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Objective: This paper aimed to describe another form of aggressive limited Granulomatosis with polyangiitis (GPA) revealed by dacryoadenitis. Methods and results: We report an unusually limited GPA in a 48-year-old man presenting with bilateral proptosis. She had never presented kidney or pulmonary manifestations, but her disease was persistently active including oto-rhino-laryngological manifestations, dacryoadenitis, and neurological manifestations unresponsive to corticosteroids and immunosuppressors. Discussion: Granulomatosis with polyangiitis (GPA) is an auto-immune inflammatory vasculitis. Involvement of lacrimal glands as the first presentation is uncommon. It is characterized by the development of granulomas. Patients with orbital mass without lacrimal gland involvement have a higher rate of systemic disease, a severe clinical course, and a higher rate of recurrences. A patient with dacryoadenitis seems to be with a good prognosis. Eye manifestations were significantly more common in patients with pachymeningitis. MPO-ANCA-positive pachymeningitis was more frequent in older female patients. PR3-ANCA-positive pachymeningitis had more severe neurological damage. Induction treatment consists of intravenous methylprednisolone (IV) associated with cyclophosphamide. Conclusion: Faced with dacryoadenitis, it is important to screen for ANCA-associated vasculitis. Abbreviations: GPA = Granulomatosis with polyangiitis, ANCA = Antineutrophil Cytoplasmic Antibodies., (#x00A9; The Authors.Romanian Society of Ophthalmology.)

A 14-year-old female presented with 2 weeks of progressive right eye pain, erythema, and proptosis. Examination demonstrated an enlarged palpable mass along the right superior lateral orbit and bilateral conjunctival petechiae. Of note, she was asymptomatic on the left side, and the petechiae were present only on the superior bulbar conjunctiva with eyelid eversion. Imaging demonstrated bilateral lacrimal gland enhancement. Testing was significant for elevated inflammatory markers, but otherwise negative workup. Biopsy of the right lacrimal gland demonstrated acute-on-chronic inflammation without evidence of lymphoproliferative disease. On repeat testing, myeloperoxidase antibody levels (MPO/p-ANCA) were elevated, indicative of an underlying immune-mediated vasculitis. This case illustrates a rare presentation of ANCA-associated vasculitis in a pediatric patient. It further demonstrates the phenomenon of initial negative serology and subsequent auto-antibody seroconversion in a patient with localized granulomatosis with polyangiitis.

Ocular involvement is present in 50-60% of granulomatosis with polyangiitis (GPA) patients and can affect any part of the ocular globe. The present study describes ophthalmologic manifestations, association with systemic symptoms, disease activity and damage in GPA. A cross-sectional study was conducted including patients with GPA who underwent rheumatologic and ophthalmologic evaluation. Demographics, comorbidities, ophthalmologic symptoms, serologic markers, radiographic studies, disease activity and damage were assessed. Descriptive statistics, correlation, univariable logistic regression analyses, Student's t, Mann-Whitney U, Chi-square and Fisher's exact tests were performed. Fifty patients were included, 60% female, the median age was 56 years, disease duration 72.5 months. Nineteen (38%) patients had ocular manifestations at GPA diagnosis, scleritis being the most frequent; 27 (54%) patients presented ocular involvement during follow-up, repeated scleritis and dacryocystitis being the most common manifestations. Concomitant ophthalmic and sinonasal involvement was present in 12 (24%). Ocular and ENT damage occurred in 58% and 70%, respectively. Epiphora and blurred vision were the most frequent symptoms; scleromalacia and conjunctival hyperemia (27%) the most frequent clinical abnormalities. Ocular involvement at diagnosis was associated with concomitant ocular and sinonasal involvement at follow-up (OR 4.72, 95% CI 1.17-19.01, p = 0.01). Ocular involvement at follow-up was associated with age at GPA diagnosis (OR 0.94, 95% CI 0.90-0.99, p = 0.03), VDI (OR 1.29, 95% CI 1.03-1.61, p = 0.02), and ENT damage (OR 5.27, 95% CI 1.37-20.13, p = 0.01). In GPA, ocular involvement is frequent, therefore, non-ophthalmologist clinicians should be aware of this manifestation to reduce the risk of visual morbidity and organ damage.

A 77-year-old Japanese woman presented to an ophthalmologist with an erythematous swollen upper eyelid and diplopia which was initially diagnosed to be idiopathic dacryoadenitis on the basis of a histological evaluation of an orbital mass that was in remission following a 3.5-month period of systemic corticosteroid therapy. She subsequently developed respiratory symptoms, and was finally diagnosed with systemic granulomatosis with polyangiitis (GPA) based on the clinical and histological features. She was successfully treated with corticosteroids and azathioprine. Dacryoadenitis in the form of an orbital inflammatory pseudotumor may be an initial presenting feature of GPA, sometimes as the limited phenotype, and occasionally progressing to systemic disease.

Aim: To estimate the frequency of lesions in the organ of vision in granulomatosis with polyangiitis (GPA) (Wegener's) and to determine their relationship to systemic diseases., Subjects and Methods: The retrospective study enrolled 218 patients followed up at the E.M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases, with a diagnosis of GPA. The frequency and association of ophthalmic manifestations with systemic involvement were statistically analyzed using PASW Statistics 18., Results: The organ of vision was impaired in 48.1% of the patients with GPA. The most common manifestations were orbital space-occupying lesion (22.9%), conjunctivitis/episcleritis (14.7%), dacryocystitis (6.0%), and scleritis (4.6%). Orbital space-occupying lesions occurred more frequently in the local type of the disease (p=0.0003), and, on the contrary, the involvement of the conjunctiva and eyeball was seen in patients with the systemic types of GPA (p=0.02)., Conclusion: The findings may suggest that the orbital lesion is an independent manifestation of GPA, which develops more commonly in its local type. Conjunctivitis/episcleritis is, on the contrary, more frequently seen in the active phase of the disease and generally in the involvement of other organs and systems.

Limited orbital granulomatosis with polyangiitis (GPA) is uncommon and its diagnosis may be delayed, especially when isolated lacrimal involvement is the initial presentation, because clinical manifestations are non-specific and systemic diagnostic criteria are not applicable. Making an early diagnosis despite the absence of systemic progression is extremely important because in some cases the disease is locally destructive, with irreversible visual and functional loss, and it can be refractory to corticosteroids and conventional immunosuppressive drugs to induce remission. The authors report an unusual limited form of orbital GPA in a 35-year-old woman presenting with bilateral dacryoadenitis, evolving later to locally aggressive bilateral orbital pseudotumour leading to proptosis, extraocular myositis, diplopia and medial deviation of the nasal septum. She had never had systemic manifestations but her disease was persistently active and unresponsive to corticosteroids and immunosuppressors. The aim of this paper is to provide further evidence of aggressive and refractory limited forms of GPA., (2016 BMJ Publishing Group Ltd.)

Wegener's granulomatosis is a systemic vasculitis of unknown aetiology. Although it classically involves the upper respiratory tract, lungs and kidneys, virtually any organ may be affected. We report a rare case of a 45 year old female who presented with bilateral-dacroadenitis, otitis media, parotid enlargement and left sided lower motor neuron type of facial palsy as the initial manifestations of Wegener's granulomatosis.

Objective: The objective of this study was to review the various clinical features associated with Wegener's granulomatosis (WG) in the head and neck region and to discuss the difficulty of diagnosing patients with early stage WG., Methods: Between January 1998 and August 2007, WG was diagnosed and treated in 16 patients at the Department of Otolaryngology, Hyogo College of Medicine. Clinical and operating records of these patients were analyzed retrospectively. Diagnosis was based on the Japanese criteria proposed by the Japanese Ministry of Health and Welfare in 1998., Results: Ten patients (62.5%) had a definite diagnosis of WG, and the other six patients (37.5%) had a probable diagnosis of WG. The period from the onset to diagnosis was between 1 month and 30 years. The generalized form of WG was observed in three patients (18.8%), and the limited form of WG was observed in the other 13 patients (81.2%). Nasal, aural, and ophthalmic symptoms were initially presented in 10, 3, and 3 patients, respectively. Cytoplasmic pattern antineutrophil cytoplasmic antibodies (cANCAs) and perinuclear pattern ANCA (pANCA) were positively detected in 68.8% (11/16) and 27.2% (3/11) of the patients, respectively. Five of 14 patients (35.7%) had pathologic features of WG in biopsy samples from the head and neck region. Three patients in whom a diagnosis of WG was difficult are presented, and immediate lessons of our experience were discussed., Conclusions: This study emphasized the difficulty of diagnosing WG, particularly at an early stage and when limited to the head and neck region. The biggest challenge faced in diagnosing WG is that it requires a high index of suspicion. When WG was suspected, we should obtain an accurate medical history from patients and repeat serologic and histopathologic examinations.

Ophthalmic manifestations are noted in about 50% of adults with Wegener's granulomatosis. Wegener's granulomatosis is a rare disease in the pediatric age group. The disease may present initially with ocular manifestations. We report the largest series to date of children whose ocular findings were the initial presenting signs of Wegener's granulomatosis.

Introduction: Wegener's granulomatosis is a necrotizing granulomatous vasculitis characterized by the involvement of the upper airways, lungs and kidneys., Case Report: A 39-year-old man presented with a 8-month history of proptosis of left eye. He had a history of recuring sinusitis and dyspnea. The diagnosis of Wegener's granulomotosis was confirmed by the nasal biopsy. A complete remission was achieved after treatment with corticosteroids, cyclophosphamide and trimethoprine sulfamethoxazole., Comments and Conclusion: Wegener's granulomatosis is a multisystemic inflammatory disease of unknown origin. The classic diagnostic triad of this disease is necrotizing granuloma of the upper or lower respiratory tract, vasculitis and nephritis. The disease is usually fatal if untreated. However, early diagnosis and immunosuppressives such as cyclophosphamide can induce long term remission.

Background: We report surgical treatment of a patient with dacryocystitis and retinal detachment (RD), which are rare ophthalmic involvements of Wegener granulomatosis (WG)., Case: The patient was a 26-year-old man with WG. He was diagnosed as having WG 4 years ago and he has been treated by maintenance doses of predonisolone and cyclophosphamide. Rheumatoid factor and serum antinuclear antibody were negative. Cytoplasmic pattern-antineutrophil cytoplasmic antibody (C-ANCA) and renal function were normal. He was found to have nasolacrimal duct obstruction and lattice degeneration bilaterally, retinal tear with RD in the left eye and tear without RD in the right eye. No sign of vasculitis was found in fluorescein angiography. Bilateral dacryocystorhinostomy was performed without any sign of postoperative necrosis of the wound. After the surgery, epiphora and eye discharge disappeared and lacrimal passage has been maintained without obstruction. The pathological findings of his nasal mucosa and lacrimal sac showed chronic inflammation and no typical changes of WG. There was no abnormal change in the conjunctiva and sclera after an uncomplicated scleral buckling surgery., Conclusion: We conclude that operations such as dacryocystorhinostomy and scleral buckling surgery may be performed successfully when WG is controlled within the normal limits of C-ANCA.

Purpose: To report a case presenting with dacryoadenitis as the earliest manifestation of systemic Wegener's granulomatosis (WG)., Design: Observational case report., Methods: A 41-year-old woman initially presented symptoms of bilateral dacryoadenitis. She subsequently developed upper and lower respiratory tract involvement, scleritis and keratitis., Results: Cytoplasmic antineutrophil antibody (c-ANCA) titer was positive. The lacrimal gland and lung biopsies were consistent with WG. The patient responded well to cyclophosphamide and prednisolone., Conclusions: Dacryoadenitis maybe the earliestpresenting manifestation of WG andprompt immunosuppressive chemotherapy may control it preventing the limited disease from progressing to a complete form and reducing its morbidity and mortality.

The clinical, radiological and pathological findings, natural history and response to treatment are investigated in 3 patients. They represent the only examples of W.G. who were seen over a 15 year period at our University Hospital and are characteristic of generalize and limited form. The role of immune complexes in the pathogenesis of W.G. and diagnostic importance of pulmonary radiological pattern are described.