scholarly journals 062. UPPER AND LOWER RESPIRATORY TRACT INVOLVEMENT IS SIMILAR IN MPO-ANCA AND PR3-ANCA GRANULOMATOSIS WITH POLYANGIITIS

Rheumatology ◽  
2019 ◽  
Vol 58 (Supplement_2) ◽  
Author(s):  
Marco Antonio Alba Garibay ◽  
Susan Hogan ◽  
Ashley Henderson ◽  
Caroline Poulton ◽  
Yichun Hu ◽  
...  
Author(s):  
Wolfgang L. Gross ◽  
Julia U. Holle

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener’s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a ’pure’ small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.


Author(s):  
Wolfgang L. Gross ◽  
Julia U. Holle

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a 'pure' small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.


2021 ◽  
Author(s):  
Elena A Lapteva ◽  
Olga N Kharevich ◽  
Victoria V. Khatsko ◽  
Natalia A. Voronova ◽  
Maksim V. Chamko ◽  
...  

Background: Lower respiratory tract (LRT) involvement, observed in about 20% of patients suffering from coronavirus disease 2019 (COVID-19) is associated with a more severe clinical course, adverse outcomes and long-term sequelae. Early identification of LRT involvement could facilitated targeted and timely interventions that could alter the short- and long-term disease outcomes. The LungPass is an automated lung sound analysis platform developed using neural network technology and previously trained. We hypothesised that the LungPass could be used as a screening tool for LRT involvement in patients with COVID-19. Methods: In a prospective observational study involving 282 individuals with presenting in the emergency department with a strong clinical suspicion of COVID-19 and imaging findings consistent with COVID-19 LRT involvement (25.5% had concomitant hypoxia), and 32 healthy controls, we assessed the sensitivity and specificity of the LungPass in identifying LRT involvement in COVID-19. We also compared the auscultatory findings of the LungPass compared to a chest physician using a traditional, high-quality stethoscope. Results: Among individuals with COVID-19 LRT involvement, the LungPass identified crackles in at least one auscultation site in 93.6% and in two or more points in 84%. Moreover, the LungPass identified any abnormal lung sound (crackles or wheeze) in at least one auscultation site in 98.6% and in at least two points in 94% of the participants. The respective percentages for the respiratory physicians were lower. Considering the presence of any added abnormal sound (crackles or wheeze) in at least two auscultation points as evidence of LRT involvement, LungPass demonstrated a sensitivity of 98.6% (95% confidence intervals [CI]: 96.4%-99.6%) and a specificity of 96.9% (95% CI: 83.8%-99.9%) in identifying COVID-19 LRT involvement. Conclusion: This exploratory study suggests the LungPass is a sensitive and specific platform for identifying LRT involvement due to COVID-19, even before the development of hypoxia.


2021 ◽  
Vol 14 (1) ◽  
pp. e237876
Author(s):  
Saddam Yasin ◽  
Kanwal Mehmood

Granulomatosis with polyangiitis (GPA) is characterised by systemic necrotising vasculitis of small arteries and veins with multitude of organ involvement, with the most common being the upper and lower respiratory tract and renal system. Gastrointestinal involvement is a rare late manifestation with a high mortality rate and usually results in intestinal perforation. Our patient presented with gastrointestinal bleeding secondary to jejunal artery vasculitis. Gastrointestinal bleeding as initial presentation of GPA is very rarely documented. CT mesenteric angiogram is helpful for the localisation of bleed in these cases. In case of refractory bleeding, surgical excision is required.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3032-3032
Author(s):  
Nicola Lehners ◽  
Paul Schnitzler ◽  
Gerlinde Egerer ◽  
Peter Dreger ◽  
Christoph Eisenbach ◽  
...  

Abstract Abstract 3032 Respiratory syncytial virus (RSV) is a pathogen associated with bronchiolitis in infants and small children. However, immunocompromised adults might also be at risk for severe lower respiratory tract involvement induced by RSV. We report the rapid control of an outbreak of nosocomial RSV infections in an institution with special focus on allogeneic and autologous stem cell transplantations. Between November 2011 and March 2012 56 patients were diagnosed with RSV infection by RT-PCR at our institution. In order to identify possible risk factors for lower respiratory tract involvement in RSV infected patients, clinical course of infection as well as radiological and laboratory findings were evaluated. Characterization of RSV strains in respiratory specimens was achieved by sequence analysis of part of the RSV glycoprotein G gene and phylogenetic comparison of outbreak and community strains was performed. Out of 56 patients with RSV infection, 39 patients showed signs of lower respiratory tract involvement. 14 fatal outcomes were observed. In most of the lethal cases coinfections with other pathogens such as Aspergillus spp. or Pseudomonas aeruginosa were present. Therefore the exact impact of RSV on the fatal outcome remains difficult, if not impossible, to assess. Age, sex, underlying disease, disease control or transplant status did not differ significantly between severe and non-severe cases. However, hypogammaglobulinemia was associated with a higher risk for fatal infection (p = 0.03) in univariate analysis, whereas therapy with oral ribavirin might show a protective effect (p = 0.02). Prolonged viral shedding was frequently observed (median 22.5 [1 – 143] days), most pronounced in patients folllowing allogeneic transplantation (p = 0.04). The major mode of RSV transmission seemed to be direct patient-to-patient contact as suggested by evaluation of patient movements on the wards. Sequence analysis on respiratory specimens obtained from 46 patients revealed the presence of a particular outbreak strain in 40 patients, characterized by an identical nucleotide sequence of RSV GA2 subtype. Phylogenetic analysis showed low prevalence of the outbreak strain in the community. By implementation of rigorous isolation measures containment of outbreak was achieved. Highly contagious viral pathogens such as RSV are able to cause rapidly spreading outbreaks among immunocompromised patients. Hypogammaglobulinemia might be a risk factor for severe lower respiratory tract infection. Treatment with oral ribavirin might have a positive effect on outcome. Rigorous isolation measures are essential to the containment of outbreak. Disclosures: Off Label Use: ribavirin aerosol is FDA approved for severe cases of RSV in infants and young children.


Author(s):  
Anna C. Rabha ◽  
Fátima R. Fernandes ◽  
Dirceu Solé ◽  
Leonard B. Bacharier ◽  
Gustavo F. Wandalsen

2008 ◽  
Vol 8 ◽  
pp. 845-847 ◽  
Author(s):  
Jouda Cherif ◽  
Sonia Toujani ◽  
Nadia Mehiri ◽  
Bechir Louzir ◽  
Nidham Kchir ◽  
...  

Sinus histiocytosis with massive lymphadenopathy (SHLM) disease is considered to be an indolent and self-limiting pathology. However, severe morbidity and mortality have been attributed to complications of SHLM. Lower respiratory tract involvement is rarely reported and is often unfavorable, and carries a particularly grave prognosis. A case of SHLM is reported, in which the patient had lower respiratory and pleural involvement.


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