Background: Granulomatosis with polyangiitis (GPA) with early manifestations simulating IgA vasculitis is a very rare childhood systemic disease. . A 10-year-old boy presented initially with cutaneous, skeletal, and abdominal signs suggestive of IgA vasculitis. Over time, the worsening of skin ulcers, orchitis, and renal involvement led to the diagnosis of GPA according to cytoplasmic positive antineutrophil cytoplasmic antibodies and renal biopsy.
Conclusion: Clinicians should be awared of the diagnostic pitfalls when making a clinical diagnosis of IgA vasculitis in children older than 7 years.
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http://dx.doi.org/10.1155/2023/9950855 | DOI Listing |
Zhongguo Dang Dai Er Ke Za Zhi
December 2024
Department of Pediatrics, First Affiliated Hospital of Henan University of Chinese Medicine, Zhengzhou 450000, China.
Objectives: To investigate the impact of the different proportions of crescent formation on clinical manifestations and pathological features in children with immunoglobulin A vasculitis with nephritis (IgAVN).
Methods: The children with IgAVN were divided into no-crescent group (75 children), ≤25% crescent group (156 children), and >25% crescent group (33 children).
Results: Compared with the no-crescent group, the other two groups had significant increases in 24-hour urinary protein, urinary immunoglobulin G (IgG)/creatinine ratio, urine red blood cell count, fibrinogen, and neutrophil-lymphocyte ratio, a significant reduction in serum IgG, and a significantly higher proportion of children with low albumin and hypercoagulability, pathological grade III+IV or diffuse mesangial proliferation (<0.
Rom J Intern Med
December 2024
Department of Nephrology, University of Medicine, and Pharmacy "Iuliu Hatieganu" Cluj-Napoca, 8 Victor Babeș Street, 400012 Cluj-Napoca, Romania.
Purpose: To provide epidemiologic data on kidney biopsy from Romania.
Methods: Retrospective observational study of kidney biopsy records for adult patients from a referral center in the north-western part of Romania, reported for 2014-2023.
Results: 556 biopsies were performed, corresponding to an incidence of 12 biopsies/m person-year with over 50% increase over the last reported year.
J Dermatol
December 2024
Department of Dermatology, Fukushima Medical University, Fukushima, Japan.
Rheumatology (Oxford)
December 2024
AP-HP, Université Paris Saclay, department of internal medicine and clinical immunology, Bicêtre Hospital, Le Kremlin Bicêtre, France.
Objective: To describe presentation, treatment and outcome of immune checkpoint inhibitor (ICI) associated-vasculitis in cancer patients in a multicentre study.
Methods: Thanks to the ImmunoCancer International Registry (ICIR), a multidisciplinary network focused on the research of the immune related adverse events related to cancer immunotherapies, patients presenting with a clinical and/or radiological suspicion of vasculitis, and histological evidence of vasculitis after being exposed to ICIs were retrospectively identified.
Results: Twenty eight cases were identified in the ICIR registry.
Mod Rheumatol Case Rep
December 2024
Department of Rheumatology, Tokyo Bay Urayasu Ichikawa Medical Center, Chiba, Japan.
Diffuse alveolar hemorrhage (DAH) is a rare and severe complication of IgA vasculitis (IgAV). Although glucocorticoids and immunosuppressive agents are used for its treatment, there is no consensus on the optimal form of treatment. We herein report the case of a 53-year-old, female patient with IgAV.
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