{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,15]],"date-time":"2026-02-15T21:31:59Z","timestamp":1771191119197,"version":"3.50.1"},"reference-count":20,"publisher":"American Academy of Pediatrics (AAP)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2010,1,1]]},"abstract":"<jats:p>Autoimmune lymphoproliferative syndrome (ALPS) is an uncommon disorder of Fas-mediated apoptosis that results in impaired lymphocyte death and, therefore, disturbed immune homeostasis. Besides presentation with lymphadenopathy and splenomegaly, patients with ALPS have a high incidence of autoimmune phenomena. To our knowledge, this is the first description of polyarteritis nodosa that includes numerous arterial aneurysms in a child with ALPS. Active vasculitis resolved after allogeneic hematopoietic stem cell transplantation. This report of polyarteritis nodosa associated with human ALPS supports previous findings in Fas-deficient mouse models that frequently develop vasculitic manifestations and suggests that apoptotic defects of lymphocytes may play a role in the pathophysiology of systemic vasculitis. Thus, patients with ALPS might be more susceptible to autoimmune vessel inflammation. This case furthermore emphasizes that even rare autoimmune manifestations should be considered and investigated in patients with immunodeficiencies, because that might help in planning treatment strategies for these patients.<\/jats:p>","DOI":"10.1542\/peds.2009-1999","type":"journal-article","created":{"date-parts":[[2009,12,22]],"date-time":"2009-12-22T01:34:22Z","timestamp":1261445662000},"page":"e169-e173","source":"Crossref","is-referenced-by-count":6,"title":["Childhood Polyarteritis Nodosa in Autoimmune Lymphoproliferative Syndrome"],"prefix":"10.1542","volume":"125","author":[{"given":"Nora","family":"Naumann-Bartsch","sequence":"first","affiliation":[{"name":"Clinic for Children and Adolescents"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Stachel","sequence":"additional","affiliation":[{"name":"Clinic for Children and Adolescents"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick","family":"Morhart","sequence":"additional","affiliation":[{"name":"Clinic for Children and Adolescents"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gundula","family":"Staatz","sequence":"additional","affiliation":[{"name":"Section for Pediatric Radiology, Radiology Institute, University Erlangen-Nuremberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jo\u0308rg","family":"Ju\u0308ngert","sequence":"additional","affiliation":[{"name":"Clinic for Children and Adolescents"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus","family":"Schwarz","sequence":"additional","affiliation":[{"name":"Institute for Transfusion Medicine, University Ulm, Ulm, Germany"},{"name":"Institute for Clinical Transfusion Medicine and Immunogenetics Ulm, German Red Cross Blood Service Baden-Wuerttemberg-Hessen, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"Holter","sequence":"additional","affiliation":[{"name":"Clinic for Children and Adolescents"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"417","published-online":{"date-parts":[[2010,1,1]]},"reference":[{"key":"2021120821184761900_R1","doi-asserted-by":"crossref","unstructured":"Bide\u0300re N, Su HC, Lenardo MJ. Genetic disorders of programmed cell death in the immune system. Annu Rev Immunol.\u20082006;24:321\u2013352","DOI":"10.1146\/annurev.immunol.24.021605.090513"},{"key":"2021120821184761900_R2","doi-asserted-by":"crossref","unstructured":"Rao VK, Straus SE. Causes and consequences of the autoimmune lymphoproliferative syndrome. Hematology.\u20082006;11(1):15\u201323","DOI":"10.1080\/10245330500329094"},{"key":"2021120821184761900_R3","doi-asserted-by":"crossref","unstructured":"Ozen S, Anton J, Arisoy N, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr.\u20082004;145(4):517\u2013522","DOI":"10.1016\/j.jpeds.2004.06.046"},{"key":"2021120821184761900_R4","doi-asserted-by":"crossref","unstructured":"Eleftheriou D, Brogan PA. Vasculitis in children. Best Pract Res Clin Rheumatol.\u20082009;23(3):309\u2013323","DOI":"10.1016\/j.berh.2009.02.001"},{"key":"2021120821184761900_R5","doi-asserted-by":"crossref","unstructured":"Kendirli T, Yu\u0308ksel S, Oral M, et al. Fatal polyarteritis nodosa with gastrointestinal involvement in a child. Pediatr Emerg Care.\u20082006;22(12):810\u2013812","DOI":"10.1097\/01.pec.0000245172.38967.d0"},{"key":"2021120821184761900_R6","doi-asserted-by":"crossref","unstructured":"Sneller MC, Straus SE, Jaffe ES, et al. A novel lymphoproliferative\/autoimmune syndrome resembling murine lpr\/gld disease. J Clin Invest.\u20081992;90(2):334\u2013341","DOI":"10.1172\/JCI115867"},{"key":"2021120821184761900_R7","doi-asserted-by":"crossref","unstructured":"Magerus-Chatinet A, Stolzenberg MC, Loffredo MS, et al. FAS-L, IL-10, and double-negative CD4\u2212CD8\u2212TCR\u03b1\/\u03b2+ T cells are reliable markers of autoimmune lymphoproliferative syndrome (ALPS) associated with FAS loss of function. Blood.\u20082009;113(13):3027\u20133030","DOI":"10.1182\/blood-2008-09-179630"},{"key":"2021120821184761900_R8","unstructured":"Rieux-Laucat F, Blache\u0300re S, Danielan S, et al. Lymphoproliferative syndrome with autoimmunity: a possible genetic basis for dominant expression of the clinical manifestations. Blood.\u20081999;94(8):2575\u20132582"},{"key":"2021120821184761900_R9","doi-asserted-by":"crossref","unstructured":"Nose M. A proposal concept of a polygene network in systemic vasculitis: lessons from MRL mouse models. Allergol Int.\u20082007;56(2):79\u201386","DOI":"10.2332\/allergolint.R-04-140"},{"key":"2021120821184761900_R10","doi-asserted-by":"crossref","unstructured":"Ozen S, Ruperto N, Dillon MJ, et al. EULAR\/PreS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis.\u20082006;65(7):936\u2013941","DOI":"10.1136\/ard.2005.046300"},{"key":"2021120821184761900_R11","unstructured":"Dolezalova\u0301 P, Telekesova\u0301 P, Nemcova\u0301 D, Hoza J. Incidence of vasculitis in children in the Czech republic: 2-year prospective epidemiology survey. J Rheumatol.\u20082004;31(11):2295\u20132299"},{"key":"2021120821184761900_R12","doi-asserted-by":"crossref","unstructured":"Han JW, Shimada K, Ma-Krupa W, et al. Vessel wall-embedded dendritic cells induce T-cell autoreactivity and initiate vascular inflammation. Circ Res.\u20082008;102(5):546\u2013553","DOI":"10.1161\/CIRCRESAHA.107.161653"},{"key":"2021120821184761900_R13","doi-asserted-by":"crossref","unstructured":"Walsh K, Smith RC, Kim HS. Vascular cell apoptosis in remodelling, restenosis, and plaque rupture. Circ Res.\u20082000;87(3):184\u2013188","DOI":"10.1161\/01.RES.87.3.184"},{"key":"2021120821184761900_R14","doi-asserted-by":"crossref","unstructured":"Sata M, Suhara T, Walsh K. Vascular endothelial cells and smooth muscle cells differ in expression of Fas and Fas ligand and in sensitivity to Fas ligand-induced cell death: implications for vascular disease and therapy. Arterioscler Thromb Vasc Biol.\u20082000;20(2):309\u2013316","DOI":"10.1161\/01.ATV.20.2.309"},{"key":"2021120821184761900_R15","doi-asserted-by":"crossref","unstructured":"Maugeri N, Rovere-Querini P, Baldini M, Sabbadini MG, Manfredi AA. Translational mini-review series on immunology of vascular disease: mechanisms of vascular inflammation and remodelling in systemic vasculitis. Clin Exp Immunol.\u20082009;156(3):395\u2013404","DOI":"10.1111\/j.1365-2249.2009.03921.x"},{"key":"2021120821184761900_R16","doi-asserted-by":"crossref","unstructured":"Sleight BJ, Prasad VS, DeLaat C, et al. Correction of autoimmune lymphoproliferative syndrome by bone marrow transplantation. Bone Marrow Transplant.\u20081998;22(4):375\u2013380","DOI":"10.1038\/sj.bmt.1701306"},{"key":"2021120821184761900_R17","doi-asserted-by":"crossref","unstructured":"Dimopoulou MN, Gandhi S, Ghevaert C, et al. Successful treatment of autoimmune lymphoproliferative syndrome and refractory autoimmune thrombocytopenic purpura with a reduced intensity conditioning stem cell transplantation followed by donor lymphocyte infusion. Bone Marrow Transplant.\u20082007;40(6):605\u2013606","DOI":"10.1038\/sj.bmt.1705775"},{"key":"2021120821184761900_R18","doi-asserted-by":"crossref","unstructured":"Saccardi R, Di Gioia M, Bosi A. Haematopoietic stem cell transplantation for autoimmune disorders. Curr Opin Hematol.\u20082008;15(6):594\u2013600","DOI":"10.1097\/MOH.0b013e3283136700"},{"key":"2021120821184761900_R19","doi-asserted-by":"crossref","unstructured":"Vaishnaw AK, Orlinick JR, Chu JL, Krammer PH, Chao MV, Elkon KB. The molecular basis for apoptotic defects in patients with CD95 (Fas\/Apo-1) mutations. J Clin Invest.\u20081999;103(3):355\u2013363","DOI":"10.1172\/JCI5121"},{"key":"2021120821184761900_R20","doi-asserted-by":"crossref","unstructured":"Vaishnaw AK, Toubi E, Ohsako S, et al. The spectrum of apoptotic defects and clinical manifestations, including systemic lupus erythematosus, in humans with CD95 (Fas\/APO-1) mutations. Arthritis Rheum.\u20081999;42(9):1833\u20131842","DOI":"10.1002\/1529-0131(199909)42:9<1833::AID-ANR7>3.0.CO;2-Q"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/125\/1\/e169\/1008802\/zpe0011000e169.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/125\/1\/e169\/1008802\/zpe0011000e169.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,9]],"date-time":"2021-12-09T01:24:45Z","timestamp":1639013085000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/125\/1\/e169\/29700\/Childhood-Polyarteritis-Nodosa-in-Autoimmune"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,1,1]]},"references-count":20,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2010,1,1]]},"published-print":{"date-parts":[[2010,1,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2009-1999","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,1,1]]}}}