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Nephrol Dial Transplant 2022 Jun 29. Epub 2022 Jun 29.

Department of Clinical Science, Intervention and Technology, Divison of Renal Medicine Karolinska Institutet, Stockholm, Sweden.

Background: IgA nephropathy (IgAN) and its systemic variant IgA vasculitis (IgAV) damage the glomeruli, resulting in proteinuria, hematuria and kidney impairment. Dendrin is a podocyte-specific protein suggested to be involved in the pathogenesis of IgAN. Upon cell injury, dendrin translocates from the slit diaphragm to the nucleus where it is suggested to induce apoptosis and cytoskeletal changes, resulting in proteinuria and accelerated disease progression in mice. Read More

Front Pediatr 2022 9;10:886371. Epub 2022 Jun 9.

Department of Pediatric Nephrology, Children's Hospital Colorado, Aurora, CO, United States.

IgA vasculitis (IgAV, also known as Henoch-Schönlein purpura or HSP) is a vasculitis of small vessels involving multiple organs, particularly of the joints, gastrointestinal tract, skin, and kidneys. Growing laboratory evidence has shown that complement plays a key role in the pathogenesis of IgAV, although direct evidence of this association in patients is lacking. We report a child with IgAV associated with clinical features of hypertension, nephrotic range proteinuria, acute kidney injury, and low serum C3, with histopathologic findings on renal biopsy of membranoproliferative glomerulonephritis with C3 and IgA co-dominance, and extensive complement derangements. Read More

Curr Rheumatol Rev 2022 Jun 23. Epub 2022 Jun 23.

Internal Medicine Department, Amita Health St Joseph Hospital, Chicago, Illinois, USA.

Background IgA vasculitis is the most common form of systemic vasculitis in children but can occur in adults. Inciting antigens include infections, drugs, foods, insect bites, and immunizations. Antibiotics and tumor necrosis factor (TNF) alpha inhibitors are the most common class of drugs that cause IgA vasculitis. Read More

Case Rep Rheumatol 2022 14;2022:9694911. Epub 2022 Jun 14.

Department of Medicine, Hadassah Medical Center, The Faculty of Medicine, Hebrew University, Jerusalem, Israel.

We report the case of a 29-year-old adult presenting with severe IgA vasculitis, with cutaneous, urologic, and renal manifestations. The late appearance of severe gastrointestinal bleeding dominated the clinical picture, necessitating the administration of tens of units of packed cells and the augmentation of the immunosuppressive protocol. It was not until therapy with intravenous immunoglobulin (IVIG) was introduced that the massive bleeding was controlled. Read More

Vaccines (Basel) 2022 Jun 9;10(6). Epub 2022 Jun 9.

Division of Rheumatology, Department of Internal Medicine, Jeonbuk National University Medical School, Jeonju City 54907, Korea.

IgA vasculitis is an immune complex-mediated small-vessel vasculitis that mainly occurs in children and is characterized by palpable purpura, arthralgia, abdominal pain, and glomerulonephritis. We report three cases of new-onset IgA vasculitis involving major organs in adult patients after they received either the ChAdOx1 viral vector (Oxford/AstraZeneca) vaccine or the messenger RNA-1273 (Moderna) vaccine. These cases suggest that COVID-19 vaccines have the potential to trigger IgA vasculitis and indicate that physicians need to monitor for this possible complication. Read More

Pediatr Allergy Immunol Pulmonol 2022 Jun;35(2):86-94

Department of General Surgery, Affiliated Children's Hospital of Nanchang University, Nanchang, China.

To study and develop a predictive model for the differential diagnosis of acute appendicitis (AA) and Henoch-Schonlein purpura (HSP) in children and to validate the model internally and externally. The complete data of AA and HSP cases were retrospectively analyzed and divided into internal and external verification groups. SPSS software was used for single-factor analysis and screening of independent variables, and R software was used for the development and verification of the diagnostic model. Read More

JAAD Int 2022 Sep 13;8:71-78. Epub 2022 Jun 13.

Division of Dermatology, University of Arizona, Tucson, Arizona.

Background: IgA vasculitis in adults has not been thoroughly studied. This has left a practice gap related to the management and follow-up of a population that is at an increased risk of comorbidities and potentially poor outcomes. For this reason, it is important to synthesize evidence from the current literature because this can help direct the movement for more robust studies to clarify best practice recommendations. Read More

Clin Rheumatol 2022 Jun 17. Epub 2022 Jun 17.

Department of Internal Medicine and Clinical Immunology, CHRU Tours, Tours, France.

Objective: There is currently no evidence of the possible benefit of plasma cell-targeting therapies (PCTT) in immunoglobulin A (IgA) monoclonal gammopathy (MG) associated with IgA vasculitis (IgAV). We report the outcome of different PCTT regimens in a cohort of MG-IgAV.

Methods: We used a French network to retrospectively describe the outcome of MG-IgAV patients treated with PCTT. Read More

Semin Arthritis Rheum 2022 Jun 9;55:152047. Epub 2022 Jun 9.

Hacettepe University Faculty of Medicine, Department of Pediatrics, Division of Rheumatology, Ankara, Turkey.

Objectives: Coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2, has opened a new era in the practice of pediatric rheumatology since it has been associated with inflammatory complications such as vasculitis and arthritis. In this review, we aimed to present a detailed analysis of COVID-19 associated pediatric vasculitis.

Methods: A systematic review of the English literature was performed through Pubmed/MEDLINE and Scopus up to January 1st, 2022. Read More

Pediatr Nephrol 2022 Jun 13. Epub 2022 Jun 13.

Department of Nephrology, Chinese PLA General Hospital, Chinese PLA Institute of Nephrology, State Key Laboratory of Kidney Diseases, National Clinical Research Center for Kidney Diseases, 28th Fuxing Road, Beijing, 100045, China.

Background: Both IgA nephropathy (IgAN) and Henoch-Schönlein purpura nephropathy (HSPN) are characterized by glomerular mesangial IgA deposition. Several large studies on adults have suggested that glomerular C4d deposition has prognostic value in IgAN. However, there are few relevant studies on the clinical value of C4d deposition in children with IgAN or HSPN. Read More

Cureus 2022 May 9;14(5):e24845. Epub 2022 May 9.

Community Medicine, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND.

Introduction The role of serum C3 and C4 levels as a marker of disease activity in cutaneous small-vessel vasculitis (CSVV) has been sparsely studied, especially in India. The primary objective was to determine the correlation between clinico-histopathological severity and serum C3 and C4 levels in CSVV. The secondary objective was to determine the association between direct immunofluorescence (DIF) findings and serum C3 and C4 levels and clinico-histopathological findings. Read More

Rheumatol Int 2022 Jun 13. Epub 2022 Jun 13.

Rheumatology Unit, Hospital Universitario de La Princesa, IIS-Princesa, Diego de León 62, 28006, Madrid, Spain.

COVID-19 has been related to several autoimmune diseases, triggering the appearance of autoantibodies and endothelial dysfunction. Current evidence has drawn attention to vasculitis-like phenomena and leukocytoclastic vasculitis in some COVID-19 patients. Moreover, it has been hypothesized that COVID-19 could induce flares of preexisting autoimmune disorders. Read More

J Emerg Nurs 2022 May 13. Epub 2022 May 13.

Introduction: Immunoglobulin A vasculitis is historically more commonly found in children after certain viral infections such as Epstein-Barr, varicella virus, and parvovirus B19. COVID-19 has not been formally established in literature as a trigger for immunoglobulin A vasculitis. However, a main pathogenetic mechanism of COVID-19 is vascular damage, which makes it likely that vasculitis associated with COVID-19 (ie, COVID-19-mediated immunoglobulin A vasculitis) could be biologically plausible, with serious implications, especially for adults. Read More

J Gastrointest Surg 2022 Jun 8. Epub 2022 Jun 8.

The First College of Clinical Medical Science, China Three Gorges University, Yichang, China.

BMJ Case Rep 2022 Jun 7;15(6). Epub 2022 Jun 7.

Internal Medicine, Hospital Beatriz Angelo, Loures, Lisboa, Portugal.

IgA vasculitis is a rare systemic vasculitis in adults, frequently more severe than in paediatric age. It manifests with cutaneous, articular, gastrointestinal and renal involvement.We present a case of a man in his 40s diagnosed with IgA vasculitis with cutaneous, joint, gastrointestinal and renal disease. Read More

Med Mol Morphol 2022 Jun 3. Epub 2022 Jun 3.

Department of Pathology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.

Angioimmunoblastic T-cell lymphoma (AITL), a hematological malignancy, originates from follicular helper T cells. The primary site of AITL is the lymph nodes, but extranodal presentation is frequent in patients with advanced stages. Here, we report a rare case of a patient with AITL presenting with rapidly progressive glomerulonephritis (RPGN). Read More

SAGE Open Med Case Rep 2022 25;10:2050313X221102112. Epub 2022 May 25.

Department of Pediatric and Public Health Sciences, Regina Margherita Children's Hospital Turin and University of Torino, Turin, Italy.

Henoch-Schönlein purpura is the most common systemic vasculitis in children, characterized by IgA deposits in small vessels. The etiology is unknown, but Henoch-Schönlein purpura typically follows an upper respiratory infection, or less frequently other infective or chemical triggers. The classic tetrad of symptoms includes palpable purpura (mandatory criterion), arthralgias, abdominal pain, and renal involvement. Read More

Eur J Dermatol 2022 01;32(1):132-134

Comput Math Methods Med 2022 23;2022:6991218. Epub 2022 May 23.

School of Basic Medical Sciences, Anhui Medical University, Hefei 230032, China.

Objective: The children with Henoch-Schönlein purpura (HSP) may suffer from renal insufficiency, which seriously affects the life and health of the children. This study aims to construct a prediction model of Henoch-Schönlein purpura nephritis (HSPN).

Methods: A total of 240 children with HSP treated in dermatology and pediatrics in our hospital were selected. Read More

Intern Med 2022 May 31. Epub 2022 May 31.

Department of Nephrology and Rheumatology, Toranomon Hospital Kajigaya, Japan.

A 43-year-old Japanese woman with rheumatoid arthritis treated by infliximab and methotrexate for 11 years was admitted for proteinuria and purpura. A kidney biopsy revealed endothelial damage-dominant nephritis with IgA deposition. Infliximab and methotrexate were discontinued, and tocilizumab was started; however, proteinuria persisted. Read More

Int Immunopharmacol 2022 Jul 27;108:108884. Epub 2022 May 27.

Sichuan Treatment Center for Gynaecologic and Breast Diseases (Gynecology), The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan, PR China. Electronic address:

In our previous meta-analysis of the neutrophil-to-lymphocyte and platelet-to-lymphocyte ratios in Henoch-Schonlein purpura (W. Fu, et al., Int. Read More

Int Immunopharmacol 2022 07 27;108:108883. Epub 2022 May 27.

Articular Orthopaedics, The Third Affiliated Hospital of Soochow University, 185 Juqian Road, Changzhou 213003, Jiangsu, China. Electronic address:

Ann Med Surg (Lond) 2022 May 20;77:103650. Epub 2022 Apr 20.

Department of Internal Medicine, Faculty of Medicine, Universitas Airlangga - Dr. Soetomo General Academic Hospital, Surabaya, Indonesia.

Background: Henoch-Schonlein purpura (HSP) in the elderly is very rare, so this case report was conducted to improve the diagnosis and management of HSP.

Case Presentation: A 65-year-old Indonesian male with severe complications of HSP had been reported. In addition to the patient's history, physical examination, laboratory and radiological findings, he performed a left sole anterior biopsy that showed interstitial infiltrate of main neutrophils, and faint granular deposits of IgA and C3 seen within the superficial vessels. Read More

Vaccines (Basel) 2022 May 9;10(5). Epub 2022 May 9.

Department of Nephrology, The Second Affiliated Hospital of Nanchang University, No. 1, Minde Road, Donghu District, Nanchang 330006, China.

The coronavirus disease 2019 (COVID-19) pandemic, caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has caused an exceptional setback to the global economy and health. Vaccination is one of the most effective interventions to markedly decrease severe illness and death from COVID-19. In recent years, there have been increasingly more reports of new acute kidney injury (AKI) after COVID-19 vaccination. Read More

Int J Mol Sci 2022 May 20;23(10). Epub 2022 May 20.

Nephrology and Kidney Transplantation Unit, Department of Translational Medicine, University of Piemonte Orientale (UPO), 17-28100 Novara, Italy.

Current treatment of primary and secondary glomerulopathies is hampered by many limits and a significant proportion of these disorders still evolves towards end-stage renal disease. A possible answer to this unmet challenge could be represented by therapies with stem cells, which include a variety of progenitor cell types derived from embryonic or adult tissues. Stem cell self-renewal and multi-lineage differentiation ability explain their potential to protect and regenerate injured cells, including kidney tubular cells, podocytes and endothelial cells. Read More

Children (Basel) 2022 May 2;9(5). Epub 2022 May 2.

Department of General Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine, Medical Center, University of Freiburg, Mathildenstrasse 1, 79106 Freiburg, Germany.

The onset of IgA nephritis in childhood and adolescence often develops into chronic glomerulonephritis with declining renal function. Although these long-term consequences are known, there is still a lack of evidence-based treatment recommendations in this age group. We report data from 22 pediatric patients who were biopsied to confirm the diagnosis of IgAN at our clinical center. Read More

Children (Basel) 2022 Apr 27;9(5). Epub 2022 Apr 27.

Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool L12 2AP, UK.

Chronic kidney disease is a recognised complication of immunoglobulin A vasculitis, (IgAV; formerly Henoch-Schonlein purpura-HSP). The pathophysiology of IgAV and why some patients develop significant renal involvement remains largely unknown. Identifying urinary inflammatory markers could direct targets for earlier intervention. Read More

Cells 2022 05 12;11(10). Epub 2022 May 12.

Institute of Experimental Immunology and Hepatology, Center of Experimental Medicine, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany.

Glomerulonephritis (GN) comprises a group of immune-mediated kidney diseases affecting glomeruli and the tubulointerstitium. Glomerular crescent formation is a histopathological characteristic of severe forms of GN, also referred to as crescentic GN (cGN). Based on histological findings, cGN includes anti-neutrophil cytoplasmic antibody (ANCA)-associated GN, a severe form of ANCA-associated vasculitis, lupus nephritis associated with systemic lupus erythematosus, Goodpasture's disease, and IgA nephropathy. Read More

JMA J 2022 Apr 25;5(2):277-279. Epub 2022 Mar 25.

Department of Pediatrics, Faculty of Medicine, University of Toyama, Toyama, Japan.

IgA vasculitis is the most common systemic small vasculitis in children. Its major clinical manifestations are palpable purpura, arthritis and arthralgias, gastrointestinal involvement, and renal manifestations. Regarding gastrointestinal manifestations, steroids are effective in reducing abdominal pain. Read More

Int J Nephrol 2022 9;2022:1749548. Epub 2022 May 9.

Department of Nephrology, Tokyo Women's Medical University, Tokyo, Japan.

Background: The importance of crescent formation in glomerulonephritis has increased. However, detailed analysis of crescentic glomerulonephritis in Asia is scarce. In addition, advances in serological diagnostic techniques (antineutrophil cytoplasmic and antiglomerular basement membrane autoantibodies) and early diagnosis have reduced the number of cases meeting the strict definition of crescentic glomerulonephritis (>50% of glomeruli are crescentic). Read More