scholarly journals ANCA-associated vasculitis in a 14 years-old patient: a clinical case

2020 ◽  
Vol 27 (5) ◽  
pp. 184-194
Author(s):  
A. V. Burlutskaya ◽  
N. V. Savelyeva ◽  
N. S. Тaran
Keyword(s):  
Respiratory Failure ◽  
Immunosuppressive Therapy ◽  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pulse Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Nasal Bleeding ◽  
Diagnostic Research

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

scholarly journals POS1230 OUTCOME FOLLOWING COVID-19 INFECTION IN ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 898.2-898
Author(s):  
A. Antovic ◽  
B. Lövström ◽  
A. Hugelius ◽  
O. Borjesson ◽  
A. Bruchfeld ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Data Retrieval ◽  
Induction Treatment ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Upper Airways ◽  
Anca Associated Vasculitis

Background:Patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) require immunosuppressive therapy for disease control and reduction of disease relapse and may be at risk for complications during Sars-CoV-2 (COVID-19) infection.Objectives:To analyze the consequences of COVID-19 in a large cohort of AAV patients regarding occurrence, need of hospitalization, treatment at the intensive care units (ICU), or death.Methods:Data were retrieved from March 2020 to mid-January 2021 from medical records from the AAV cohort (n=233). Patients diagnosed with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) or eosinophilic granulomatosis with polyangiitis (EGPA) were included. Data included age, gender, diagnosis, ongoing immunosuppressive medication at onset of COVID-19 or at last follow-up in non-COVID individuals. Renal involvement (ever) and estimated glomerular filtration rate (eGFR) were included. COVID-19 was confirmed either by a positive PCR test in the upper airways or by serology. Severe COVID-19 was defined as need of non-invasive ventilation, ICU care, and/or death.Results:The cohort comprised of 172 patients with GPA, 50 with MPA and 11 with EGPA. There were 121 females (52%). During the study period, 20 patients (8.6%) were diagnosed with COVID-19. The median age at data retrieval in all patients was 68 years (21-93), in the COVID-19 group 63 (29-93) and 68.5 (21-90) years in the non-COVID patients.Fourty-three patients in all (18%) were hospitalized during the study period of which 11 (4.7%) due to COVID-19 infection. In all, 8 deaths occurred of which 3 were related to COVID-19.At data retrieval, 110 (47%) patients were on prednisolone treatment, 10/20 (50%) in the COVID-19 group and 100 (47%) in the non-COVID-19 group (p=0.5), with significantly higher doses in COVID-19 patients (p<0.001). In patients hospitalized with COVID-19, 6/11 (54.5%) were on prednisolone, median dose 5 mg/day (0-50). In the total group 112 (48%) were on disease modifying anti-rheumatic drugs (DMARD) and 64 (27.5%) on rituximab as maintenance therapy. Eight patients were on induction treatment with either cyclophosphamide or rituximab.Of the 20 COVID-19 cases, 8 had severe COVID-19. Of these, 2 were inactive without immunosuppressive treatment, 4 had stable disease with prednisolone (5-7.5 mg/day) in combination with DMARDs, and 2 were active treated with high dose prednisolone (25-50 mg/day) in combination with cyclophosphamide and rituximab (n=1) or rituximab (n=1).A higher proportion of patients had active AAV (p=0.03) in the severe COVID-19 then in the non-COVID group (10/213 patients).In the group with the severe COVID-19, 1/8 (12%) patient had rituximab as maintenance therapy, compared to 61/213 (28.6%) in the group of non-COVID-19 patients (p=0.5).Renal involvement (ever) was present in 144 patients (62%), in 6 patients (30%) with COVID- 19, from which 5 (62%) were in the group of severe COVID-19 patients. Median eGFR did not differ between severe COVID-19 and remaining patients with renal involvement independently of COVID-19 infection.Conclusion:We found a high rate of severe COVID-19 infection in our cohort of AAV patients which indicates risk for serious complications, especially in patients with active disease and intense immunosuppressive therapy. Maintenance therapy with rituximab did not seem to increase the risk for severe COVID-19. The findings stress the need for continued shielding and early vaccination in AAV patients.Disclosure of Interests:None declared

scholarly journals Granulomatous Inflammation in ANCA-Associated Vasculitis

2021 ◽  
Vol 22 (12) ◽  
pp. 6474
Author(s):  
Antje Müller ◽  
Bettina Krause ◽  
Anja Kerstein-Stähle ◽  
Sara Comdühr ◽  
Sebastian Klapa ◽  
...  
Keyword(s):  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Local Development ◽  
Systemic Vasculitis ◽  
Kidney Tissue ◽  
Granulomatous Inflammation ◽  
Giant Cells ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Factors Affecting ◽  
Anca Associated Vasculitis

ANCA-associated vasculitis (AAV) comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). While systemic vasculitis is a hallmark of all AAV, GPA is characterized by extravascular granulomatous inflammation, preferentially affecting the respiratory tract. The mechanisms underlying the emergence of neutrophilic microabscesses; the appearance of multinucleated giant cells; and subsequent granuloma formation, finally leading to scarred or destroyed tissue in GPA, are still incompletely understood. This review summarizes findings describing the presence and function of molecules and cells contributing to granulomatous inflammation in the respiratory tract and to renal inflammation observed in GPA. In addition, factors affecting or promoting the development of granulomatous inflammation such as microbial infections, the nasal microbiome, and the release of damage-associated molecular patterns (DAMP) are discussed. Further, on the basis of numerous results, we argue that, in situ, various ways of exposure linked with a high number of infiltrating proteinase 3 (PR3)- and myeloperoxidase (MPO)-expressing leukocytes lower the threshold for the presentation of an altered PR3 and possibly also of MPO, provoking the local development of ANCA autoimmune responses, aided by the formation of ectopic lymphoid structures. Although extravascular granulomatous inflammation is unique to GPA, similar molecular and cellular patterns can be found in both the respiratory tract and kidney tissue of GPA and MPA patients; for example, the antimicrobial peptide LL37, CD163+ macrophages, or regulatory T cells. Therefore, we postulate that granulomatous inflammation in GPA or PR3-AAV is intertwined with autoimmune and destructive mechanisms also seen at other sites.

scholarly journals Cutaneous manifestations of ANCA-associated vasculitis and immunosuppressive therapy: cause-effect relationships (a case report)

2020 ◽  
Vol 12 (3) ◽  
pp. 79-84
Author(s):  
Madina A. Kitova ◽  
Maksim V. Maksimov ◽  
Valeriy N. Marchenko ◽  
Elena A. Bruchkus ◽  
Denis A. Davydov
Keyword(s):  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Secondary Infection ◽  
Skin Lesions ◽  
Cutaneous Manifestations ◽  
Multiple Organs ◽  
Anca Associated Vasculitis ◽  
Clinical Triad ◽  
The One

Granulomatosis with polyangiitis, formerly known as Wegeners granulomatosis, is an autoimmune ANCA-associated systemic vasculitis characterized by extensive damage to multiple organs and systems. Besides a typical clinical triad of ENT, lungs, and kidneys injury, various types of skin lesions can be found in 1050% of cases. A severe course of the disease and low survival of patients often requires using aggressive treatment in a form of combined immunosuppressive therapy. On the one hand, it generally improves the prognosis, and on the other is itself associated with numerous complications. One of them is a secondary infection. Skin is the second most common localization of infection after the respiratory system. Preceding skin lesions caused by vasculitis may increase the risk of infection. Thus, patients with ANCA-associated vasculitis should be carefully observed for cutaneous manifestation, both before and during the immunosuppressive therapy.

scholarly journals A rare variant of ANCA-associated eosinophilic granulomatosis with polyangiitis complicated by diffuse alveolar hemorrage

2019 ◽  
Vol 29 (2) ◽  
pp. 229-234
Author(s):  
Anna S. Zaytseva ◽  
Igor' E. Stepanyan ◽  
Irina Yu. Shabalina ◽  
Lyudmila I. Dmitriyeva ◽  
Evgeniy I. Shmelev
Keyword(s):  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Cardiac Injury ◽  
Diffuse Alveolar Hemorrhage ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Elderly Female ◽  
Anca Associated Vasculitis ◽  
Eosinophilic Granulomatosis ◽  
Limited Usefulness

Diagnostic criteria of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) are of limited usefulness as they involve resembling conditions and do not make possible distinguishing nosology of vasculitis. A challenging clinical case of an elderly female patient with ANCA-associated vasculitis, diffuse alveolar hemorrhage syndrome, cardiac injury and blood eosinophilia, but without bronchial asthma, is described in the article.

scholarly journals P091 Can use of high cost drugs for rare rheumatic diseases be reliably identified within routinely collected NHS data? Results from a pilot study of rituximab use in vasculitis using data from the National Disease Registration Service (NDRS) and the Registration of Complex Rare Diseases - Exemplars in Rheumatology (RECORDER) project

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Mithun Chakravorty ◽  
Fiona Pearce ◽  
Megan Rutter ◽  
Peter Lanyon ◽  
Jeanette Aston ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Systemic Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Research Support ◽  
Anca Associated Vasculitis ◽  
Icd 10 ◽  
Eosinophilic Granulomatosis ◽  
Registration Service ◽  
Research Grant

Abstract Background/Aims  Understanding real-world usage of high cost drugs and subsequent outcomes are crucial to support high quality care, adoption of innovation, and reduce unwarranted variation in treatment. Hospital Episode Statistics (HES) contain diagnoses (coded by ICD-10) and procedures/treatment (coded by OPCS) for admissions in England. However, OPCS codes are not specific for individual drugs, for example X921 (cytokine inhibitors band 1) includes rituximab and 15 other drugs. We aimed to validate the accurate identification of patients treated with rituximab for ANCA-associated vasculitis (AAV) using HES data. Methods  We used data available to the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS), part of NDRS at Public Health England and their legal permissions (CAG 10-02(d)/2015). We extracted from HES all patients treated at one hospital who ever had a coded diagnosis of either granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa or 'arteritis unspecified’, which is applied by coders to several clinical terms including “systemic vasculitis” and “ANCA associated vasculitis”. Enabled by data sharing agreements, we reviewed hospital records of those patients who had a Finished Consultant Episode (FCE) during 2018/19, to validate diagnoses and whether X921 reliably identified rituximab. Results  Initially using codes only for granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, or microscopic polyangiitis we identified 65 people, 60 of whom had AAV confirmed in their medical notes (Positive predictive value (PPV) 92.3%; 95% CI 83.0-97.5). When we expanded our search to include in addition people with codes for polyarteritis nodosa or ‘arteritis unspecified’ who had rheumatology or renal follow up, we identified an additional 13 people with AAV and 10 who had other types of systemic vasculitis (PPV for AAV 79.5, 95% CI 69.6-87.4; PPV for systemic vasculitis 90.9%, 95% CI 82.9-96.0). Among patients identified only by the specific ICD-10 codes for the 3 subtypes of AAV there were 51 episodes coded as X921, of which 50 correctly identified a rituximab infusion (and 1 was tocilizumab). No additional infusions for these patients were missed by the coding. The PPV was 98.0% (95% CI 89.6-100). Among patients identified by the more inclusive algorithm, there were 61 episodes coded as X921 in that year. Of these, 59 were for rituximab treatment for AAV and an additional 4 rituximab infusions occurred but were not coded. The PPV was 96.7% (95% CI 88.7-99.6), and the sensitivity was 93.7% (95% CI 84.5-98.2). Conclusion  This pilot study demonstrates how the use of novel algorithms within the NDRS RECORDER project can enable the identification and registration of people with AAV and their high-cost treatment at whole-population level. Further work is now underway to study this national cohort, and to apply this methodology to other rare autoimmune diseases and high cost drugs. Disclosure  M. Chakravorty: None. F. Pearce: Grants/research support; Recipient of research grant from Vifor Phama. Vifor Pharma had no influence on the design, interpretation or reporting of this work. M. Rutter: None. P. Lanyon: Grants/research support; Recipient of research grant from Vifor Phama. Vifor Pharma had no influence on the design, interpretation or reporting of this work. J. Aston: None. M. Bythell: None. S. Stevens: None.

scholarly journals GRANULOMATOSIS WITH POLYANGIITIS (WEGENER): FEATURES OF DIAGNOSIS IN CONDITIONS OF COVID-19 INFECTION (CLINICAL CASE)

2021 ◽  
Vol 19 (3) ◽  
pp. 331-334
Author(s):  
N. V. Hlutkina ◽  
Keyword(s):  
Respiratory Failure ◽  
Otitis Media ◽  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Organ Damage ◽  
Multiple Organ ◽  
State Of Health ◽  
Moderate Severity ◽  
The Given

Background. Multivascular and multiple organ damage in COVID-19 infection poses difficulties in the diagnosis of comorbidities, especially vasculitis. Aim. The goal is to analyze a clinical case of diagnosing granulomatosis with polyangiitis (Wegener) in conditions of COVID-19 infection. Material and methods. An example of the difficulty of diagnosing granulomatosis with polyangiitis (Wegener) in conditions of COVID-19 infection is given. Results. Within three months, the patient received treatment for sinusitis, otitis media. However, against the background of the antibacterial therapy, the state of health did not improve. On the fourth month from the onset of the disease, the patient was admitted to the pulmonology department with a diagnosis of a viral infection caused by SARS Cov-2 (IgM +, IgG-), moderate severity, respiratory failure 0 ?, after the examination, the diagnosis was made: granulomatosis with polyangiitis (Wegener's), acute course, high activity associated with antibodies to Pr-3 (79 n/ml), advanced stage with lesions of ENT organs in the form of exudative otitis media, bilateral mastoiditis. The damage to the lungs in the form of endobronchitis, pulmonary disseminated form (partly with disintegration). respiratory failure 1. Reconvalescent of SARS Cov-2 infection. Conclusions. Based on the given example of this clinical case, the difficulty of diagnosing systemic vasculitis, namely granulomatosis with polyangitis (Wegener's), in conditions of COVID-19 infection is demonstrated.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis Presenting with Myocarditis as an Initial Symptom: A Case Report and Review of the Literature

2021 ◽  
pp. 329-333
Author(s):  
Kanako Kurihara ◽  
Jun Tsugawa ◽  
Shinji Ouma ◽  
Toshiyasu Ogata ◽  
Mikiko Aoki ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Nerve Biopsy ◽  
Pulse Therapy ◽  
Sural Nerve Biopsy ◽  
Lower Limbs ◽  
Rapid Progression ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Initial Symptom ◽  
Steroid Pulse ◽  
Eosinophilic Granulomatosis

A 66-year-old woman with a history of bronchial asthma had shortness of breath and fatigue upon mild exercise. She was diagnosed as congestive heart failure. A blood test showed eosinophilia without the presence of anti-neutrophil cytoplasmic antibody (ANCA), and a myocardial biopsy specimen revealed eosinophilic infiltration in the myocardium. Eosinophilia was improved when she was administered short-term methylprednisolone. After that, she had numbness and pain in her lower limbs with re-elevation of eosinophils. She had dysesthesia and hypalgesia in the distal part of the limbs. Sural nerve biopsy revealed axonal degeneration and thickness of the arterial wall, indicating a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Two courses of steroid pulse therapy were performed, resulting in marked improvement of her sensory symptoms. ANCA-negative EGPA might be associated with myocarditis and peripheral neuropathy. A sufficient immunotherapy should have been considered to prevent rapid progression.

scholarly journals POS0830 FACTORS INFLUENCING PATIENT-REPORTED OUTCOMES IN ANCA-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 668.2-669
Author(s):  
S. Monti ◽  
P. Delvino ◽  
C. Klersy ◽  
G. Coppa ◽  
A. Milanesi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Patient Reported Outcomes ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Damage Accrual ◽  
Patient Reported

Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared

scholarly journals THU0300 RISK FACTORS FOR COMPLICATIONS AND REFRACTORY COURSE IN PATIENTS WITH ANCA-ASSOCIATED SYSTEMIC VASCULITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 378.2-378
Author(s):  
A. Chudinov ◽  
I. Belyaeva ◽  
M. Pervakova ◽  
V. Mazurov ◽  
O. Inamova ◽  
...  
Keyword(s):  
Risk Factors ◽  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Induction Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Disease Risk ◽  
Systemic Vasculitis ◽  
Cardiovascular Complications ◽  
Infectious Complications ◽  
Eosinophilic Granulomatosis With Polyangiitis

Background:ANCA-associated systemic vasculitis (AAV) is characterized by a high incidence of complications and high mortality. The most significant complications during the first 3 years of the disease are infectious and cardiovascular. Development of chronic kidney disease also impairs the prognosis of AAV. Refractory to induction therapy can significantly increase the severity of organ lesions in patients with AAV.Objectives:The aim of this study was to determine risk factors for complications and refractory course in patients with AAV.Methods:Patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) were observed during the first 3 years of the disease and included in this study between 2010 and 2018. Most common infectious complications requiring inpatient treatment were pneumonia, mycosis, sepsis, purulent arthritis, purulent otitis media. Cardiovascular complications included pulmonary thromboembolism, myocardial infarction, ischemic stroke, venous thrombosis.Results:In total 209 (165 [79%] female and mean age 51.8 ± 13.2 years) AAV patients (94 GPA; 46 MPA; and 69 EGPA) were included in the analysis. Risk factors for infectious complications were BVAS level at the beginning of induction therapy > 25 (OR – 2.92, 95% CI (1.53;5.45) p<0.001), usage of prednisone in doses more than 60 mg / day at the induction of remission (OR – 2.76, 95% CI (1.45;5.29) p=0.003), usage of prednisone in doses ≥ 10 mg / day after 6 months of induction therapy (OR – 2.60, 95% CI (1.38;4.93) p=0.003), ANCA-PR3 positivity (OR – 2.25, 95% CI (1.13;4.46) p=0.017) and presence of diabetes mellitus in the AAV onset (OR – 1.77, 95% CI (1.14;3.45) p=0.038). Patients with AAV had following risk factors for cardiovascular complications: male (OR – 2.28, 95% CI (1.33;3.88) p=0.002), BVAS level > 25 (OR – 2.1, 95% CI (1.11;3.16) p=0.008) and presence of coronary artery disease in the AAV onset (OR – 2.2, 95% CI (1.18;4.10) p=0.015). ANCA positivity (OR – 5.62, 95% CI (2.1;14.9) p<0.001), presence of rapidly progressive glomerulonephritis in the first 3 months from onset AAV (OR – 5.02, 95% CI (3.42;7.35) p<0.001) and over 60 years of age (OR – 2.17, 95% CI (1.38;3.44) p=0.001) were risk factors of development of chronic kidney disease. Risk factors for refractory to induction therapy in patients with AAV were ANCA-PR3 positivity (OR – 3.13, 95% CI (1.63;6.02) p<0.001), BVAS level > 25 (OR – 2.63, 95% CI (1.74;4.34) p<0.001), initiation of therapy after 4 months from the onset of clinical manifestations (OR – 2.17, 95% CI (1.26;3.91) p=0.005). We additionally defined that identification of pathological phenotypes of alpha-1-antitrypsin was risk factors for refractory course in patients with GPA manifestations (OR – 2.66, 95% CI (1.12;6.33) p=0.048).Conclusion:Our study has shown that high disease activity, ANCA positivity and comorbid pathology increase risk of serious complications. Early administration of immunosuppressive therapy, adequate steroid dosing and use of risk factors for complications and refractory course in clinical practice can significantly improve the prognosis of AAV.Disclosure of Interests:None declared

Systemic Vasculitis Syndromes

2017 ◽  
Author(s):  
Alexandra Villa-Forte ◽  
Brian F Mandell
Keyword(s):  
Kawasaki Disease ◽  
Blood Vessels ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Plain Radiograph ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Saddle Nose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Saddle Nose Deformity

Vasculitis is defined by histologic evidence of inflammation that involves the blood vessels. The diagnosis of a specific primary vasculitic disorder depends on the pattern of organ involvement, the histopathology, the size of affected blood vessels, and the exclusion of diseases that can cause “secondary” vasculitis. This review presents an approach to the patient suspected of having vasculitis, and goes on to discuss small vessel vasculitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, and Behçet disease. Figures show classification of the systemic vasculitis syndromes, the relationships among the causes of small vessel (“hypersensitivity”) vasculitis, palpable purpura of the distal extremities, saddle nose deformity, the nodular infiltrates of the lung in granulomatosis with polyangiitis shown on plain radiograph as well as computed tomography, necrotizing scleritis, livedo reticularis, and angiograms of a patient with Takayasu arteritis. Tables list selected laboratory tests for patients with multisystem disease and possible vasculitis, practical comments on immunosuppressive therapies for vasculitis, features of vasculitis, diagnostic criteria for Kawasaki disease, and giant cell arteritis. This review contains 8 highly rendered figures, 5 tables, and 59 references.

Sign in / Sign up

Export Citation Format

Share Document