Cough and Nasal Pain: Putting Together an Uncommon Diagnosis
The pulmonary vasculitides include a wide spectrum of disorders that have in common inflammation and destruction of pulmonary blood vessels. Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is the most common of the antineutrophil cytoplasmic antibody–associated small-vessel vasculitides, and affects a number of organ systems. GPA affects a wide age range, although it typically presents in middle adulthood. The classic triad of GPA—upper airway involvement (sinusitis, otitis media, mucosal ulcerations, airway inflammation), lower respiratory tract disease, and glomerulonephritis—is no...
Source: Clinical Pulmonary Medicine - March 1, 2019 Category: Respiratory Medicine Tags: Images In Pulmonary Medicine Source Type: research
Rituximab as an effective and probably safe treatment for granulomatosis with polyangiitis (Wegener's Granulomatosis).
PMID: 30785092 [PubMed - as supplied by publisher] (Source: International Immunopharmacology)
Source: International Immunopharmacology - February 20, 2019 Category: Allergy & Immunology Authors: Tavakolpour S, Alesaeidi S Tags: Int Immunopharmacol Source Type: research
Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis): Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome
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. (Source: Annals of Plastic Surgery)
Source: Annals of Plastic Surgery - February 7, 2019 Category: Cosmetic Surgery Tags: Transplantation Surgery and Research Source Type: research
Erythematous and Vascular Oral Mucosal Lesions: A Clinicopathologic Review of Red Entities
AbstractErythematous lesions of the oral mucosa are common and can reflect a variety of conditions, ranging from benign reactive or immunologically-mediated disorders to malignant disease. Together with vascular abnormalities, which can vary from reddish to bluish-purple in color, the differential diagnosis for erythematous oral mucosal change is quite diverse. This review focuses on salient clinical features and histopathologic findings of selected conditions which clinically present as red or vascular-like oral mucosal alterations, including oral vascular malformations and neoplasms, pyogenic granuloma, localized juvenil...
Source: Head and Neck Pathology - January 29, 2019 Category: Pathology Source Type: research
Isolated choroid plexus involvement in a case of granulomatosis with polyangiitis negative for antineutrophil cytoplasmic antibodies (ANCA)
granulomatosis with polyangiitis (GPA, formerly Wegener's) is a systemic necrotizing vasculitis that predominantly involves respiratory tract and kidneys [1,2]. GPA usually presents with nervous system involvement in the later course, mainly affecting the peripheral nervous system [3]. Vasculitis has been reported up to 6% of GPA cases with central nervous system (CNS) involvement [2,4]. Detection of either anti-proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA can support the diagnosis, but in some cases ANCA are not detected [3]. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - January 26, 2019 Category: Neurology Authors: Margoni M, Barbareschi M, Rozzanigo U, Chioffi F, Paolazzi G, Marangoni S Tags: Letter to the Editor Source Type: research
Radiotherapy in advanced glottic laryngeal carcinoma in a patient with Wegener & rsquo;s granulomatosis: how much radiation dose is needed?
(Source: OncoTargets and Therapy)
Source: OncoTargets and Therapy - January 22, 2019 Category: Cancer & Oncology Tags: OncoTargets and Therapy Source Type: research
[Modern management of otologic consequences of Wegener's granulomatosis - a case report and review of the literature].
Abstract
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 treatmen...
Source: Orvosi Hetilap - January 1, 2019 Category: General Medicine Authors: Tóth I, Kaszás B, Horváth G, Piski Z, Bakó P, Lujber L, Gerlinger I, Révész P Tags: Orv Hetil Source Type: research
Transfusion of Soluble Target Antigens to Pre-Immunized Recipients: A Previously Overlooked Mechanism in Transfusion-Related Acute Lung Injury
Transfusion related lung injury (TRALI) is a leading cause for transfusion related morbidity and mortality in industrialized countries. The majority of cases is induced by antibodies present in donor plasma, and exclusion of antibody carriers from the donor pool has decreased the number of TRALI cases significantly. However, TRALI is still reported. We have identified a new and previously overlooked mechanism in which soluble antigens present in the blood component become the target of pre-existing antibodies present in the recipient.Some of us have recently reported a case of TRALI in a female patient precipitating after ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Bayat, B., Nielsen, K., Burg-Roderfeld, M., Bein, G., Sachs, U. J. Tags: 401. Basic Science and Clinical Practice in Blood Transfusion: Clinical and Translational Studies Source Type: research
Eyelid oedema as an initial manifestation of Wegener's granulomatosis
Conclusion: Azithromycin associated with RAR is not effective as adjuvant therapy in patients with chronic periodontitis. (Source: Revista Espanola de Cirugia Oral y Maxilofacial)
Source: Revista Espanola de Cirugia Oral y Maxilofacial - November 12, 2018 Category: ENT & OMF Source Type: research
Pattern of Scleritis in an Egyptian Cohort.
CONCLUSION: The visual prognosis of an eye with scleritis varies with the subtype of scleral inflammation. In our cohort, it was found to be poorer in eyes with necrotizing scleritis with inflammation compared to other subtypes.
PMID: 30412434 [PubMed - as supplied by publisher] (Source: Ocular Immunology and Inflammation)
Source: Ocular Immunology and Inflammation - November 10, 2018 Category: Allergy & Immunology Tags: Ocul Immunol Inflamm Source Type: research
Diffuse Alveolar Hemorrhage and Pulmonary Vasculitides: Histopathologic Findings
Semin Respir Crit Care Med 2018; 39: 425-433 DOI: 10.1055/s-0038-1669412Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. In the lung, vasculitis impacts both the pulmonary vessels ...
Source: Seminars in Respiratory and Critical Care Medicine - November 7, 2018 Category: Respiratory Medicine Authors: Scapa, Jason V. Fishbein, Gregory A. Wallace, W. Dean Fishbein, Michael C. Tags: Review Article Source Type: research
Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment
Semin Respir Crit Care Med 2018; 39: 434-458 DOI: 10.1055/s-0038-1660874Granulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is the most common of the pulmonary vasculitides. GPA typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive “geographic” necrosis, and granulomatous inflammation. The spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site ...
Source: Seminars in Respiratory and Critical Care Medicine - November 7, 2018 Category: Respiratory Medicine Authors: Lynch, Joseph P. Derhovanessian, Ariis Tazelaar, Henry Belperio, John A. Tags: Review Article Source Type: research
Reconstruction of Wegener Granulomatosis Nose Deformity Using Fascia Lata Graft
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...
Source: Journal of Craniofacial Surgery - November 1, 2018 Category: Surgery Tags: Clinical Studies Source Type: research
Brown Tumor of the Cervical Spine in a patient with secondary hyperparathyroidism: A case Report
ConclusionThe importance of the prompt diagnosis of the brown tumor is to establish a multidisciplinary management to prevent progression, neurologic complications and sequelae despite its benign behavior. (Source: International Journal of Surgery Case Reports)
Source: International Journal of Surgery Case Reports - September 18, 2018 Category: Surgery Source Type: research
Application of 3D Printing for Patient-Specific Silicone Stents: 1-Year Follow-Up on 2 Patients
We report the 1-year outcome of 2 patients with airway disease caused by granulomatosis with polyangiitis (Wegener ’s) affecting the left main bronchus and secondary carina. These patients had not responded to systemic therapy or standard bronchoscopic techniques and had complications with commercially available airway stents. We describe a first-in-human, clinical experience to address these issues. Using com puted tomography (CT) imaging and 3D printing technology, we generated and implanted patient-specific silicone airway stents to address airway disease. 3D patient-specific stent prescription was created using a CT ...
Source: Respiration - September 17, 2018 Category: Respiratory Medicine Source Type: research
