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Connective tissue disease
Published in Catherine Nelson-Piercy, Handbook of Obstetric Medicine, 2020
Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody-related systemic vasculitis involving predominantly the upper respiratory system (sinuses, nose), the lungs (causing haemoptysis due to alveolar haemorrhage), and the kidneys.
Successful Pulmonary Rescue of Adult Onset Granulomatosis with Polyangiitis Using Extracorporeal Membrane Oxygenation and Window Thoracostomy
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
Ashish Pulikal, Jason Long, Benjamin Haithcock
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s disease, is an autoimmune disease with features of medium-to-small vessel vasculitis and granulomatous inflammation most frequently affecting the pulmonary-renal axis. While the prevalence in the United States is only three cases per 100,000 people, there is a strong predominance to male individuals of northern European descent (>90% of cases in the US). The symptoms manifest with varying degrees of severity, and the development of pulmonary insufficiency necessitating extracorporeal oxygenation is exceedingly rare and infrequently reported in this patient population [1]. Our patient represents one of only thirteen reported cases of successful hospital discharge following severe pulmonary GPA treated with aggressive immunosuppressive therapies vvECMO. As well, our patient is the only reported case requiring adjunctive window thoracostomy for Stenotrophomonal/Pseudomonal cavitary superinfection and aspergillosis. All medical and surgical treatment was delivered at a major academic quaternary care hospital.
Granulomatous Conditions of the Nose
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
Joanne Rimmer, Valerie J. Lund
Granulomatosis with polyangiitis (GPA) is a systemic condition characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis affecting small- to medium-sized vessels with focal or proliferative glomerulonephritis.27 GPA was formerly known as Wegener’s granulomatosis, after Wegener’s article on rhinogenic granulomatosis in 1939, but has been renamed as part of a move towards a vasculitis terminology based on pathology.28,29 GPA classically involves a triad of upper airway, lung and renal disease, but limited or localized forms of the condition have been reported in up to 30% of cases.30 Thus, the true incidence of the condition is difficult to determine. Whilst up to 1967 only 138 unequivocal cases had been recorded in the literature, the incidence is now estimated to be 10–15 per million per year with a prevalence of up to 5 per 100,000 population in Europe.30-32
Balloon dilatation versus CO2 laser surgery in subglottic stenosis, a retrospective analysis of therapeutic approaches
Published in Acta Oto-Laryngologica, 2023
Anders Erlandsson, Mimmi Werner, Anna Holm, Alexandra Schindele, Katarina Olofsson
Subglottic stenosis (SGS) is a recurrent disease characterized by progressive reduction of the airway due to soft tissue inflammation [1]. Patients suffer from intensifying loss of breath, dyspnoea, wheezing, chronic cough, and affected voice. SGS is in most cases initially perceived as treatment-resistant asthma by both patients and providers; further diagnostics and treatment are therefore often delayed. New diagnostic methods are warranted and are under development [2,3]. The incidence of idiopathic SGS (iSGS) ranges from 2–2.5 cases per 1,000,000 individuals annually, with proposed local increase in a study from Canada [4]. Including all known causes the incidence is reported to be 4.9 cases per 1,000,000 individuals annually with major impact on quality of life as well as on healthcare facilities [3,5]. The annual healthcare costs for patients with SGS are comparable to the financial challenges addressed patients with chronic diseases such as chronic obstructive pulmonary disease (COPD) and diabetes mellitus [6]. SGS can develop after iatrogenic injuries, for example endotracheal intubation damage. Autoimmune disorders, such as granulomatosis with polyangiitis (GPA), are also recognized risk factors. However, in the majority of cases, the cause of SGS remains unknown. The unknown cause adds complexity in decision making regarding therapy, highlighting the need to map the therapeutic interventions available [1].
Subclinical Alterations in Retinal Layers and Microvascular Structures with OCTA in ANCA-Associated Vasculitides
Published in Ocular Immunology and Inflammation, 2023
Yusuf Ziya Güven, Fahrettin Akay, Berkay Akmaz, Dilek Solmaz, Önay Gercik, Servet Akar
Antineutrophil cytoplasm antibody (ANCA) associated vasculitides (AAV) can be grouped under four titles. The main two of these are Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA; formerly known as Wegener’s granulomatosis) and the other two are eosinophilic granulomatosis with polyangiitis (EGPA; also called Churg–Strauss syndrome), and renal limited vasculitis (RLV).1,2 Granulomatosis with polyangiitis (GPA) is a systemic inflammatory disease whose histopathological features usually include granuloma formation, necrosis, and vasculitis of small vessel.2 It was first described by Godman and Churg in 1954 as the triad of necrotizing granuloma, systemic vasculitis and necrotizing glomerulonephritis in the upper and lower respiratory tract.3 Sometimes it progresses with limited organ involvement, sometimes it can turn into a more general form with the involvement of the nose, lung and kidney.4 Patients with AAV usually have nontypical symptoms like fever, slim, weakness, arthralgia, and myalgia.5 It has been shown that patients with GPA have upper respiratory tract involvement in more than 90%, kidney involvement in 77–85%, and dermatological involvement in 50%.5,6 Ophthalmological involvement represents a significant cause of morbidity that occurs in around 50% of GPA patients, which can cause any eye structure involvement, although it is mostly orbital involvement.7 Loss of vision or total blindness due to late diagnosis or inadequate treatment can be seen in 8–37% of patients.8
How best to manage relapse and remission in ANCA-associated vasculitis
Published in Expert Review of Clinical Immunology, 2022
Xavier Puéchal, Loïc Guillevin
It is now well recognized that a prior relapse, diagnosis of granulomatosis with polyangiitis (GPA), proteinase-3 (PR3)-ANCA positivity, and/or higher estimated glomerular filtration rate increase the probability of relapse [6]. Early drug withdrawal [7], mycophenolate mofetil (MMF) [8] or methotrexate (MTX) for remission induction [9], rather than CYC, maintenance therapy with MMF versus azathioprine (AZA) [10] or AZA versus rituximab (RTX) [11] also put the patient at higher risk of relapse. The abilities of CD19+ B-cell reconstitution and/or anti-PR3-ANCA-titer change to predict relapse have not been proven [12]. Continued regular clinical monitoring of AAV patients after remission induction enables the early detection of relapses with less advanced manifestations than initially [13,14]. Herein, maintenance therapies are reviewed, and we discuss new recommended strategies to prevent and treat relapses, focusing on GPA and microscopic polyangiitis (MPA).