Persistence and Severity of Cutaneous Manifestations in IgA Vasculitis Is Associated with Development of IgA Vasculitis Nephritis in Children. 2022

Mario Sestan, and Sasa Srsen, and Nastasia Kifer, and Matej Sapina, and Mateja Batnozic Varga, and Aleksandar Ovuka, and Martina Held, and Ana Kozmar, and Marijan Frkovic, and Gordana Laskarin, and Alenka Gagro, and Marija Jelusic
Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia.

OBJECTIVE The purpose of this study was to evaluate the prevalence and severity of skin involvement in children with IgA vasculitis (IgAV) and its relationship with clinical and biochemical parameters and the risk of developing IgA vasculitis nephritis (IgAVN), the only cause of long-term morbidity and the main prognostic factor in IgAV patients. METHODS This national multicenter retrospective study included 611 patients under the age of 18 years with IgAV referred to five Croatian tertiary hospitals between 2009 and 2019. Patient data were collected from a database with systematic analysis of IgAV patients in the Croatian population. RESULTS Among the 611 children, 205 (33.55%) had purpura on the lower extremities, in 207 (33.88%) the rash extended on the trunk, in 149 (24.39%) it extended to the upper extremities, in 32 (5.24%) the rash was generalized, while 15 (2.47%) had the most severe skin symptoms: bullae, ulcerations, and necroses. IgAVN developed in 130 (21.28%) and persistent IgAVN (present for >3 months) in 48 (7.86%) children. Multivariate logistic regression found that presence of ulcerations and necroses (OR 3.20 [95% CI 1.03-9.91]), persistent purpura (OR 2.89 [95% CI 1.71-4.88]), and higher age (OR 1.16 [95% CI 1.09-1.23]) were significant predictors of IgAVN, whereas persistent purpura (OR 20.11 [95% CI 1.09-372.52]), male sex (OR 3.32 [95% CI 1.13-9.80]), and higher age (OR 1.15 [95% CI 1.00-1.30]) were predictors of persistent IgAVN. Among the laboratory parameters, higher serum urea (OR 1.43 [95% CI 1.03-2.00]) and reduction in activated partial thromboplastin time (OR 0.83 [95% CI 0.74-0.93]) were shown to have a significant impact on increasing the risk of persistent IgAVN. CONCLUSIONS With increasing severity and duration of cutaneous manifestations in IgAV, the risk of developing IgAVN increases, making the prognosis worse, with a greater likelihood to need more aggressive treatment.

UI MeSH Term Description Entries
D007070 Immunoglobulin A Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions. IgA,IgA Antibody,IgA1,IgA2,Antibody, IgA
D008297 Male Males
D009393 Nephritis Inflammation of any part of the KIDNEY. Nephritides
D011695 IgA Vasculitis A systemic non-thrombocytopenic purpura caused by HYPERSENSITIVITY VASCULITIS and deposition of IGA-containing IMMUNE COMPLEXES within the blood vessels throughout the body, including those in the kidney (KIDNEY GLOMERULUS). Clinical symptoms include URTICARIA; ERYTHEMA; ARTHRITIS; GASTROINTESTINAL HEMORRHAGE; and renal involvement. Most cases are seen in children after acute upper respiratory infections. Allergic Purpura,Anaphylactoid Purpura,Henoch Purpura,Henoch-Schoenlein Purpura,Purpura Hemorrhagica,Purpura, Nonthrombocytopenic,Purpura, Schoenlein-Henoch,Rheumatoid Purpura,Schoenlein-Henoch Purpura,Vasculitis, Hemorrhagic,Henoch Schonlein Purpura,Henoch-Schonlein Purpura,Purpura, Nonthrombopenic,Purpura, Schonlein-Henoch,Hemorrhagic Vasculitis,Hemorrhagica, Purpura,Henoch Schoenlein Purpura,Henoch Schonlein Purpuras,Henoch-Schonlein Purpuras,Nonthrombocytopenic Purpura,Nonthrombopenic Purpura,Nonthrombopenic Purpuras,Purpura, Allergic,Purpura, Anaphylactoid,Purpura, Henoch,Purpura, Henoch Schonlein,Purpura, Henoch-Schoenlein,Purpura, Henoch-Schonlein,Purpura, Rheumatoid,Purpura, Schoenlein Henoch,Purpura, Schonlein Henoch,Purpuras, Henoch Schonlein,Purpuras, Henoch-Schonlein,Purpuras, Nonthrombopenic,Purpuras, Schonlein-Henoch,Schoenlein Henoch Purpura,Schonlein Purpura, Henoch,Schonlein Purpuras, Henoch,Schonlein-Henoch Purpura,Schonlein-Henoch Purpuras,Vasculitis, IgA
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D014657 Vasculitis Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body. Angiitis,Angiitides,Vasculitides

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