scholarly journals A case of vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis: a case report

2022 ◽  
Vol 23 (1) ◽  
Author(s):  
Hanui Park ◽  
Miji Lee ◽  
Jin Seon Jeong
Keyword(s):  
Infective Endocarditis ◽  
Clinical Features ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Immunoglobulin A ◽  
Maintenance Hemodialysis ◽  
Iga Vasculitis ◽  
Pharmacological Agents ◽  
Case Presentation ◽  
First Case

Abstract Background Immunoglobulin A vasculitis (IgA vasculitis) is one of the most common forms of vasculitis in children. It rarely occurs in adults. It is a systemic vasculitis with IgA deposition and is characterized by the classical tetrad of purpura, arthritis/arthralgia, gastrointestinal and renal involvement. Certain types of infections, and pharmacological agents have been reported to be associated with IgA vasculitis. Here, we describe a case of IgA vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis. Case presentation A 70-year-old man undergoing hemodialysis was admitted because of skin purpura, abdominal pain, diarrhea, and lower back pain. We suspected him as IgA vasculitis based on the clinical features and skin biopsy findings. Transesophageal echocardiography revealed infective endocarditis, which predisposed him to IgA vasculitis. He was treated with antibiotics and low-dose corticosteroids, which led to resolution of vasculitis. Conclusions This is the first case of IgA vasculitis triggered by infective endocarditis in a patient undergoing hemodialysis. Patients undergoing hemodialysis are at a high risk of infection because of immune dysfunction and frequent venipuncture. The incidence of infective endocarditis associated with IgA vasculitis is very low, but it has been repeatedly reported. Therefore, it is necessary to consider infective endocarditis in patients with clinical features that indicate IgA vasculitis.

scholarly journals Pediatric Granulomatosis With Polyangiitis Mimicking IgA Vasculitis: A Case Report

2020 ◽  
Vol 13 ◽  
pp. 117954412096737
Author(s):  
Vadood Javadi Parvaneh ◽  
Arezoo Shirzani ◽  
Khosro Rahmani ◽  
Reza Shiari
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
High Titer ◽  
Renal Involvement ◽  
Iga Vasculitis ◽  
Case Presentation ◽  
Tissue Biopsy ◽  
Anca Associated Vasculitis ◽  
Wide Range ◽  
Time Of Presentation

Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.

scholarly journals Leflunomide therapy for IgA vasculitis with nephritis in children

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Ling Hou ◽  
Zhou Zhang ◽  
Yue Du
Keyword(s):  
Pediatric Patients ◽  
Systemic Vasculitis ◽  
Immunoglobulin A ◽  
Case Series ◽  
International Study ◽  
Immunosuppressive Agent ◽  
Iga Vasculitis ◽  
Intravenous Steroid ◽  
First Case ◽  
Schonlein Purpura

Abstract Background Henoch-Schönlein purpura (HSP), also called IgA vasculitis, is a systemic vasculitis characterized by deposits of immunoglobulin A in blood vessels. Renal impairment of these patients is the main determinant of prognosis. The optimal treatment of HSP nephritis (HSPN) in children remains controversial, but many clinicians administer an immunosuppressive agent with a corticosteroid. A previous study reported that leflunomide (LEF) with a corticosteroid was effective for adult patients with HSPN and nephrotic proteinuria. However, data on this treatment in pediatric patients is limited. Methods We described our experience at a single center on the use of LEF in 5 pediatric patients who had IgA vasculitis with proteinuria that was nearly 50 mg/kg (nephrotic range) and remained high despite administration of intravenous steroid, and biopsy-proven nephritis. All patients had class II to IIIb lesions based on the International Study of Kidney Disease in Children (ISKDC). Results We successfully treated all 5 children who had IgA vasculitis with nephritis using LEF with a corticosteroid. Four patients achieved a complete remission of proteinuria, and 1 patient had significantly reduced proteinuria. The children received LEF for 6 months to 12 months, and none of them had severe adverse events. Conclusions To our knowledge, this is the first case series to report successful treatment of pediatric HSPN with LEF in combination with a corticosteroid.

scholarly journals Rivaroxaban-related acute kidney injury in a patient with IgA vasculitis

2019 ◽  
Vol 12 (1) ◽  
pp. e227756 ◽  
Author(s):  
Yoshihiko Fujino ◽  
Chisato Takahashi ◽  
Kensuke Mitsumoto ◽  
Takashi Uzu
Keyword(s):  
Atrial Fibrillation ◽  
Acute Kidney Injury ◽  
Renal Function ◽  
Systemic Vasculitis ◽  
Kidney Injury ◽  
Close Monitoring ◽  
Iga Vasculitis ◽  
Direct Oral Anticoagulant ◽  
Macroscopic Haematuria ◽  
First Case

Anticoagulants have recently been recognised as a cause of acute kidney injury (AKI). We describe the case of a 75-year-old man with IgA vasculitis and atrial fibrillation treated with rivaroxaban, who presented with macroscopic haematuria and an acute decline in renal function. Two months before referral, he noted palpable purpuric lesions and was diagnosed with IgA vasculitis based on skin biopsy findings; the skin lesion disappeared following treatment with a steroid external preparation. Renal biopsy revealed glomerular haemorrhage and red blood cell casts. Although rivaroxaban was withdrawn, his kidney function worsened and he was started on haemodialysis. His renal function did not recover. To the best of our knowledge, this is the first case of direct oral anticoagulant (DOAC)-related AKI in systemic vasculitis. During DOAC therapy, close monitoring of a patient’s urinalysis results and their renal function may be required for patients with systemic vasculitis to avoid AKI.

scholarly journals IgA Vasculitis and COVID-19 in Children: A Systematic Review

2021 ◽  
Author(s):  
Eva Maria Glenn Lecea ◽  
Daniela Aguilar Abisad ◽  
Ana Paredes ◽  
Arielle Hay
Keyword(s):  
Systematic Review ◽  
Causality Assessment ◽  
Case Reports ◽  
Meta Analysis ◽  
Renal Involvement ◽  
Immunoglobulin A ◽  
Case Series ◽  
Iga Vasculitis ◽  
Female Ratio ◽  
Inflammatory Conditions

Abstract Background: Immunoglobulin A vasculitis is the most common form of vasculitis in children. The diagnosis is made clinically and patients will present with a rash, together with gastrointestinal, musculoskeletal, and renal system involvement. Progress in the classification of the systemic vasculitides has facilitated better understanding of the pathogenesis underlying these inflammatory conditions. Over the past year, several cases of IgA vasculitis have been reported in both children and adults in association with SARS-CoV2 infection, raising the question of whether there is any causal or even a synergistic association.Methods: This systematic review was performed following the guidelines of Meta-analysis of Observational Studies in Epidemiology. A literature search was conducted using MEDLINE, SciELO and Google Scholar using the search terms “COVID-19” or “SARS-CoV-2" in combination with “IgA vasculitis”, or “Henoch-Schonlein Purpura”. We considered articles to be eligible for inclusion if they reported a case report or series of cases of IgA vasculitis associated with proven COVID-19 infection. We excluded cases from further review if the case reported was a patient older than 18 years. WHO causality assessment categories were used to standardize case causality. Results: After reviewing the complete article and applying our exclusion criteria, 12 articles describing 12 cases of COVID-19 associated IgA vasculitis in children were included. In 83% of the cases the diagnosis of COVID-19 was made on presentation of IgA symptoms or on presentation to seek medical care. In 17% of cases the SARS Cov-2 test was positive before IgA vasculitis symptoms presentation. The mean age of the patients was 7.3 years of age (SD ±4.8). Male to female ratio was 3:1. Lower extremity purpura was present in all 12 patients. Gastrointestinal manifestations were present in 7 patients. Oligoarthritis was present in 7 patients. Three patients presented renal involvement with hematuria/proteinuria. Conclusions: During the pandemic, several autoimmune phenomena have been described to co-occur with or following COVID-19. The exact role of COVID-19 in the development of these IgA-related diseases is still being explored. Our review of case series and case reports with standardized causality assessment identified 12 cases of IgA vasculitis associated with COVID-19 infection in children.

Immunoglobulin A Vasculitis Complicated with Clostridium difficile Infection: a Rare Case Report and Brief Review of the Literature

2016 ◽  
Vol 25 (2) ◽  
pp. 235-238 ◽  
Author(s):  
Camelia Cojocariu ◽  
Carol Stanciu ◽  
Codrina Ancuta ◽  
Mihai Danciu ◽  
Stefan Chiriac ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Clostridium Difficile ◽  
Clostridium Difficile Infection ◽  
Rare Complication ◽  
Renal Involvement ◽  
Immunoglobulin A ◽  
Corticosteroid Treatment ◽  
Watery Diarrhea ◽  
Iga Vasculitis ◽  
Cutaneous Lesions

Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is a leukocytoclastic type of vasculitis affecting small vessels with a deposition of immune IgA complexes, clinically characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthralgia (or arthritis), and gastrointestinal and renal involvement. Although the cause of the disease remains unknown, immune complexes of IgA and unidentified antigens seem to play a central pathogenic role. The diagnosis is easily established in the presence of purpura, but may be challenging in its absence, especially when colicky abdominal pain precedes the cutaneous lesions. IgA vasculitis is usually a self-limited disease with a benign course and symptomatic treatment is sufficient for most; in severe cases, however, corticosteroids are necessary.We describe the case of a young adult male presenting with severe abdominal pain, vomiting and fever (38.4ºC). Clinical examination, abdominal ultrasound and plain abdominal radiography excluded an acute abdomen. The occurrence of arthralgia involving both knees and erosive duodenitis at endoscopy, 48 hours upon admission, suggested the diagnosis of IgA vasculitis, confirmed on the following day by the presence of typical purpuric rash on the lower extremities. Corticosteroid therapy led to the resolution of all gastrointestinal and joint manifestations as well as to a significant improvement of cutaneous purpura. However, during the 3rd week of corticosteroid treatment, the patient developed watery diarrhea and the clinical suspicion of Clostridium difficile infection (CDI) was confirmed. The treatment with metronidazole led to the resolution of diarrhea.The peculiarity of this case resides in several aspects: the gastrointestinal and joint manifestations preceded purpura, making diagnosis more difficult; CDI is an extremely rare complication of IgA vasculitis, being, in fact, the second case reported in adults in the literature.Abbreviations: CDI: Clostridium difficile infection; CRP: C-reactive protein; EGD: esophagogastroduodenoscopy; ESR: erythrocyte sedimentation rate; IgA: immunoglobulin A; WBC: white blood cell.

scholarly journals SEVERE GASTROINTESTINAL MANIFESTATIONS COMPLICATING HENOCH-SCHÖNLEIN PURPURA IN ADULT: A RARE CASE IN INDONESIA

2018 ◽  
Vol 11 (11) ◽  
pp. 11
Author(s):  
William Ray Cassidy ◽  
Ketut Suryana ◽  
Dewi Catur Wulandari
Keyword(s):  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Immunoglobulin A ◽  
Henoch Schonlein Purpura ◽  
Gastrointestinal Manifestations ◽  
Patient Morbidity ◽  
Schonlein Purpura ◽  
Term Monitoring ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP), also known as immunoglobulin A vasculitis, is a self-limiting, small vessel systemic vasculitis rarely found in adults characterized by palpable purpura, arthritis or arthralgia, gastrointestinal, and renal involvement. We report a case of a 31-year-old male with HSP complicated with severe gastrointestinal manifestations mimicking acute abdomen. He was successfully managed with conservative approach. Meticulous diagnosis, treatment, and long-term monitoring are important to reduce patient morbidity.

scholarly journals IgA Vasculitis Complicated by Both CMV Reactivation and Tuberculosis

2021 ◽  
Vol 13 (3) ◽  
pp. 416-420
Author(s):  
Małgorzata Mizerska-Wasiak ◽  
Maria Winiarska ◽  
Karolina Nogal ◽  
Karolina Cichoń-Kawa ◽  
Małgorzata Pańczyk-Tomaszewska ◽  
...  
Keyword(s):  
Renal Function ◽  
Systemic Vasculitis ◽  
Pediatric Population ◽  
Immunoglobulin A ◽  
Antimicrobial Treatment ◽  
Iga Vasculitis ◽  
Cmv Infection ◽  
Mycobacterium Tuberculosis Infection ◽  
Complicated Course ◽  
Cmv Reactivation

Immunoglobulin A (IgA) vasculitis is the most common systemic vasculitis in the pediatric population. We present the case of a patient with IgA vasculitis with nephritis who developed cytomegalovirus (CMV) infection followed by Mycobacterium tuberculosis infection. In the literature, there are a few cases of IgA nephropathy accompanied by reactivation of CMV or tuberculosis. To the best of our knowledge, this is the first reported case of IgA vasculitis complicated by both CMV reactivation and tuberculosis. It is important to detect infections in patients with IgA vasculitis because they can induce and exacerbate the symptoms of the disease. Effective antimicrobial treatment facilitates the management of proteinuria and slows down the decline of renal function. Immunosuppressive therapy is a risk factor for reactivation of latent infections and makes patients more susceptible to its generalized and complicated course. This can be prevented by actively screening for hidden sites of infection.

scholarly journals Mixed Cryoglobulinaemia Vasculitis after Persistent Hepatitis C Virus Eradication

2021 ◽  
Author(s):  
Margarida Gaudêncio ◽  
Rui Nogueira Rui Nogueira ◽  
Nuno Afonso Oliveira Nuno Afonso Oliveira
Keyword(s):  
Case Report ◽  
Hepatitis C Virus ◽  
Hepatitis C ◽  
Interferon Therapy ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Medium Size ◽  
Virus Eradication ◽  
First Case ◽  
Mixed Cryoglobulinaemia

Mixed cryoglobulinaemia vasculitis (MCV) is a systemic vasculitis of the small and medium-size vessels caused by active hepatitis C (HCV) infection in >80% of cases. Beuthien et al. presented the first case of MCV with undetectable HCV after 10 months of therapy. In the last few years, more authors have described other cases of symptomatic MCV after 1 year of persistent HCV eradication. Here, we present a case report of a 57-year-old man who developed MCV with renal involvement after 3 years of HCV eradication with interferon therapy.

scholarly journals OP0038 SPLICEOSOME ALTERATIONS IN LEUCOCYTES FROM APS, SLE AND SLE+APS PATIENTS ARE CLOSELY RELATED TO THEIR MAIN CLINICAL FEATURES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 20.2-20
Author(s):  
A. M. Patiño-Trives ◽  
C. Perez-Sanchez ◽  
A. Ibañez-Costa ◽  
P. S. Laura ◽  
M. Luque-Tévar ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Immune Cells ◽  
Inflammatory Mediators ◽  
Clustering Analysis ◽  
Renal Involvement ◽  
Molecular Clustering ◽  
Cluster 2 ◽  
Splicing Machinery

Background:To date, although multiple molecular approaches have illustrated the various aspects of Primary Antiphospholipid Syndrome (APS), systemic lupus erythematosus (SLE) and antiphospholipid syndrome plus lupus (APS plus SLE), no study has so far fully characterized the potential role of posttranscriptional regulatory mechanisms such as the alternative splicing.Objectives:To identify shared and differential changes in the splicing machinery of immune cells from APS, SLE and APS plus SLE patients, and their involvement in the activity and clinical profile of these autoimmune disorders.Methods:Monocytes, lymphocytes and neutrophils from 80 patients (22 APS, 35 SLE and 23 APS plus SLE) and 50 healthy donors (HD) were purified by immunomagnetic selection. Then, selected elements of the splicing machinery were evaluated using a microfluidic qPCR array (Fluidigm). In parallel, extensive clinical/serological evaluation was performed, comprising disease activity, thrombosis and renal involvement, along with autoantibodies, acute phase reactants, complement and inflammatory molecules. Molecular clustering analyses and correlation/association studies were developed.Results:Patients with primary APS, SLE and APS plus SLE displayed significant and specific alterations in the splicing machinery components in comparison with HD, that were further specific for each leukocyte subset. Besides, these alterations were associated with distinctive clinical features.Hence, in APS, clustering analysis allowed to identify two sets of patients representing different molecular profile groups with respect to the expression levels of splicing machinery components. Principal component analyses confirmed a clear separation between patients. Clinically, cluster 1 characterized patients with higher thrombotic episodes and recurrences than cluster 2 and displayed a higher adjusted global APS score (aGAPSS). Accordingly, these patients showed higher levels of inflammatory mediators than cluster 2.Similarly, in patients with APS plus SLE, clustering analysis allowed to identify two sets of patients showing differential expression of splicing machinery components. Clinical and laboratory profiles showed that cluster 2 characterized patients that had suffered more thrombotic recurrences, most of them displaying an aGAPSS over 12 points and expressing higher levels of inflammatory mediators than cluster 1. The incidence of lupus nephropathy was similarly represented in both clusters.Lastly, in SLE patients, molecular clustering analysis identified two sets of patients showing distinctive clinical features. One cluster characterized most of the patients positive for anti-dsDNA antibodies, further suffering lupus nephropathy, and a high proportion of them also presenting atheroma plaques and high levels of inflammatory mediators.Correlation studies further demonstrated that several deranged splicing machinery components in immune cells (i.e. SF3B1tv1, PTBP1, PRP8 and RBM17) were linked to the autoimmune profile of the three autoimmune diseases, albeit in a specific way on each disorder. Accordingly, in vitro treatment of HD lymphocytes with aPL-IgG or anti-dsDNA-IgG changed the expression of spliceosome components also found altered in vivo in the three autoimmune diseases. Finally, the induced over/downregulated expression of selected spliceosome components in leukocytes modulated the expression of inflammatory cytokines, changed the procoagulant/adhesion activities of monocytes and regulated NETosis in neutrophils.Conclusion:1) The splicing machinery, profoundly altered in leukocytes from APS, APS plus SLE and SLE patients, is closely related to the activity of these diseases, their autoimmune and inflammatory profiles. 2) The analysis of the splicing machinery allows the segregation of APS, APS plus SLE and SLE, with specific components explaining the CV risk and renal involvement in these highly related autoimmune disorders.Acknowledgements:Funded by ISCIII, PI18/00837 and RIER RD16/0012/0015 co-funded with FEDERDisclosure of Interests:None declared

scholarly journals Immediate post partum macular subretinal bleeding in a highly myopic patient: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Karen Bitton ◽  
J.-L. Bacquet ◽  
F. Amoroso ◽  
S. Mrejen ◽  
M. Paques ◽  
...  
Keyword(s):  
Valsalva Maneuver ◽  
Post Partum ◽  
Pathologic Myopia ◽  
Pathological Myopia ◽  
Case Presentation ◽  
Sudden Loss ◽  
Corrected Visual Acuity ◽  
First Case ◽  
History Of ◽  
Macular Hemorrhage

Abstract Background Pathologic myopia is a major cause of visual impairment and blindness. Case presentation We report a case of an immediate post partum macular subretinal bleeding observed in a highly myopic patient. A 30-years-old woman presented two days after childbirth for sudden loss of vision in her right eye. Multimodal imaging showed macular hemorrhage masking a subtle yellowish linear lesion corresponding to lacker crack. Due to the lack of evidence for choroidal neovascularization, a simple clinical and imaging monitoring was recommended. Six weeks later, we noted an improvement in her best-corrected visual acuity and a decreased in size of the macular hemorrhage. Conclusions This is the first case reporting a macular subretinal bleeding on macular lacquer cracks in a highly myopic patient in immediate post partum. Valsalva maneuver associated with vaginal delivery could explain the occurrence of the hemorrhage associated with lacquer crack. However, natural history of pathological myopia could not be excluded.

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