scholarly journals P186 Efficacy and safety of rituximab in the treatment of systemic vasculitis: experience at a single centre

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Carl Orr ◽  
Ayna Verdiyeva ◽  
Nadia Ahmad ◽  
Hairulhadi Ariff ◽  
Raashid Luqmani
Keyword(s):  
Systemic Vasculitis ◽  
Demographic Data ◽  
Underlying Disease ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Chimeric Antibody ◽  
Efficacy And Safety ◽  
Electronic Patient Records ◽  
Patient Demographic Data ◽  
Age Profile

Abstract Background Rituximab is a monoclonal chimeric antibody targeting the mature B cell molecule CD20, expressed on the cell surface. It is licenced and widely used in the treatment of autoimmune diseases, including the ANCA associated vasculitides and rheumatoid arthritis. The efficacy and safety of rituximab in rare diseases requires ongoing appraisal. Methods We interrogated the records of 62 consecutive patients treated with rituximab for small vessel systemic vasculitis at our unit over the last 3 years, using a contemporaneously maintained central database, as well as individual electronic patient records. Results The patient demographic data, and main results are presented in Table 1. 31 (50%) of the patients treated with rituximab were female, and the indication for treatment in most cases was ANCA associated vasculitis (77.4%). After a mean follow up period of 2.04 years, the mean reduction in BVAS was 1.64 (3.83). 5 severe adverse events were recorded, defined as death or hospitalisation potentially related to treatment with rituximab. 3 patients died of respiratory sepsis, 1 experienced a wound infection, and 1 experienced an ischaemic colon. Four patients were noted to have low immunoglobulins. Conclusion Rituximab is an effective treatment for many patients with systemic, small vessel vasculitis. Understanding the safety profile of this drug in the context of our cohort is challenging, given the significant morbidity associated with the underlying disease, as well as the age profile and concomitant immunosuppressive medications. Continued surveillance regarding efficacy and safety of rituximab in the small vessel systemic vasculitides remains invaluable. Disclosures C. Orr None. A. Verdiyeva None. N. Ahmad None. H. Ariff None. R. Luqmani None.

scholarly journals Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review

Pathogens ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 31
Author(s):  
Céline Betti ◽  
Pietro Camozzi ◽  
Viola Gennaro ◽  
Mario G. Bianchetti ◽  
Martin Scoglio ◽  
...  
Keyword(s):  
Mycoplasma Pneumoniae ◽  
Legionella Pneumophila ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Bacterial Pathogen ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Systemic Involvement ◽  
Symptomatic Disease ◽  
Atypical Pathogen

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.

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.

scholarly journals The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis

Antibodies ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 31 ◽  
Author(s):  
Elena Csernok
Keyword(s):  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Urticarial Vasculitis ◽  
International Consensus ◽  
Anca Associated Vasculitis ◽  
Distinct Disease ◽  
A Current ◽  
Made In

Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture’s syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. The revised 2017 international consensus recommendations for ANCA detection support the primary use PR3- and MPO-ANCA immunoassays without the categorical need for additional indirect immunofluorescence (IIF). Interestingly, the presence of PR3- and MPO-ANCA have led to the differentiation of distinct disease phenotype of AAV: PR3-ANCA-associated vasculitis (PR3-AAV), MPO-ANCA-associated vasculitis (MPO-AAV), and ANCA-negative vasculitis. Further studies on the role of these autoantibodies are required to better categorize and manage appropriately the patients with small-vessel vasculitis and to develop more targeted therapy.

Seroepidemiological Study of Hepatitis A in Medical Students of Mazandaran University of Medical Sciences in 2019

2021 ◽  
Author(s):  
Saman Shirazinia ◽  
Navid Reza Shayan ◽  
Negin Ghiyasi Moghaddam ◽  
Nima Ameli ◽  
Shahriar Alian ◽  
...  
Keyword(s):  
Medical Students ◽  
Hepatitis A ◽  
Demographic Data ◽  
Underlying Disease ◽  
Medical Sciences ◽  
Cross Sectional ◽  
Vaccination History ◽  
Previous Infection ◽  
Patient Demographic Data ◽  
History Of

Introduction: Hepatitis A (HA) is a common infectious disease caused by the HA virus that primarily affects the liver. We need to determine the safety status and infection rate in the community. Therefore, we decided to study the Seroepidemiological of HA in medical students of Mazandaran University of Medical Sciences in 2019.Material and Methods: This was a cross-sectional descriptive-analytical study prospectively examining anti-HAV antibodies in medical students of Mazandaran University of Medical Sciences in 2019. Patient demographic data, laboratory results of anti-HAV IgG and vaccination history were recorded. Four cc of blood were drawn from each student for the anti-HAV IgG test and after the test was performed. Data were analyzed using SPSS 24 software.Results: One hundred and fifty-five students were interviewed. The mean age (standard deviation) of the students in this study was 23.81 (1.47) years. Ninety-nine (63.9%) of the students were male. Therefore, it was caused by previous infection with HAV and 120 (77.4%) cases were considered susceptible individuals whose serum anti-HAV IgG was negative. This was not seen to be statistically significant (P=0.754). According to the test, 77.4% of the predictions were correct. None of the variables of sex, age, history of underlying disease, place of residence, and history of travel had a significant effect on the incidence and positivity of anti-HAV IgG.Conclusion: This study showed that the incidence of anti-HAV IgG was positive in 22.6% of the students, which is much lower than previous studies. This may be due to the higher level of health in this segment of the population.

scholarly journals AB0098 ASSOCIATED FACTORS IN ONE-YEAR MORTALITY IN PATIENTS WITH SMALL VESSEL SYSTEMIC VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1078.2-1078
Author(s):  
E. M. Escudero Tepale ◽  
M. A. Saavedra ◽  
D. Miranda ◽  
Z. Castro ◽  
M. J. R. Arriga Torres
Keyword(s):  
Associated Factors ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Remission Induction ◽  
First Year ◽  
Factors Associated ◽  
Number Of Patients ◽  
One Year

Background:The highest mortality rates in patients with small vessel systemic vasculitis occur within the first year after the diagnosis, however associated factors have been scarcely studied in our population.Objectives:To identify mortality associated factors at the time of diagnosis in patients with small vessel systemic vasculitis.Methods:Retrospective cohort (2009-2020) involving 81 patients diagnosed with systemic small vessel vasculitis. Demographic, clinical and biochemical parameters were studied as potential factors associated with one-year mortality.Results:Of the total of patients (n=81), 36 (44.4%) had generalized granulomatosis with polyangiitis, 32 (39.5%) had localized granulomatosis with polyangiitis and 5 (6.2%) had early systemic granulomatosis with polyangiitis, 7 (8.6%) had microscopic polyangiitis and 1 (1.25%) had eosinophilic granulomatosis with polyangiitis. Twenty-two deaths (27%) were observed, 14 of them (63.6%) happened within the first year of diagnosis. The leading cause of death was infection (64%). Patients who died within the first year of diagnosis had a higher frequency of hypoalbuminemia (p=0.05) and also presented hemoglobin lower than 10.8 g/dL (p=0.035) in comparison with those who died after the first year of diagnosis. Remission induction treatment did not differ between both groups.Conclusion:Our study suggests that hypoalbuminemia and anemia are factors associated with a higher mortality within the first year after the diagnosis in patients with systemic small vessel vasculitis which contrast with previously reported data. The study design and the reduced number of patients are two major limitations of the study.References:[1]Flossmann O, Berden A, Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70:488-94.Disclosure of Interests:None declared

scholarly journals Henoch-Schonlein Purpura in Children: The Role of Corticosteroids

2019 ◽  
Vol 7 (11) ◽  
pp. 1812-1814
Author(s):  
Bella Kurnia
Keyword(s):  
Abdominal Pain ◽  
Renal Disease ◽  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Henoch Schonlein Purpura ◽  
Progression Of Renal Disease ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

BACKGROUND: Henoch- schonlein purpura (HSP) is an IgA- mediated systemic small vessel vasculitis. It is the most common form of systemic vasculitis in children.CASE REPORT: A 9 years old girl admitted to the hospital with chief complain of purplish red rash on both legs since approximately 1 week with painful knees and ankles that make the patient unable to walk. The patient was diagnosed with HSP and was treated with corticosteroid and analgesics. The patients only stayed for 2 nights at the hospital and discharged from the hospital with the ability to walk and experience no pain. CONCLUSION: The role of corticosteroids in the treatment of HSP is still controversial. But from various research, we can conclude that the role of corticosteroid in HSP is as a symptom reliever (reduce abdominal pain and arthritis), but does not slow the progression of renal disease.

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.

scholarly journals Antineutrophil cytoplasm antibodies directed against myeloperoxidase augment leukocyte-microvascular interactions in vivo

Blood ◽  
2005 ◽  
Vol 106 (6) ◽  
pp. 2050-2058 ◽  
Author(s):  
Mark A. Little ◽  
C. Lucy Smyth ◽  
Rashmi Yadav ◽  
Lyn Ambrose ◽  
H. Terence Cook ◽  
...  
Keyword(s):  
Leukocyte Adhesion ◽  
Systemic Vasculitis ◽  
Potential Effect ◽  
Passive Transfer ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Mesenteric Hemorrhage ◽  
Experimental Autoimmune

Abstract Systemic small vessel vasculitis is associated with antineutrophil cytoplasm antibodies (ANCAs). While there is mounting in vitro evidence to suggest that ANCAs are capable of enhancing leukocyte-endothelial interactions, no in vivo evidence for this has been provided. In this study a novel rat model of ANCA-associated experimental autoimmune vasculitis (EAV), induced by immunization with human myeloperoxidase (MPO), was used to analyze directly the potential effect of ANCAs on leukocyte-venular wall interactions in vivo as observed by intravital microscopy. These rats developed anti-MPO antibodies directed against rat leukocytes, showed pathologic evidence of small vessel vasculitis, and had enhanced leukocyte adhesion and transmigration in response to the chemokine Groα (CXCL1 [CXC ligand 1]). Passive transfer of immunoglobulin from rats with EAV to naive rats conferred enhanced adhesion and transmigration responses in the recipients. Furthermore, rats with EAV and recipients of ANCA-positive immunoglobulin developed extensive microvascular injury, as manifested by mesenteric hemorrhage, in response to CXCL1. This study provides the first direct in vivo evidence for the ability of ANCAs to enhance leukocyteendothelial interactions and cause microvascular hemorrhage, thereby providing a mechanism by which ANCAs could exert pathogenic effects in systemic vasculitis. (Blood. 2005;106:2050-2058)

scholarly journals Patient with Antineutrophil Cytoplasmic Antibody Associated Small Vessel Vasculitis, Acute Renal Failure, and Coronavirus Disease-19 Pneumonia: A Diagnostic and Therapeutic Challenge

2020 ◽  
Vol 8 (T1) ◽  
pp. 542-547
Author(s):  
Biljana Gerasimovska-Kitanovska ◽  
Gjulshen Selim ◽  
Zvezdana Petronijevik ◽  
Blagica Pecanova ◽  
Gjorgi Stojchev ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Pulmonary Hemorrhage ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Necrotizing Glomerulonephritis ◽  
Pulmonary Renal Syndrome

BACKGROUND: Antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV) has a predilection for the kidney and more than three quarters of patients have renal involvement with rapidly progressive glomerulonephritis. Small-vessel systemic vasculitis may present as pulmonary-renal syndrome and is characterized by necrotizing glomerulonephritis and pulmonary hemorrhage. Diagnosis and therapy for AAV in coronavirus disease (COVID) COVID-19 pandemic require multi-disciplinary collaboration due to the affection of multiple systems and risks associated with immunosuppressive medications. CASE REPORT: A 69-year-old non-smoker, non-diabetic female presented in the outpatient unit at the department of pulmonology with dry cough, malaise, and sub-febrile temperature, lasting for 1 month. The patient had a high suspicion of severe pulmonary-renal syndrome, ANCA-AAV, and acute renal failure requiring hemodialysis. She was treated with corticosteroids, cyclophosphamide, and plasma exchange. The treatment led to temporary improvement. Infections with COVID-19, Enterococcus in the urine, and Acinetobacter in the tracheal aspirate further complicated the clinical picture and despite antibiotic treatment, use of tocilizumab and convalescent plasma, the outcome was lethal. CONCLUSION: It is important to establish the diagnosis and distinguish accurately between vasculitis and infection to provide adequate and timely therapy.

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