasculitis that affects respiratory and kidney vessels. It primarily involves the nose and sinuses but can progress systemically. Granulomatosis with polyangiitis causes severe nasal deformities, impacting aesthetics and breathing. Literature focuses on rhinoplasty for saddle nose deformities in non-GPA patients, but its suitability for GPA patients remains uncertain., Aim: Our study aims to assess the effectiveness and safety of rhinoplasty in GPA patients by analyzing the existing literature., Methods: Four databases were searched; 2 reviewers independently screened the retrieved references, then relevant data for our study were extracted. Categorical outcomes were analyzed using pooled proportions and 95% CI. Statistical heterogeneity was assessed using the I2 statistics χ 2 test with a P -value lower than 0.1 indicating heterogeneity., Results: In our systematic review and meta-analysis, we incorporated 16 studies encompassing 136 patients who underwent nasal reconstruction as part of their treatment for GPA. The collective findings indicate a favorable success rate for rhinoplasty, with a pooled ratio of 89% (95% CI: 84%, 95%). The rate of revision surgeries was relatively low, with a pooled rate of 19% (95% CI: 12%, 26%). Furthermore, rhinoplasty demonstrated comparatively lower rates of recurrence (pooled rate: 22%, 95% CI: 3%, 42%) and complications (pooled rate: 12%, 95% CI: 7%, 18%)., Conclusion: Rhinoplasty benefits GPA patients with saddle nose deformity and septal perforation with high success rates and low recurrence, revision, and complication outcomes. However, more research is needed for validation and refinement., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Facial rejuvenation outcomes have not been well studied in patients with granulomatosis with polyangiitis (GP)-formerly Wegener granulomatosis. This report highlights a case of a 49-year-old female with a history of GP, presenting with facial aging and functional nasal concerns. The patient underwent facial rejuvenation and nasal reconstruction procedures, including primary open functional rhinoplasty, septal perforation repair with Alloderm, deep-plane rhytidectomy, platysmaplasty, nanofat grafting, and fractionated erbium laser to the face (sparing the nose and peri-incisional areas). Despite a routine operation, postoperative course was complicated by wound healing and vascular congestion issues related to her underlying autoimmune disease. This case highlights the risks associated with postsurgical healing in patients with GP undergoing esthetic surgery. To the best of our knowledge, this report is the first to discuss management considerations in a patient with GP undergoing facial rejuvenation surgery., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Background: Granulomatosis with polyangiitis (GPA) leads to progressive destruction of the nasal tissues resulting varying degrees of saddle deformity and nasal obstruction. Reconstructive techniques are numerous, but there are no large series reporting their results. Objective: This study sought to measure complications and outcomes after rhinoplasty for GPA. Methods: We conducted a retrospective review of 42 patients with GPA who underwent nasal reconstruction of saddle nose deformity between 2005 and 2019 using primarily costal cartilage and soft tissue grafts. Results: Thirty-six patients met the criteria for inclusion. All were followed for a minimum of 12 months. Six patients required revision surgery due to infection or GPA flare ups. Five patients had complications. All patients were given a questionnaire at 12 months to rate their degree of satisfaction with their appearance and breathing. Conclusion: The findings of this study suggest that the use of strong cartilage grafts and the timing of surgery result in improvement in breathing and appearance after rhinoplasty in patients with GPA. Clinical Trial Registration number: REB # 21-125.

A 42-year-old man had been treated for granulomatosis with polyangiitis for eight years. He was referred to our department with the diagnosis of right pneumothorax on chest radiograph. After chest drainage, the surgical treatment was performed because of continuing air leak from chest tube. Under thoracoscopic approach, the pleural adhesions were carefully dissected and the air leak site was sutured and enforced by a polyglycolic acid sheet with fibrin glue. No recurrence of pneumothorax was observed six months after surgery.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous lesions and is classified as ANCA-associated vasculitis (AAV). We herein report a case of GPA that was remitted by resection of a pulmonary lesion without immunosuppressive therapy. We detected activated neutrophils and neutrophil extracellular traps (NET) formation in resected lung tissue by immunofluorescence. Activated neutrophils and NETs might be involved in the pathophysiology of AAV and induce the vicious cycle of ANCAs and NETs. In cases of GPA with no other severe lesions, the reevaluation of the disease activity after diagnostic resection is crucial for considering the need for immunosuppressive therapy.

Objective: Subglottic stenosis (SGS) is a known complication of granulomatosis with polyangiitis (GPA). We investigated the impact of medical and surgical interventions on the surgical dilation interval and characterized patients with glottic involvement., Study Design: A retrospective chart review of patients with GPA-associated SGS was performed from 2010 to 2019., Setting: Tertiary academic medical center., Methods: The impact of medical and surgical interventions on dilation interval was assessed. The prevalence of glottic involvement was assessed, and clinical characteristics and outcomes were compared with patients without glottic involvement., Results: A total of 39 patients with GPA-associated SGS were analyzed. Dilation intervals in patients receiving leflunomide (n = 4; median, 484 days; 95% CI, 405-1099) were greater than in those not receiving leflunomide (median, 155 days; 95% CI, 48-305; P = .033). The surgical technique used did not affect dilation interval. Patients with glottic involvement (n = 13) had a greater incidence of dysphonia (13/13 vs 15/26 [58%], P = .007) and a shorter dilation interval with involvement (median, 91 days; interquartile range, 70-277) versus without involvement (median, 377 days; interquartile range, 175-1148; hazard ratio, 3.38; 95% CI, 2.26-5.05; P < .001). Of 13 patients, 8 (62%) did not have glottic involvement on first presentation., Conclusion: Although GPA is classically thought to affect the subglottis, it also involves the glottis in a subset of patients. These patients have greater complaints of dysphonia and require more frequent surgery. Systemic therapy may increase dilation intervals. In this preliminary study, patients taking leflunomide demonstrated an improvement, highlighting the need for further study of immunosuppression regimens in the treatment of GPA-associated SGS.

Objectives/hypothesis: Severe chronic rhinosinusitis (CRS) in patients with granulomatosis with polyangiitis (GPA) failing medical therapies can be treated with paranasal sinus surgery. Whether this surgery protects from progressive sinonasal damage remains unknown. Here, we aimed to analyze time-dependent relations between sinus surgeries and computed tomography (CT) imaging features in the CRS of GPA., Study Design: Longitudinal observational study., Methods: We assessed CRS features including bone thickening by global osteitis scoring scale, bone erosions, and mucosal thickening by Lund-Mackay scores in serial paranasal sinus CT scans (742 CT scans in total) from a cohort of 127 well-characterized GPA patients. Data on sinonasal surgical procedures were from a mandatory national registry and from chart review. We defined the time from baseline CT to last CT as the study observation period in each patient. Datasets were analyzed by linear mixed models., Results: We found that 23/127 cohort patients had one or more paranasal sinus surgical procedures, and 96% of these (22/23) had osteitis by CT after surgery. In patients with nasal surgery alone or no surgery, we identified osteitis in 7/11 (64%) and 45/93 (48%), respectively. During the observation period of a median of 5 years, 38 patients had progression of their sinus osteitis, with the highest annual osteitis progression rates observed around the time of surgery., Conclusions: In this cohort, paranasal sinus surgery was associated with prevalence, severity, and progression rate of sinus osteitis, indicating that sinus surgery does not reduce the bone damage development in the CRS of GPA., Level of Evidence: 4 Laryngoscope, 130: E460-E468, 2020., (© 2020 The Authors. The Laryngoscope published by Wiley Periodicals, Inc. on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Background: In this study we aim to describe presenting characteristics and identify prognostic factors for disease resolution in patients with chronic rhinosinusitis (CRS) in the setting of eosinophilic granulomatosis with polyangiitis (EGPA)., Methods: Patients evaluated at a tertiary care center with diagnoses of EGPA and CRS were identified. Descriptive statistics were obtained. Univariate analysis was used to search for prognostic factors associated with higher Lund-Mackay score at presentation and disease resolution., Results: Forty-four patients were included with a mean age of 52.7 (standard deviation, 14) years. Twenty-one patients (47.7%) were female, all had a diagnosis of asthma, and 36 (83.7%) had eosinophils >10%. Common presenting symptoms for CRS included nasal discharge (87.9%) followed by nasal congestion (83.9%) and facial pain and pressure (83.8%). Medical management of CRS included systemic corticosteroids (93.2%) and systemic antibiotics (75%). Surgical intervention occurred in 29 patients (67%). Nine patients (20.5%) had resolution of sinus symptoms, including 4 with imaging confirmation. Fourteen patients (31.8%) had continued CRS, but with improved symptoms, whereas 9 patients (20.5%) had continued CRS with no improvement in symptoms. Eleven patients (25%) were lost to follow-up and 4 (9.1%) died. Univariate analysis did not show antineutrophil cytoplasmic antibody positivity, presence of peripheral eosinophilia, gender, age, or absence of systemic therapy to be predictive of higher Lund-Mackay score at presentation or predictive of disease resolution., Conclusion: CRS in patients with EGPA is often refractory to medical and surgical management. Treatment of these patients should occur in a multidisciplinary setting., (© 2019 ARS-AAOA, LLC.)

Background: We report new data for a rare face transplant performed 3 years ago. Granulomatosis with polyangiitis (GPA) (Wegener) is a severe autoimmune necrotizing vasculitis and parenchymal inflammatory disease that can affect any organ including those of the craniofacial region. Skin involvement manifests as malignant pyoderma. This account (1) highlights the technical details of face transplantation for this unique indication, (2) reports the 3-year posttransplant outcome, and (3) describes relevant immunological aspects., Methods: A Le Fort III near-total face and near-total scalp transplant was performed after extensive trauma and subsequent bone and soft tissue infection in a patient with GPA. Incisions were planned along facial aesthetic subunits. The vascular pedicle comprised the facial and superficial temporal arteries bilaterally. The functioning left eye was preserved and fitted into the donor tissues., Results: The procedure took 21 hours, and transfusion was limited to 4 units of packed red cells. Early medical and surgical complications were successfully treated. At 3 years, acceptable aesthetic outcome was achieved with adequate color match and scalp hair growth. The patient has recovered light touch, temperature, and 2-point discrimination and has evidence of symmetric cheek elevation albeit with limited eyelid and frontalis function. GPA relapse did not occur. Four acute rejections were fully reversed., Conclusions: This case represents a new underlying disease (trauma + GPA) leading to face transplantation and a unique clinical scenario where allografting was indicated for potentially life-threatening and sight-preserving reasons and not for mere functional and aesthetic concerns. Despite complexity, 3-year clinical outcome is encouraging, and the patient is no longer at risk for dural exposure, meningitis, and related morbidity.

Wegener's granulomatosis - or, in other words, granulomatosis with polyangiitis - is an anti-neutrophil cytoplasmic antibody associated granuloma forming vasculitis, mainly affecting the respiratory tract and the renal system. Otologic manifestations of Wegener's disease can be otitis media with effusion or chronic silent mastoiditis with conductive hearing loss, but sensorineural hearing loss can also evolve. The diagnosis is based on the clinical appearance as well as the immunoserological and histopathological results. It is of paramount importance to begin a combined immunosuppressive treatment immediately, besides eradicating the otologic manifestations. The intractable cases of chronic otitis media due to Wegener's granulomatosis are challenging any ear surgeons. Subtotal petrosectomy has proved to be an effective solution in such cases to create a dry ear and to provide a safe surgical field for hearing restoration. The authors reviewed the literature and report a case history to present the modern management of Wegener's granulomatosis with otologic manifestation. Orv Hetil. 2019; 160(4): 151-157.

Wegener granulomatosis (WG) is a necrotizing vasculitis that involves the upper respiratory tract, the lungs, and the kidneys. It also causes deformities of the nose in practically all patients, altering the cartilaginous and bony structure as well as the inner lining of the nose.The surgical repair of these nasal distortions is a challenge mainly due to the impairment of wound healing and graft take that is caused both by the disease itself and by the prolonged immunosuppressant treatment.Many different reconstruction plans have been developed, using costal or calvarial bone grafts, cartilage grafts and local skin and mucosa flaps. Results have been diverse.The authors present the case of a 56-year-old patient who suffered from WG and consulted for nasal sequelae. She initially underwent several failed attempts at reconstruction; finally, the authors performed a nasal repair using a double strip of fascia lata as a dorsal graft, with no complications and a good aesthetic result.Fascia lata graft is a new and good option for nasal reconstruction in patients with WG because it is easily obtainable, it can be set in place through closed rhinoplasty and it can achieve good vascularization with low risk of resorption or disease reactivation.

Background: Hypertrophic pachymeningitis (HP) is a rare primary or secondary inflammatory disorder that manifests with thickening of dura mater involving predominantly the tentorium and falx in the focal form. Hydrocephalus has not been reported in association with secondary HP., Case Description: A 61-year-old woman presented with intracranial HP of the posterior fossa secondary to Wegener granulomatosis not responsive to corticosteroid therapy. Owing to the association of noncommunicating hydrocephalus, endoscopic third ventriculostomy was performed. There was immediate improvement of neurologic symptoms and no postoperative complications. The patient had very good clinical and radiologic outcome at 1-year follow-up., Conclusions: In a case of noncommunicating hydrocephalus related to focal HP of the posterior fossa, endoscopic third ventriculostomy was effective in resolving symptoms related to increased intracranial pressure. The physiopathogenetic mechanisms and therapeutic strategies were discussed along with a review of the most relevant literature., (Copyright © 2018 Elsevier Inc. All rights reserved.)

A 58-year-old man visited the emergency department with vague anterior chest discomfort. From the computed tomographic findings, our initial suspicion was intramural hematoma in the ascending aorta. After a comprehensive discussion with relevant departments, emergent operation was planned. Intrapericardial space, however, was severely adhered, suggesting chronic inflammation. Biopsies were performed, and additional surgical procedures were discontinued. The patient was finally diagnosed as having granulomatosis with polyangiitis. Careful approach with high-degreed suspicion for granulomatosis with polyangiitis, although rare, is required when preoperative findings are similar to the present case., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: To investigate the risk factors and treatment strategies for pneumothorax secondary to granulomatosis with polyangiitis (GPA)., Method: Retrospective analysis of cases with pneumothorax secondary to GPA from our own practice and published on literature., Results: A total of 25 patients, 18 males and 7 females, mean age 44 ± 15.7 years, were analyzed. Diagnosis included pneumothorax (11 cases), hydropneumothorax (n = 5), empyema (n = 8) and hemopneumothorax (n = 1). 88% (22/25) patients showed single/multiple pulmonary/ subpleural nodules with/without cavitation on chest imaging. Erythrocyte sedimentation rate and C-reactive protein were both elevated. Corticosteroids and immunosuppressive agents were used in 16 cases. Five cases received steroid pulse therapy, of which 4 patients survived. Pleural drainage was effective in some patients. Seven patients underwent surgical operations. In the 10 fatal cases, infection and respiratory failure were the most common cause. Lung biopsy/ autopsy showed lung/pleural necrotizing granulomatous vasculitis, breaking into the chest cavity, pleural fibrosis, bronchial pleural fistula, etc. The mean age in the death group was greater than the survival group (53 ± 12.9 years vs 40.1 ± 14.7 years, p = 0.05), the ineffective pleural drainage was also higher in the death group (5/5 vs 0/7, p = 0.01)., Conclusions: Pneumothorax was seen in the active GPA, due to a variety of reasons, and gave rise to high fatality rate. Aggressive treatment of GPA can improve the prognosis. Older and lack of response for pleural drainage indicates poor prognosis.

Inflammatory lesions of the breast encompass primary reactive processes and local manifestation of systemic diseases. They are very rare and they are generally treated without resort to biopsy. Nevertheless they could be clinically challenge mimicking malignant process and needing surgery to reach a correct diagnosis. Here we describe a rare case of breast granulomatosis with polyangiitis, which presented with radiological and clinical alarming features that immediately raised the suspicious of malignancy leading to breast-conserving surgery., (© Copyright Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.)

Clinical Case: A 43 year-old woman consulted due to 2 months of swelling on the superolateral side of the left orbit, with pain and erythema. An excisional biopsy was performed that revealed vasculitis with polyangiitis of the lacrimal gland. A systemic study showed that no other system was compromised., Discussion: Orbital involvement occurs in up to 60% of patients with granulomatosis with polyangiitis. The involvement of the lacrimal gland is rare and often unilateral. Serological tests are generally negative, both in initial stages, as in localized forms of the disease., (Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

A 35-year-old woman who had previously undergone a lung transplantation presented with severe abdominal pain and vomiting. The gastroscopy showed diffuse ulcerative gastric lesions. Tests for varicella zoster virus and Epstein-Barr virus via polymerase chain reactions (PCR) on endoscopically obtained gastric biopsies were found to be positive and confirmed varicella gastritis. Intravenous antiviral therapy with acyclovir was administered resulting in a normalization of all clinical symptoms, especially of abdominal pain and inflammation parameters.

Introduction: Wegener granulomatosis (WG) is a rare idiopathic autoimmune disease causing necrotizing granulomatous vasculitis. Whether as the first symptom or as part of systemic changes, ocular manifestations in WG patients are not specific. Any part of the eyes can be affected, with the anterior segment and orbit most commonly involved. So, early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients., Clinical Findings/patient Concerns: Here we present a rare case of orbital WG of a 22-year-old woman was admitted to the hospital because of intense pain associated with decreased visual acuity in her right eye since 1 day. She had been previously diagnosed with WG at our hospital., Imaging Diagnosis: Orbital computed tomography imaging showed diffuse swelling of intraorbital muscles, and space-occupying lesions were present in both eyes. Most postnasal anatomical structures were absent, appearing as a massive cavity shadow. Orbital magnetic resonance imaging showed a shadow of orbital soft tissues., Conclusion: WG is a serious, fatal disease. Early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients.

Background: The transsphenoidal approach is considered the gold standard for resection of pituitary adenomas and other sellar region lesions. This approach is guided by a few fundamental anatomic landmarks that conduct the surgeon toward the sellar floor. Some anatomic structures may vary a lot (e.g., intrasphenoidal septa, intercarotid distance) and may be difficult to identify. Pneumatization and conformation of the sphenoidal sinus (SS) plays a key role in accessing the floor of the sella and other skull base structures. A poorly pneumatized SS may be a relative contraindication to the transsphenoidal approach. We analyzed outcome and complications in transsphenoidal surgery for sellar lesions with a difficult SS., Methods: We analyzed 243 consecutive patients who underwent a transsphenoidal approach for sellar lesions. Patients with poor pneumatization of the SS were included. Neurosurgical and endocrinologic outcomes were reported., Results: Successful treatment using a transsphenoidal approach with neuronavigation and Doppler ultrasound was achieved in 15 patients with a low degree of pneumatization of the SS. A pituitary adenoma was present in 13 of 15 patients. Endocrinologic and neurosurgical outcomes were similar to patients with normal pneumatization of the SS, showing a cure of disease in 6 of 9 patients with functioning adenomas and an improvement of symptoms in cases of nonfunctioning adenomas., Conclusions: Patients with a poorly pneumatized SS can be treated safely with a transsphenoidal approach using image guidance techniques to avoid major neurovascular complications., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Cutaneous metaplastic synovial cysts (CMSCs) are rare tumors typically comprising a solitary, well-circumscribed cystic mass that is not connected to the joint. Synovial cysts have been reported predominantly by orthopedists or pathologists; however, the presence of CMSC is not generally well recognized by dermatologists. Herein, we report a CMSC in a 68-year-old woman receiving systemic corticosteroid therapy for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA). We attempt to delineate the clinical characteristics of this unusual neoplasm by reviewing the literature, focusing especially on dermatological descriptions. Histologic examination of the surgical specimen in the current case revealed that the cystic wall was lined with layers of flattened synovial cell-like cells and connective tissues, mimicking the synovial membrane. Positive immunoreactivity of the lining cells against vimentin was detected, but no immunoreactivity against cytokeratin, carcinoembryonic antigen (CEA), CD68, or S-100 was detected. The pathogenesis of CMSC remains unclear, but it has been tightly linked to direct traumatic stimuli or relative tissue fragility, which potentially accounts for CMSC development in our case. Most CMSCs reported by dermatologists are located on the extremities, whereas those described by other specialists tend to be distributed more globally. Preoperative diagnoses are often either epidermal cyst or suture/foreign body granuloma. Incomplete surgical excision of usual synovial cysts may lead to local recurrence, which has been reported in oral and maxillofacial surgery, but not in dermatologic surgery. This fact could be explained by the technical difficulties of surgical excision related to anatomical location. Dermatologists need to be aware of CMSC, and CMSC should be included in the differential diagnosis of subcutaneous cysts.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis in patients with bronchial asthma and eosinophilic rhinosinusitis. Serum IgG4 levels are markedly elevated in patients with active EGPA, a disease which has been closely associated with IgG4-related disease (IgG4RD). A 68-year-old male with a history of asthma and eosinophilic rhinosinusitis developed vasculitis and orbital symptoms. The results of a laboratory examination showed eosinophilia (4,067/μl; 39%), while image evaluations revealed hypertrophy of the rectus muscles, trigeminal nerve, lachrymal gland, and bilateral submandibular glands. Biopsy of the paranasal sinus showed the prominent infiltration of eosinophils and IgG4-positive plasma cells. The patient was diagnosed with EGPA concomitant with IgG4RD and treated with systemic steroids. Although concomitant cases of EGPA with IgG4RD are extremely rare, clinical manifestations associated with both diseases are sometimes mixed. Therefore, systemic scrutiny may be required for cases of EGPA with high serum IgG4 levels and pathognomonic symptoms or findings of IgG4RD

Granulomatosis with polyangiitis (Wegener disease) is a rare antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis that seldom involves brain and meninges. We present a case of a 35-year-old woman with granulomatosis with polyangiitis involving the brain and the meninges. The histologic diagnosis required a complete resection of the lesion. Despite the possibility of postoperative wound infection, surgery was mandatory to ascertain the diagnostic and to treat locally., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Gynceological involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides is rare. We describe a case of ANCA-associated vasculitis in an elderly woman presenting with fevers, cough and a rash. Further investigations revealed vasculitic involvement of the ovaries, fallopian tubes, uterus and omentum with an eosinophil-rich infiltrate., (© 2016 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Case Report: The case is presented of a 49 year-old woman with an orbital mass originating from the rhinosinus. She had a history of Wegener's granulomatosis, refractory to both biological and immunosuppressive therapy. Clinical examination showed proptosis, diplopia, and restriction of ocular movements., Discussion: Orbital mass resection was performed, due to its rapid growth, and lack of response to medical treatment., (Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Granulomatosis with polyangiitis (Wegener granulomatosis) is a rare disease that commonly starts in the craniofacial region and can lead to considerable facial disfigurement. Granulomas and vasculitis, however, can involve many other tissues (especially pulmonary and renal). Dermatologic and subcutaneous components can lead to malignant pyoderma., Methods: The authors describe a unique pathologic condition, where significant Le Fort type trauma was associated with subsequent development of granulomatosis with polyangiitis and malignant pyoderma. Successive operations to excise necrotic tissue and reconstruct the defects were followed by worsening inflammation and tissue erosions. Trauma and surgery in proximity to the eye and sinuses masked the initial clinical presentation and led to delay in diagnosis and disease progression. The resultant facial disfigurement and tissue loss were substantial., Results: Despite multiple confounding factors, accurate diagnosis was eventually established. This was based on persistence of sinus inflammations in the absence of infective agents, proven sterility of lung lesions, and antineutrophil cytoplasmic antibody positivity with proteinase 3 specificity. Skin lesion biopsy specimens were identified as pyoderma gangrenosum and later as malignant pyoderma. Institution of immunosuppressive therapy allowed successful control of the disease and wound healing. The resulting craniofacial destruction, however, necessitated facial vascularized composite allotransplantation., Conclusion: Recognition of this rare pathologic association is essential, to prevent delays in diagnosis and treatment that can lead to major craniofacial tissue loss., Clinical Question/level of Evidence: Therapeutic, V.

Background: Wegener's granulomatosis is a systemic vasculitis of the small- and medium-sized vessels, produced by the action of ANCA, which involves the respiratory tract, kidneys, and eyes, with a potential for lethal evolution in the first year after diagnosis. Its association with chronic lymphocytic leukemia is rarely described in the literature, and it may be difficult to diagnose and to treat this association., Case Presentation: We present the case of a 73-year-old Caucasian patient, a rare case in which Wegener's granulomatosis is associated in a patient with chronic lymphocytic leukemia, who is admitted in the Infectious Disease Department for fever, diplopia, headache, purulent and hemorrhagic nasal secretions, intense asthenia, and weight loss. The patient had associated eyelid edema; scleritis; chemosis; subconjunctival hemorrhage at the left eye; swelling of the left region of the eyehole, of the base of the nasal pyramid, and of the left zygomatic region; anterior nasal bleeding; pustulous non-itching lesions at the cervical region and posterior thorax; enlarged bilateral axillary lymph nodes; hepatomegaly; and moderate splenomegaly. During the surgical treatment of the pansinusitis, a biopsy from the tissue is taken; the biopsy fragments of the nasal mucosa pleads for Wegener's granulomatosis. The c-ANCA were positive. The patient's evolution was favorable under treatment with meropenem, teicoplanin, fluconazole, transfusions of platelet concentrates, and methylprednisolone., Conclusions: The real dimension of the association between chronic lymphocytic leukemia and Wegener's granulomatosis is not known; it may be useful to evaluate the vasculitis by testing ANCA routinely in patients with chronic lymphocytic leukemia and by histopathological examinations of the lesions.

Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a multiorgan sclerotic disorder that is strongly linked to the administration of gadolinium-based chelates used for intravenous contrast in magnetic resonance imaging procedures. Although involvement of truncal skin is a relatively common, few reports of its appearance on mammography exist in the literature., (Copyright © 2015 Mosby, Inc. All rights reserved.)

Preemptive mapping of the airway is a useful adjunct to conventional clinical assessments when airway management planning for patients with complex head and neck pathology. Nasendoscopy is frequently used for this purpose but is also invasive and poorly tolerated in a subset of patients and, even in expert hands, may not allow complete visualization of the glottis and subglottic structures. We present a novel tool for difficult airway management planning in the form of virtual endoscopy by applying free online OsiriX software to head and neck computed tomographic scans to generate a "fly-through" airway reconstruction. To highlight how well virtual endoscopy correlates to conventional fibreoscopy, we compare both of these assessment tools in 3 patients with glottic, subglottic, and multilevel airway pathologies. Virtual endoscopy represents a unique, noninvasive, safe, and accurate airway assessment and educational resource, which warrants further exploration., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Midline Destructive Lesions (MDL) are well known to cause nasal problems. There is a long differential diagnosis of such lesions. However, in the pediatric population, the 2 main diseases to be aware of are Non-Hodgkin's T-cell lymphoma and granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). The authors present the report of a 15-year-old boy who presented with epiphora, chemosis, and limitation of left abduction. CT scan of his orbits suggested a destructive lesion of the ethmoid sinuses. His laboratory investigations revealed a positive ANCA. The patient underwent endoscopic sinus surgery, and this was characteristic for granulomatosis with polyangiitis. He was treated with systemic steroids and then maintained on cyclophosphamide, which controlled his disease activity. This case highlights the need for ophthalmologists to have a high index of suspicion for MDL and concomitant orbital disease.

Background: IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease, is characterized by elevated serum IgG4 and infiltration of IgG4-positive plasma cells in glandular tissues. Recently, several studies reported both malignant lymphoma developed on the background of IgG4-associated conditions and IgG4-producing malignant lymphoma (non-IgG4-related disease)., Case Presentation: We report on the case of a 70-year-old man who was strongly suspected IgG4-DS because of high serum IgG4 concentration (215 mg/dl) and bilateral swelling of parotid and submandibular glands. Biopsies of cervical lymph node and a portion of submandibular gland were performed. These histopathological findings subsequently confirmed a diagnosis of marginal zone B cell lymphoma., Conclusion: Differential diagnosis of IgG4-DS is necessary from other disorders, including Sjögren's syndrome, sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, and cancer. We suggest that biopsy of swollen lesions is important for a definitive diagnosis of IgG4-DS and discuss the mechanism of development in this case.

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis, associated with antineutrophil cytoplasmic autoantibody-associated systemic vasculitis, and it can affect many organ systems via the inflammation of small-to-medium-sized vessels. Cardiac involvements in GPA are relatively rare. We report a 75-year-old woman who was diagnosed with GPA and rapid progressive glomerulonephritis that resulted in a partial posteromedial papillary muscle rupture, but with no coronary angiographic findings. The surgical and pathological findings with regard to the ruptured papillary muscle revealed necrotic muscle and acute ischemic change. The mechanism of papillary muscle rupture in GPA is coronary vasculitis leading to myocardial infarction. The ischemic change is not always detected on coronary angiography, so assessment using an echocardiogram is important.

Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination revealed subglottic infiltrations and vocal fold oedema which required urgent tracheotomy. During few days she developed gingival ulcerations and pulmonary infiltration with negative serum c-ANCA titers. The histopathological examination of subglottic and gingival biopsies and the clinical picture established the diagnosis of GPA. She was treated with prednisone and cyclophosphamide with recovery; however, during over 3 years of follow-up, pulmonary symptoms relapsed and subglottic stenosis persisted. The difficulties in diagnosis and treatment in this unusual presentation of GPA are outlined with conclusion that in patients with subglottic infiltration, which develops rapidly, even when this is a sole presentation of the disease, and when c-ANCA are negative, GPA should always be considered.

Endobronchial involvement is a relatively uncommon but well-described presentation in Granulomatosis with polyangiitis (GPA). Self-expandable metallic stents (SEMs) should be reserved for the malignant airway disorder to maintain airway patency, but have been used for benign disease in specific cases. We present a case of longstanding endobronchial GPA with recurrent bronchial stenosis. Three SEMs were deployed in the distal left main bronchus 10 years prior. Two were removed in the standard manner, but the remaining stent SEM was completely embedded in the bronchial mucosa making its removal extraordinarily difficult. We placed an oversized silicone stent inside the stent leading to necrosis of the mucosa allowing for a less formidable removal of the embedded stent. Another silicone stent was temporarily placed. SEMs removal can be extremely complicated and should only be performed by experienced bronchoscopists in an institution with sufficient resources.

Introduction: ANCA vasculitis may involve the skin and develop slowly without specific histology, and without autoantibodies., Case Report: We report a 50-year-old woman who experienced bilateral mastectomy because of ulcero-necrotic, non-specific inflammatory cutaneous lesions of the breasts. First considered by others as a malinger patient, she developed oto-neurological lesions leading to the diagnosis of Wegener's granulomatosis. Five years later, specific antibodies of the disease were present., Conclusion: Cutaneous involvement by ANCA vasculitis can be isolated for a long time. Physicians must have a high degree of suspicion to avoid diagnostic delay of ANCA vasculitis., (Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Aim of the Work: The aim of the present work was to study the role of vascular surgery in the management of primary vasculitis patients with peripheral ischemic manifestations., Patients and Methods: Ten primary vasculitis patients with peripheral ischemic manifestations were studied and reviewed for the diagnosis, clinical manifestations, investigations, treatment options and role of vascular surgery. The Birmingham Vasculitis Activity Score (BVAS) was recorded., Results: Giant cell arteritis was present in one patient; granulomatosis with polyangiitis in 5, essential cryoglobulinemic vasculitis in 3 and 1 (child) had Henoch-Schönlein purpura. They showed the following peripheral vascular manifestations: intermittent claudications, Raynauds, deep venous thrombosis and thrombophelebitis in 10% each; digital ulceration and trophic changes in 20% while acrocyanosis and dry gangrene were present in 30%. Renal involvement was present in 60% of patients. The mean BVAS was 11.5 ± 6.57 at initial presentation. The disease activity remarkably improved over the disease course in all patients to be at their last visit (2.6 ± 2.22) (p=0.002). Regarding the vascular surgery role in their management, in addition to their medical treatment, 40% required an additional surgical intervention. Two had a minor amputation of the toes; one performed thoracoscopic cervical sympathectomy and another needed tibial angioplasty., Conclusion: Primary vasculitis patients presenting with peripheral ischemic manifestations require surgical attention. Their management is essentially medical and individualized to the diagnosis and presenting symptoms. Endovascular treatment may offer a safe and less invasive approach in high surgical risk patients. Sympathectomy is of high therapeutic potential in those with severe pain and trophic changes.

The paper describes a case of Wegener's granulomatosis with upper airway and lung involvements in a 14-year-old patient. Cavernous pulmonary tuberculosis was presumptively diagnosed during planned examination. The diagnosis of Wegener's granulomatosis was established by examining the materials obtained during marginal resection of the lung. The clinical and laboratory indices and histological findings of the biopsy specimens taken during open lung biopsy were considered.

Objective: Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is an autoimmune disease characterized by inflammation of blood vessels most often seen in the upper respiratory tract, lungs, kidneys, and skin. Central nervous system (CNS) involvement of GPA is rare, particularly in the pituitary, and can be difficult to treat., Methods: Case report., Results: We present a 30-year-old woman with pituitary and ocular GPA, whose unusually recalcitrant disease led to the development of pan-hypopituitarism and near-total vision loss. After failing multiple systemic immunosuppressants, she was ultimately treated with the novel immunomodulatory agent rituximab together with pulse corticosteroids, which achieved a gratifying response., Conclusion: Pituitary and optic chiasm involvement is a rare complication of GPA. We believe this case illustrates the complexity of management of pituitary GPA and provides insight into the potential utility of the biologic agent rituximab in this disease.