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Respiratory Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
Ian Pavord, Nayia Petousi, Nick Talbot
Patients typically present with a nasal discharge, respiratory symptoms (cough, pleurisy and perhaps haemoptysis) and general malaise. The lungs are involved in most cases. Chest radiography shows rounded opacities, which may cavitate. There may also be pleural effusions and areas of infiltration. Antineutrophil cytoplasmic antibody (ANCA) is usually positive.
Optic Neuropathies Associated with Systemic Disorders And Radiation-Induced Optic Neuropathy
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
GPA is a systemic necrotizing vasculitis. As such, it is linked with polyarteritis nodosa (PAN) and other anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides, namely microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) (9). The clinical features of these conditions are protean and can be both life- and organ-threatening (9). For the purpose of this review we will focus on GPA, because it is the most common systemic necrotizing vasculitis subtype associated with optic neuropathy (9). GPA typically targets vessels in the upper and lower respiratory tracts, paranasal sinuses, kidneys and lungs (1, 9, 10). Unlike some of the other systemic inflammatory conditions described, GPA affects both sexes equally, with 40–55 years representing the typical age range of diagnosis (1, 9, 10). Caucasians are the most commonly affected ethnic group, whereas African Americans represent only 2–8% of this patient population, in most studies (1, 9, 10).
Vasculitis
Published in Jason Liebowitz, Philip Seo, David Hellmann, Michael Zeide, Clinical Innovation in Rheumatology, 2023
Michelle L. Robinette, Eli Miloslavsky, Zachary S. Wallace
Although the vasculature is well-suited for biomarker discovery, given direct apposition to the blood system, clinically useful markers specific to vasculitis are generally lacking. The most well-studied and frequently used disease-specific biomarker in vasculitis is the ANCA in AAV. First described in the 1980s, the expanding use of ANCA testing has led to improved recognition and earlier treatment initiation for patients with ANCA-associated vasculitis (98). In particular, the use of enzyme-linked immunosorbent assays (ELISAs) to detect ANCA specific for MPO or PR3 has largely replaced the use of immunofluorescence (e.g., perinuclear or cytoplasmic staining) in recent years (99, 100). This shift has made ANCA testing less subject to interobserver variability and improved performance overall, as detailed in recent studies.
Efficacy of protein A immunoadsorption and therapeutic plasma exchange in ANCA-associated vasculitis with severe renal involvement: a retrospective study
Published in Annals of Medicine, 2023
Xiaojuan Liu, Ming Xia, Di Liu, Haiyang Liu, Chengyuan Tang, Guochun Chen, Yu Liu, Fang Yuan, Hong Liu
A total of 83 patients meeting AAV diagnostic criteria were initially screened from 1 January 2018 to 28 February 2021 in the study. According to the inclusion and exclusion criteria, 48 patients including 18 patients with PAIA treatment and 30 patients with TPE treatment were enrolled in the retrospective study. The baseline characteristics are shown in Table 1, and the extrarenal manifestations of these patients are listed in Table 2. Overall, the median age was 61 years old, and 34 patients (70.8%) were males. The median disease duration was 1.5 months. Forty-six patients (95.8%) were positive for MPO-ANCA, and the other two (4.2%) were positive for PR3-ANCA. The median baseline serum creatinine (SCr) and eGFR were 497.6 μmol/L and 9.8 mL/min/1.73 m2, respectively. Thirty-two patients (66.7%) required haemodialysis with SCr ≥ 500 μmol/L. All patients had active disease at baseline, with a median baseline BVAS of 15.9. Forty-seven patients (97.9%) received GC in combination with CYC, and one patient (2.1%) received GC in combination with RTX as induction of remission. There were no significant differences between the baseline of PAIA and TPE groups. The median follow-up time was 14.5 months.
Circulating immune-complexes and complement activation through the classical pathway in myeloperoxidase-ANCA-associated glomerulonephritis
Published in Renal Failure, 2022
Tadasu Kojima, Dan Inoue, Takeaki Wajima, Takahiro Uchida, Muneharu Yamada, Isao Ohsawa, Takashi Oda
Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (AAGN) is a serious kidney disease characterized clinically by rapidly progressive glomerulonephritis (RPGN) and histologically by necrotizing glomerulonephritis with crescents. Although the precise pathogenic mechanism of AAGN has not been fully elucidated, the central role of ANCA has been accepted widely. ANCA is the autoantibody against neutrophil proteins, two major targets of which are proteinase 3 and myeloperoxidase (MPO). Both ANCAs can bind to and activate primed neutrophils expressing target antigens of ANCA on their cell surface, causing respiratory burst with release of neutrophil extracellular traps (NETs), containing DNA fibers, histones, coated with neutrophil derived proteinases such as MPO and neutrophil elastases [1,2]. NETs components are suspected to cause tissue injury and augment autoimmunity, leading to further production of ANCA.
Complement activation prior to symptom onset in myeloperoxidase ANCA-associated vasculitis but not proteinase 3 ANCA associated vasculitis - A Swedish biobank study
Published in Scandinavian Journal of Rheumatology, 2022
L Johansson, E Berglin, O Eriksson, AJ Mohammad, J Dahlqvist, S Rantapää-Dahlqvist
This study was performed using serum samples collected from individuals before the onset of symptoms of AAV. The identification of individuals and samples has been presented in detail in a previous publication (9), while the procedure will be summarized here. The Swedish National Inpatient Register and the Cause of Death Register were used to identify individuals diagnosed with AAV as first diagnosis in the discharge summary and/or cause of death between 1987 and 2011. The personal identity numbers of the individuals with AAV were linked to the registers of five biobanks in Sweden (age ≥ 18 years and samples > 1 month and < 10 years before date of symptom onset). Medical records were reviewed to identify the time-point for symptom onset and to confirm the diagnosis of AAV using the European Medicines Agency (EMA) algorithm (10). Information about ANCA positivity at diagnosis and clinical manifestations at symptom onset and at diagnosis was collected from the medical records.