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Patients with granulomatosis with polyangiitis occasionally present with cutaneous manifestations, which are important clues for the early diagnosis. Although pyoderma gangrenosum-like ulcers are rarely observed, a unique case with unusual clinical features is presented herein. A 75-year-old woman with positive proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) repeatedly developed aseptic abscesses on the abdomen, buttock, lower legs, and forearms. Histopathological features of biopsy taken from ulcers showed necrotizing granulomatous inflammation with multinucleated giant cells without leukocytoclastic vasculitis. The present case was initially diagnosed as limited granulomatosis with polyangiitis without renal and lung involvement. However, two years later, she died of cerebral hemorrhage.

Patients with granulomatosis with polyangiitis occasionally present with cutaneous manifestations, which are important clues for the early diagnosis. Although pyoderma gangrenosum-like ulcers are rarely observed, a unique case with unusual clinical features is presented herein. A 75-year-old woman with positive proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) repeatedly developed aseptic abscesses on the abdomen, buttock, lower legs, and forearms. Histopathological features of biopsy taken from ulcers showed necrotizing granulomatous inflammation with multinucleated giant cells without leukocytoclastic vasculitis. The present case was initially diagnosed as limited granulomatosis with polyangiitis without renal and lung involvement. However, two years later, she died of cerebral hemorrhage. [ABSTRACT FROM AUTHOR]

Background: Granulomatosis with polyangiitis (GPA) is characterised by granulomatous inflammation and small-to-medium vessel necrotising vasculitis, mainly affecting respiratory tract and kidneys. Renal involvement presenting as tumour-like lesions poses diagnostic and treatment challenges. Methods: Following the observation of a GPA patient presenting with multiple renal tumour-like lesions, we conducted a systematic literature review on MEDLINE/PubMed, EMBASE, and Cochrane databases. Data gathered from the literature were analysed to summarise the diagnostic approach, management, and outcome of renal GPA-related tumour-like lesions. Results: a 49-year-old female presented with persistent constitutional symptoms and multiple bilateral renal lesions. Renal biopsy showed chronic interstitial inflammation with necrotising granulomas. Laboratory tests disclosed positive anti-proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) leading to a final diagnosis of GPA. She was effectively treated with high-dose glucocorticoids and rituximab. Literature search yielded 41 articles, concerning 42 GPA patients with renal masses, presenting bilaterally in 23.8% of the cases. Positive PR3-ANCA was observed in 86.5% of the cases. Half of 42 patients showed kidney abnormalities. Treatment with glucocorticoids (83.3%) and immunosuppressive agents (80.9%) resulted in an overall good remission rate and favourable prognosis. Conclusions: GPA should be considered in the differential diagnoses of kidney tumour-like lesions. The diagnosis is challenging, and histological examination greatly contributes to the diagnostic work-up. [ABSTRACT FROM AUTHOR]

Introduction Wegener granulomatosis (WG) appears with clinical symptoms, including recurrent respiratory infection, renal manifestations, and nonspecific systemic symptoms. Objective To study the clinical manifestations of WG in Iranian ethnicities, and data on 164 patients were recorded from 2013 to 2018. Methods The data included demographics, symptoms, and the Birmingham Vasculitis Activity Score (BVAS). The symptoms involved the following sites: the nose, sinus, glottis, ears, lungs, kidneys, eyes, central nervous system, mucous membranes, skin, heart, stomach, intestine, as well as general symptoms. The clinical manifestations of nine ethnicities were analyzed. Results In total, 48% of the patients were male and 51% were female, with a median age of 51 years. The BVAS was of 15.4, the sites most involved were the sinus (n = 155), nose (n = 126), lungs (n = 125), and ears (n = 107). Gastrointestinal (n = 14) and cardiac (n = 7) involvement were less common. Among the patients, 48.17% were Persian, 13.41% were Azari, 11.17% were Gilaki, 11.17% were Kurd, and 10.9% were Lor. Conclusion Our findings indicated that the sinus, nose, lungs, and ears were the sites most involved, and gastrointestinal and cardiac involvement were less common. In the present study, involvement of the upper and lower respiratory tract was higher than that reported in Western and Asian case series. Moreover, we report for the first time that, in all patients with ear involvement, the left ear was the first to be affected. The clinical manifestations among Iranian ethnicities were not different, and the Gilaki ethnicity had the highest BVAS, mostly because the weather was humid; therefore, in Iran, in areas with humidity, the rate of the disease was higher. [ABSTRACT FROM AUTHOR]

Introduction Wegener granulomatosis (WG) appears with clinical symptoms, including recurrent respiratory infection, renal manifestations, and nonspecific systemic symptoms.

Background: Granulomatosis with polyangiitis (GPA) is characterised by granulomatous inflammation and small-to-medium vessel necrotising vasculitis, mainly affecting respiratory tract and kidneys. Renal involvement presenting as tumour-like lesions poses diagnostic and treatment challenges. Methods: Following the observation of a GPA patient presenting with multiple renal tumour-like lesions, we conducted a systematic literature review on MEDLINE/PubMed, EMBASE, and Cochrane databases. Data gathered from the literature were analysed to summarise the diagnostic approach, management, and outcome of renal GPA-related tumour-like lesions. Results: a 49-year-old female presented with persistent constitutional symptoms and multiple bilateral renal lesions. Renal biopsy showed chronic interstitial inflammation with necrotising granulomas. Laboratory tests disclosed positive anti-proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) leading to a final diagnosis of GPA. She was effectively treated with high-dose glucocorticoids and rituximab. Literature search yielded 41 articles, concerning 42 GPA patients with renal masses, presenting bilaterally in 23.8% of the cases. Positive PR3-ANCA was observed in 86.5% of the cases. Half of 42 patients showed kidney abnormalities. Treatment with glucocorticoids (83.3%) and immunosuppressive agents (80.9%) resulted in an overall good remission rate and favourable prognosis. Conclusions: GPA should be considered in the differential diagnoses of kidney tumour-like lesions. The diagnosis is challenging, and histological examination greatly contributes to the diagnostic work-up.

Wegener granulomatosis (WG) now known as granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disorder of undivulged etiology affecting the respiratory tract including paranasal sinuses, nasal cavity, lungs, and kidneys predominantly. GPA presenting as a solitary renal mass is rarely seen. We present a case report of a 27-year-old female presenting with a right renal mass along with pain, low-grade fever, and arthralgia. Computed tomography scan of the abdomen revealed a hypodense low attenuated renal mass with indistinct margins. Ultrasound-guided biopsy revealed features typical of GPA. She was started on oral steroids (prednisolone 40 mg) and azathioprine. She developed pain, vomiting, and diarrhea after starting treatment with azathioprine. Azathioprine was stopped and rituximab 1 g weekly was started for 4 weeks followed by 500 mg 6 monthly injections. She got symptomatic relief at 4 weeks with a diminution of renal mass at 6 months follow-up. We report this rare entity of WG presenting as renal mass. Suspecting and diagnosing renal mass as a part of GPA prevented us from undertaking unnecessary surgical treatment in this patient. Medical treatment with steroids and rituximab is effective in inducing remission and maintenance.

Granulomatosis with polyangiitis (GPA) is one of three described anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Early diagnosis and treatment of GPA is paramount, as it may help prevent irreversible end-organ damage, especially renal and pulmonary failure. A 72-year-old male with a past medical history of lung adenocarcinoma in remission, chronic sinusitis status-post multiple sinus surgeries, and coronary artery disease presented with shortness of breath, dark urine, and asymmetric polyarthralgias. He had an acute kidney injury, leukocytosis, with urinalysis demonstrating pyuria and hematuria, without casts. Chest imaging showed cavitary nodular opacities in addition to interval increase of existing nodules compared to the most recent scan one month prior. His acute kidney injury progressed to renal failure requiring hemodialysis, and he developed an inflammatory polyarthritis. GPA was suspected clinically so he was started on high-dose intravenous corticosteroids, and subsequently plasmapheresis and rituximab. Serology returned with highly positive proteinase-3 antibodies, and cytoplasmic ANCA positivity on immunofluorescence. Renal biopsy demonstrated severely active pauci-immune glomerulonephritis. Several months after discharge, the patient passed away from gram positive bacteremia. This patient's recurrent sinusitis, pulmonary nodules, and subsequent renal failure were highly suggestive of GPA. A biopsy is recommended to confirm the diagnosis of GPA, but treatment should not be delayed if there is a high index of suspicion for the disease. Induction therapy with corticosteroids combined with rituximab or cyclophosphamide has significantly decreased the mortality of patients with GPA. Patients with GPA often have preceding history of nasopharyngeal and upper airway disease, and can present with fluctuating pulmonary infiltrates. Early recognition and treatment of patients with GPA can prevent life-threatening complications and reduce mortality.

Abstract Tuberculosis is the most common diagnosis in India in presence of constitutional symptoms such as cough, fever, and weight loss with lung parenchymal abnormality irrespective of microscopy or nucleic acid amplification test abnormalities in high TB prevalent tropical settings. Pulmonary manifestations of systemic vasculitis have very diverse involvement ranging from the nodule to consolidation. Bronchus sign is classically described in lung malignancies than Wegener disease. The acino-nodular pattern is classical of pulmonary tuberculosis, sometimes documented in fungal infections. In this case report, a 45-year-old female, presented with constitutional symptoms with lung parenchymal nodules, without mycobacterial microscopic or genome documentation, received empirical antituberculosis treatment with the progression of the disease without clinical or radiological response. Bronchoscopy workup is inconclusive and tropical screen for bacterial, fungal, TB, and malignancy was negative. Vasculitis workup was done in view of the presence of persistent fever and documented PR3-ANCA positive with very highly raised titers. We have started on steroid and cyclophosphamide and clinical response documented with near-complete resolution of shadows in 12 weeks. Pulmonary manifestations of Wegener disease are rare and underestimated and early pickup of the entity in course of illness will have a good outcome with an excellent prognosis.

A 61‐year‐old male patient with Wegener's granulomatosis was admitted due to neck pain and quadriparesis. Clinical evaluation showed severe cervical noninfectious spondylodiscitis, myelopathy, sagittal imbalance, and atlantoaxial instability. A combined anterior and posterior approach was implemented. Postoperative clinical evaluation showed improved neurologic status. [ABSTRACT FROM AUTHOR]

Introduction. Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is characterized by necrotizing granulomatous inflammation in various tissues, including blood vessels, but primarily in the respiratory tract and kidneys. Clinical manifestations can be diverse, including inflammation of the eye and adnexa. Optic neuritis is a very rare ophthalmological manifestation of GPA, not previously described in a teenager. Case report. We presented a case of a 16-year-old girl with a rare extrapulmonary manifestation of GPA. The girl had a previous history of GPA and complained of a sudden blurred vision in the left eye. She was promptly referred to an ophthalmologist who noted a decreased visual acuity of 20/400 in the left eye. Colour vision was impaired in the spectrum of red colour. Clinical examination revealed normal anterior segment findings. On ophthalmoscopy, the left optic nerve oedema was noted. Urgent computed tomography of the left orbit showed a soft tissue mass around the optic nerve in the apex of the orbit. Magnetic resonance imaging confirmed the diagnosis of optic perineuritis. After pulse doses of methylprednisolone, the girl achieved complete resolution of vision in the left eye. Conclusion. If untreated, inflammation of the optic nerve can lead to a permanent loss of vision. Prompt diagnostic and adequate treatment of patients with GPA is needed in order to prevent vision-threatening complications and control the systemic disease.

Abstract A 61‐year‐old male patient with Wegener's granulomatosis was admitted due to neck pain and quadriparesis. Clinical evaluation showed severe cervical noninfectious spondylodiscitis, myelopathy, sagittal imbalance, and atlantoaxial instability. A combined anterior and posterior approach was implemented. Postoperative clinical evaluation showed improved neurologic status.

In an adult female who has been using asthma treatment for years but has not benefited from the treatment, flattening was noted in the inspiratory and expiratory curves of spirometric evaluation. Subsequent examination revealed the presence of subglottic stenosis as the underlying pathology of fixed upper airway obstruction.

Granulomatosis with polyangiitis (GPA) is a rare cause of prostatitis. Our case illustrates a case of granulomatous prostatitis secondary to unrecognised GPA requiring multiple surgical interventions. The patient presented with lower urinary tract symptoms. They underwent two endoscopic prostatic resections for recurrent urinary obstruction due to granulomatous prostatitis before a diagnosis of GPA was made. The rarity of this pathology, challenges in making a diagnosis and the systemic nature of this disease are emphasised.

We describe a case of granulomatosis with polyangiitis (GPA; formerly named Wegener granulomatosis) that presented initially as florid areas of gingival swelling. The patient also had upper respiratory symptoms that included sinus congestion and cough of recent onset. Clinical-pathologic correlation aided the interpretation of non-specific biopsy findings and immediate referral to an appropriate medical specialist. Treatment was rendered at an early stage of disease with a good response to date. Review of the literature indicates that gingival swelling, often with the characteristic appearance of "strawberry gingivitis" may represent the initial sign of disease in 2% of patients with GPA. Biopsy of gingival lesions often shows a non-specific histologic appearance that should be interpreted in the context of the clinical appearance and pertinent medical history. The clinical investigations that lead to definitive diagnosis and treatment are presented to facilitate the management of this uncommon but potentially fatal condition. [ABSTRACT FROM AUTHOR]

In an adult female who has been using asthma treatment for years but has not benefited from the treatment, flattening was noted in the inspiratory and expiratory curves of spirometric evaluation. Subsequent examination revealed the presence of subglottic stenosis as the underlying pathology of fixed upper airway obstruction. [ABSTRACT FROM AUTHOR]

Background Although ceramic brackets have been extensively used for decades in orthodontics there is not till today any study focusing on the possible deterioration of mechanical properties after in vivo ageing. Objectives To determine whether the mechanical properties of alumina orthodontic brackets change after intraoral ageing thereby assessing the validity of a theoretical model established for the performance of ceramics in wet environments. Materials and methods Two alumina brackets, one single crystal (Radiance, American Orthodontics, Sheboygan, WI) and one polycrystalline (Clarity, 3M, St. Paul, MN) were included in this study. Ten brackets for each group were collected from different patients after a minimum of 3-month intraoral exposure, whereas as-received brackets of the same manufacturers were used as controls. The specimens were subjected to Raman spectroscopy and were then embedded in epoxy resin and metallographic ground and polished. The mechanical properties of four groups (radiance control: RAC, radiance-retrieved RAR, clarity control: CLC and clarity-retrieved CLR) were determined using instrumented indentation testing according to ISO 14577-2002. The mechanical properties tested were Martens hardness (HM), indentation modulus (E IT), the ratio of elastic to total work, commonly known as elastic index (η IT), and fracture toughness (K IC). The numerical results were statistically analysed employing two-way analysis of variance (ANOVA) and Tukey multiple comparison test at a = 0.05. Results Raman analysis revealed that both brackets are made of a-Al2O3 (corundum). No statistically significant differences were found for HM (N/mm2): RAC = 7249 (1507), RAR = 6926 (1144), CLC = 8052 (1360), CLR = 7390 (2393), or for E IT (GPa): RAC = 141 (27), RAR = 139 (23), CLC = 139 (28), CLR = 131 (47). However, significant differences were identified between the two alumina brackets tested for η IT (%): RAC = 55.7 (4.2), RAR = 54.0 (3.5), CLC = 62.5 (4.4), CLR = 61.8 (4.7), while K IC was measured only for the polycrystalline bracket (Clarity) because of the complicated fractured pattern of the single-crystal bracket. Both brackets share equal HM and E IT before and after orthodontic intraoral ageing. Limitations Whereas the study assessed the changes after intraoral exposure per theoretical model, which describes the reduction of critical stress to induce fracture after wetting, long-term intraoral ageing could have induced more pronounced effects. Conclusions/Implications The results of this study indicate that 3 months of intraoral ageing do not change the mechanical properties of single-crystal and polycrystalline orthodontic brackets tested, thus indicating that the Griffith theory may not be applied to the case of manufactured ceramic brackets owing possibly to internal defects. [ABSTRACT FROM AUTHOR]

Cytomegalovirus infection, which can occur as a result of reactivation due to immunosuppressive treatment in patients with granulomatosis with polyangiitis, is a serious condition that should be kept in mind because of its fatal course. In this article, we report a 49-year-old male patient with a diagnosis of granulomatosis with polyangiitis who developed a life-threatening colonic ulcer due to cytomegalovirus colitis and a shrunken spleen with irregular contours that was detected on abdominal computed tomography. This is a rare case of cytomegalovirus disease in a patient with granulomatosis with polyangiitis and splenic necrosis. [ABSTRACT FROM AUTHOR]

Introduction: Wegener Granulomatosis is a rare multisystemic disease with an unknown cause, characterized by necrotic granulomatous lesions in respiratory tract, systemic vasculitis in small arteries and veins and necrotizing glomerulonephritis. Wegener can affect any organ including kidneys, eyes or other organs but classically affects upper and lower respiratory tract. One of the rare but important signs of this disease is oral involvement, generally occurring in 6-13% of patients, however, oral involvement as the primary manifestation of disease, occurs in only 5-6% of cases. The most common oral manifestation is strawberry gingivitis. Patients: Our patient was a 35 year-old man with gingival bleeding during brushing which began approximately 45 days before referring to the department of oral and maxillofacial diseases, Mashhad Dental School. In intraoral examination, his gingiva had a papillomatous appearance and was purple in color (strawberry appearance). Due to the presence of strawberry appearance in absence of plaque, primary diagnosis of Wegener granulomatosis was established and the patient was referred for histopathological evaluation. In laboratory tests, C-ANCA was positive and P-ANCA was negative. Finally, diagnosis of Wegener granulomatosis was confirmed and his treatment was started. Rheumatologic condition of patient's lungs was evaluated by chest X-ray and CT-scan and blood tests, biochemistry tests and urine analysis were performed for the patient. He did not have pulmonary or renal involvement. In our study, the patient was followed up after 1, 2 and 11 months from the first visit. Discussion: Up to now, few reports have been published on Wegener disease with oral involvement and in most of these articles, Wegener was diagnosed after respiratory symptoms and kidney or other organs involvement. Only in few studies was Wegener diagnosis confirmed on the basis of oral symptoms and gingival involvement. Immediate and aggressive administration of immunotherapy treatments are required due to the fatal nature of the disease as the survival rate of patients with untreated WG is low and 90% of these patients die within 1 year after respiratory or kidney involvement .

Abstract Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease’s onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates. Interstitial involvement, pleural disease and pulmonary embolism are less common. Histology may show necrotizing or granulomatous vasculitis of small arteries and veins of the lung, but since typical features may be patchy, the site for lung biopsy should be carefully chosen with the help of imaging techniques such as computed tomography. Bronchoalveolar lavage is helpful to confirm the diagnosis of alveolar haemorrhage. Pulmonary function tests are frequently altered, showing a reduction in the diffusion capacity for carbon monoxide, which can be associated with obstructive abnormalities related to airway stenosis. Nodular lung lesions tend to regress with immunosuppressive therapy, but lung disease may also require second line treatments such as plasmapheresis. In cases of massive diffuse alveolar haemorrhage, ventilator support is crucial in the management of the patient.

Introduction: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are forms of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV most commonly affects the upper and lower respiratory tract as well as the kidneys. The first symptoms are often nonspecific, requiring careful differential diagnosis with infections and malignancies. Materials and methods: We analyzed the clinical and radiological data of 38 patients (20 females and 18 males) diagnosed with ANCA-associated vasculitis. Lung involvement was observed in 29 cases. Lung ultrasound (LUS) was performed on 21 patients from the study group and compared to chest CT. For 7 patients the examination was conducted repeatedly. Results: In total, 35 LUS and CT examinations were performed, revealing the following lesions: nodules, infiltrates with and without features of disintegration, caves (n = 17), diffuse alveolar hemorrhage (n = 3), and features of interstitial lung disease (ILD) with pulmonary fibrosis (PF) (n = 11). In 2 cases LUS and CT were negative. In 4 cases LUS was negative, despite a positive CT result. Conclusions: Both in CT and LUS, images of pulmonary lesions were consistent though highly variable. Therefore, further studies are required for a larger group of patients. [ABSTRACT FROM AUTHOR]

Aims: Cytomegalovirus (CMV) infection under immunosuppression sometimes causes death. This study aimed to elucidate risk factors for CMV infection in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: Patients with AAV who underwent remission induction treatment at Okayama University Hospital between 2006 and 2016 were retrospectively analyzed. The primary outcome was the development of CMV infection within 3 months. Results: Of the 111 patients, 13 (11.7%) patients developed CMV infection. Patients with CMV infection were older (p = 0.030) and had a higher body mass index (p = 0.029) in comparison to those without CMV infection. A higher proportion had a severe form (p = 0.001) and granulomatosis with polyangiitis (GPA) (p = 0.001), as well as a higher Birmingham Vasculitis Activity Score (p = 0.018) and C-reactive protein (p = 0.018) levels at baseline. Using logistic regression analysis, severe form and GPA were independent risk factors (odds ratio [OR] = 9.68, 95% confidence interval [CI] = 1.92–60.23, and OR = 7.46, 95% CI = 1.46–47.60, respectively). In addition, patients with CMV infection were more likely than those without infection to be glucocorticoid-related diabetes mellitus (p = 0.025). Conclusion: Our study highlights disease severity and subgroups of AAV as risk factors for CMV infection. [ABSTRACT FROM AUTHOR]

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), are autoimmune conditions associated with small vessel inflammation. Earlier studies indicate that complement activation via the alternative pathway plays a major role in the pathogenesis. In this study we have investigated if ANCA-activation of neutrophils from AAV patients leads to activation of the alternative complement pathway. C5a-primed neutrophils (PMN) from 10 AAV patients and 10 healthy controls (HC) were stimulated with PMA or IgG purified from PR3-ANCA positive patients (ANCA IgG). The supernatants were analyzed for release of complement proteins and markers of different granules by ELISA, and release of microparticles (MP) by flow cytometry. The ability of the supernatants to activate the alternative complement pathway was determined by incubation with normal serum and C3bBbP and C5a were measured by ELISA. MP were analyzed by flow cytometry and removed by centrifugation. The supernatants from the AAV patients’ neutrophils produced significantly more C3bBbP compared with HCs (p = 0.0001). C3bBbP levels correlated with the number of MP. After removal of MP from the supernatants, alternative pathway activation was significantly lower. This study shows that primed and ANCA-stimulated neutrophils from AAV patients have a greater ability to activate the alternative complement pathway compared to primed neutrophils from healthy controls. This finding emphasizes the role of complement in the pathogenesis of AAV - underlining the therapeutic potential of C5a and other complement blockade. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA) is an idiopathic vasculitis of medium and small arteries, characterized by necrotizing granulomatous inflammation. GPA typically affects upper and lower respiratory tract with coexisting glomerulonephritis. This disease is generally characterized by antineutrophil cytoplasm antibodies (ANCA), nevertheless, there are rare cases with negative ANCA. GPA affects people at any age, with predominance of the sixth and seventh decade of life. In 80%–95% of the patients the first symptoms of GPA are otorhinolaryngological manifestations of head and neck including nose/sinuses, ears, eyes, larynx/trachea, oral cavity, and salivary glands. Diagnosis of GPA is based on Criteria of the American College of Rheumatology. In clinical practice diagnosis, the presence of distinctive ANCA antibodies and biopsy of affected organ are crucial. GPA must be differentiated from neoplastic, infectious or inflammatory ulcerative lesions of the head and neck. The standard treatment procedure is divided into two essential phases, induction and maintenance. The induction phase is based on combination of systemic corticosteroid and immunosuppressant therapy, whereas the maintenance phase comprises corticosteroids and azathioprine/methotrexate supplementation. Surgical treatment ought to be considered for patients who are not responding to pharmacotherapy.

Although there are many diseases which can cause cavernous sinus syndrome, granulomatosis with polyangiitis (GPA) is rare. This paper reports a case of GPA in cavernous sinus to illustrate the important role of angiitis in cavernous sinus syndrome, which neurologists should pay more attention to. A 37-year-old woman presented palsy of left cranial nerves (CN) II, III, IV, VI and the branches of CN V. MRI showed an enhanced mass in the left cavernous sinus. Laboratory tests showed cytoplasmic ANCA was positive. The nasal mucosa biopsy revealed an inflammatory reaction, while a former renal biopsy had confirmed crescentic glomerulonephritis. The patient was diagnosed as cavernous sinus syndrome with GPA. After the therapy of pulse corticosteroids, the patient was given cyclophosphamide and tacrolimus as a long-term sequential therapy, which achieved a good response. GPA is not a common cause of cavernous sinus syndrome. Clinicians should have the insight into the potential pathogeny of cavernous sinus syndrome. Especially for female patients, the ANCA test may have more important value. Corticosteroids and immunosuppressants are effective treatment methods for GPA. DOI: 10.3969/j.issn.1672-6731.2015.05.015

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. Highlights • WG may induce hypertrophic pachymeningitis. • Posterior fossa HP can cause acute noncommunicating hydrocephalus. • Steroid therapy may be not effective in management of acute hydrocephalus. • ETV can be effective in resolving clinical deterioration and avoiding complications of ventriculoperitoneal shunt. • With ETV in HP-related hydrocephalus, immunosuppressive therapy can be postponed. [ABSTRACT FROM AUTHOR]

Objective: Identification of a biomarker for disease activity in eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss) remains an unmet need. This study examined the value of serum periostin, a marker of type 2 inflammation, as a measure of disease activity in patients with EGPA. Methods: Participants enrolled in a multicenter, prospective cohort of patients with EGPA were included in this study if they had disease activity (defined as Birmingham Vasculitis Activity Score [BVAS] > 0) during follow-up. Serum levels of periostin were measured at flare visit as well as two pre- and two post-flare visits, if available. The outcome of disease activity was assessed either with BVAS or Physician Global Assessment (PGA). Mixed-effect models were used to examine the association between periostin levels and disease activity. Comparisons were made with a historical cohort of healthy individuals and patients with asthma. Results: In the 49 patients included in the study, the median periostin level was 60 ng/ml (IQR 50 to 73) in all visits and did not significantly change across visits. Multivariate analyses found no association between periostin level and presence or absence of flare according to the BVAS (adjusted OR 1.00 [95% CI 0.98 to 1.02], p = 0.98) but an increase in periostin level was significantly associated with greater disease severity during a flare according to the PGA (adjusted beta-coefficient 0.02 [95% CI 0.004 to 0.03], p = 0.01). Periostin levels in EGPA were significantly higher than previously studied healthy controls and patients with asthma. Conclusion: In EGPA serum periostin level is modestly associated with greater disease severity during a flare but does not discriminate active from inactive disease. Periostin levels in EGPA are higher than in other previously studied cohorts, including healthy populations and patients with asthma, and are relatively stable over time. [ABSTRACT FROM AUTHOR]

Herein, we report a case of otitis media caused by methicillin-resistant Staphylococcus aureus (MRSA), presenting as falsely positive for proteinase 3 (PR3)-antineutrophil cytoplasmic antibodies (ANCA). A 47-year-old woman was referred to our hospital with a complaint of left otorrhea. An otorrhea culture yielded MRSA, and the patient was treated using tympanoplasty. Postoperative administration of teicoplanin lead to drug-induced neutropenia and was discontinued 4 days after the operation. One month after the operation, the patient's otorrhea recurred, and it was accompanied by hearing impairment. The otorrhea culture yielded MRSA again, while serum was positive for PR3-ANCA (6.8 U/mL). As MRSA was detected in the patient's otorrhea sample, she was treated with linezolid. Her symptoms then improved immediately. Although the PR3-ANCA positivity remained, the patient's otorrhea and hearing impairment had not recurred for 3 years when this report was submitted. Therefore, we conclude that this is a case of false PR3-ANCA positivity. [ABSTRACT FROM AUTHOR]

Objective: Rare co-existance of disease or pathology Background: Systemic vasculitis can present with a multitude of symptoms involving multiple organ systems. Clinicians should avoid anchoring bias and be cognizant that different types of vasculitides can be present in the same patient and that the diagnosis of one should not preclude the subsequent diagnosis of another. Case Report: A 67-year-old woman was referred for evaluation of episodes of epistaxis and recurrent severe sinusitis. Her physical examination showed nasal congestion and purpuric rash on the lower extremities. CT of the sinuses showed severe mucosal thickening. ANCA serologies were positive with a c-ANCA titer of 1: 5120 and anti- proteinase-3 (anti-PR3) antibodies of 1061 units. Serum creatinine was elevated at 1.32 mg/dL (GFR of 40.62 ml/min). Urine analysis showed proteinuria and hematuria. The patient declined treatment initially, but while awaiting kidney biopsy she developed episodes of headache and blurry vision. She underwent right temporal artery biopsy 4 days later, which confirmed the diagnosis of GCA. The biopsy showed characteristic histopathology findings and she was started on 60 mg of prednisone daily. The kidney biopsy showed pauci-immune crescentic glomerulonephritis (PICGN) consistent with ANCA-associated vasculitis. We identified all the cases of co-presentation of GCA and GPA in the literature and summarized their clinical features in this report. Conclusions: Astute clinicians should be cognizant of overlapping and atypical presentations of vasculitides to avoid delayed diagnosis and errors in management. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA) involves upper and lower respiratory tracts and kidneys. Lung involvement is among the most important organ involvements in GPA. GPA’s lung involvement might be confused with other granulomatous conditions with lung involvement. In this report, we presented clinical features of two cases with GPA who had been diagnosed as tuberculosis (TBC) and well treated with anti-tuberculosis (anti-TBC) drugs. However, one of two cases had ear-nose-throat (ENT) manifestations before the diagnosis of TBC and her extrapulmonary findings related with GPA have added to clinical features in the following years. In the second case, the manifestations of GPA appeared after 13 months of anti-TBC treatment. We speculated that lung involvement in these cases may be due to GPA rather than TBC. Our aim was to highlight difficulties in the differential diagnosis between GPA and TBC and suggest the possible beneficial effect of anti-TBC drugs on the lung involvement due to GPA in light of the literature data. [ABSTRACT FROM AUTHOR]