ANCA-Associated Vasculitis

Objective.Aspects of antineutrophil cytoplasmic antibodies–associated vasculitis (AAV) prioritized by patients with AAV were described using the International Classification of Function, Disability, and Health (ICF).Methods.Items identified during 14 individual interviews were incorporated into an ICF-based questionnaire administered to participants of 2 vasculitis patient symposia: 36 in the United Kingdom and 63 in the United States.Results.Categories identified as at least “moderately relevant” by ≥ 5% of subjects included 44 body functions, 14 body structures, 35 activities and participation, 31 environmental factors, and 38 personal factors.Conclusion.Identified categories differ from those identified by the current Outcome Measures in Rheumatology (OMERACT) core set and those prioritized by vasculitis experts.

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A proposed role of neutrophil extracellular traps and their interplay with fibroblasts in ANCA-associated vasculitis lung fibrosis

Autoimmunity Reviews ◽

10.1016/j.autrev.2021.102781 ◽

2021 ◽

pp. 102781

Author(s):

Miguel Negreros ◽

Luis F. Flores-Suárez

Keyword(s):

Lung Fibrosis ◽

Neutrophil Extracellular Traps ◽

Extracellular Traps ◽

Anca Associated Vasculitis

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The cancer risk according to three subtypes of ANCA-associated vasculitis: a propensity score-matched analysis of a nationwide study

Seminars in Arthritis and Rheumatism ◽

10.1016/j.semarthrit.2021.03.014 ◽

2021 ◽

Author(s):

Sang Tae Choi ◽

Song Vogue Ahn ◽

Poong Hoon Lee ◽

Chang Mo Moon

Keyword(s):

Propensity Score ◽

Cancer Risk ◽

Nationwide Study ◽

Anca Associated Vasculitis

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AB0507 INVASIVE ASPERGILLOSIS IN ADULT RHEUMATOLOGICAL PATIENTS IN SAINT PETERSBURG, RUSSIA.

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.3796 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1551.1-1552

Author(s):

V. Mazurov ◽

O. Shadrivova ◽

M. Shostak ◽

L. Martynova ◽

M. Tonkoshkur ◽

...

Keyword(s):

Risk Factors ◽

Systemic Lupus Erythematosus ◽

Renal Failure ◽

Invasive Aspergillosis ◽

Lupus Erythematosus ◽

Granulomatosis With Polyangiitis ◽

Control Group ◽

Systemic Lupus ◽

Anca Associated Vasculitis ◽

Underlying Diseases

Background:Invasive aspergillosis (IA) is a severe opportunistic infection that is not well understood in rheumatological patients.Objectives:To study risk factors, etiology, clinical manifestations and results of treatment of IA in adult rheumatological patients.Methods:Retrospective analysis of 830 patients (1998-2019) with “proven” and “probable” IA (EORTC / MSG, 2019), adults - 699 (84%). The main group included 18 (3%) adult rheumatological patients with IA, a control group included 610 (87%) adult hematological patients. Rheumatological patients were older, the average age was 59 years (21–75) vs 45 years (18–79), p = 0.005, and among them there were more women – 56% vs 42%, p = 0.01.Results:In rheumatological patients with IA, underlying diseases were ANCA-associated vasculitis (28%), granulomatosis with polyangiitis (22%), periarteritis (11%), systemic lupus erythematosus (22%), rheumatic heart disease (11%) and ankylosing spondylitis (6%). In the control group, underlying diseases were acute leukemia (45%), lymphomas (34%), chronic leukemia (9%), multiple myeloma (7%), myelodysplastic syndrome (3%), and other hematological diseases (2%).The main risk factors for IA development in rheumatological patients were: systemic steroids use (89% vs 69%), prolonged lymphocytopenia (76% vs 65%, median - 14 vs 12 days), treatment in ICU (44% vs 18%, p = 0.01), acute or chronic renal failure (39% vs 1%, p = 0.0008) and immunosuppressive therapy (28% vs 25%). Severe neutropenia was noted significantly less frequently (18% vs 83%, p = 0.0001). Additional risk factors were decompensated diabetes mellitus (17% vs 2%, p = 0.004), previous surgery (17% vs 1%, p = 0.001) and organ transplantation (6% vs 0%). In rheumatological patients, lung (83% vs 98%, p = 0.0001) and ≥2 organs (6% vs 8%) involvement were less common. Heart (11% vs 0%), sinuses (6% vs 5%) and central nervous system (6% vs 4%) involvement more often developed. In rheumatological patients, respiratory failure (61 vs 37%, p = 0.03), hemoptysis (28% vs 7%, p = 0.0001) and chest pain (17% vs 7%, p = 0, 04) were noted more often, less often - fever ≥380С (67% vs 85%, p = 0.01) and cough (61% vs 70%). CT signs of lung damage were similar in both groups, but rheumatologic patients were more likely to show an «air crescent» sign and / or destruction cavity (44% vs 10%, p = 0.0001). In rheumatologic patients, IA was more often confirmed by isolation ofAspergillusspp. from BAL (80% vs 45%, p = 0.005) and by histological examination (22% vs 7%, p = 0.01). The main pathogens wereA. fumigatus(50% vs 43%),A. niger(29% vs 32%), andA. flavus(14% vs 17%).Rheumatological patients were less likely to receive antifungal therapy 89% vs 99%, p = 0,0003. The main drug in both groups was voriconazole. The overall 12-week survival did not significantly differ between groups, but was lower in rheumatological patients with IA (69% vs 81%).Conclusion:In rheumatological patients, invasive aspergillosis more often developed at an older age, mainly in women. The main background diseases were ANCA-associated vasculitis, granulomatosis with polyangiitis, and systemic lupus erythematosus. Typical risk factors were steroids and immunosuppressants use, prolonged lymphocytopenia, ICU stay, and renal failure. The main causative agents wereA. fumigatus,A. niger, andA. flavus. The main localization of infection were lungs. Respiratory failure, hemoptysis and heart involvement were typical. The overall 12-week survival of rheumatological patients with invasive aspergillosis was 69%.Disclosure of Interests:None declared

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