{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T11:31:43Z","timestamp":1789212703253,"version":"build-2803163510"},"reference-count":56,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2009,1]]},"DOI":"10.1097\/bor.0b013e32831cb939","type":"journal-article","created":{"date-parts":[[2008,12,11]],"date-time":"2008-12-11T08:20:46Z","timestamp":1228983646000},"page":"78-84","source":"Crossref","is-referenced-by-count":56,"title":["Rheumatologic and autoimmune manifestations of primary immunodeficiency disorders"],"prefix":"10.1097","volume":"21","author":[{"given":"Ramona","family":"Goyal","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariel C","family":"Bulua","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikolay P","family":"Nikolov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pamela L","family":"Schwartzberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard M","family":"Siegel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-14-20210128","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1056\/NEJM199508173330707","article-title":"The primary immunodeficiencies","volume":"333","author":"Rosen","year":"1995","journal-title":"N Engl J Med"},{"key":"R2-14-20210128","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1097\/01.md.0000133624.65946.40","article-title":"Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of the literature","volume":"83","author":"Michel","year":"2004","journal-title":"Medicine (Baltimore)"},{"key":"R3-14-20210128","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.jaut.2005.04.006","article-title":"Treatment and outcome of autoimmune hematologic disease in common variable immunodeficiency (CVID)","volume":"25","author":"Wang","year":"2005","journal-title":"J Autoimmun"},{"issue":"Suppl 1","key":"R5-14-20210128","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1007\/s10875-007-9159-y","article-title":"Autoimmunity in severe combined immunodeficiency (SCID): lessons from patients and experimental models","volume":"28","author":"Milner","year":"2008","journal-title":"J Clin Immunol"},{"key":"R6-14-20210128","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1016\/0049-0172(93)80073-O","article-title":"Hypogammaglobulinemia and rheumatic disease","volume":"22","author":"Lee","year":"1993","journal-title":"Semin Arthritis Rheum"},{"key":"R7-14-20210128","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1053\/gast.2001.28022","article-title":"Dysfunctional LAD-1 neutrophils and colitis","volume":"121","author":"Uzel","year":"2001","journal-title":"Gastroenterology"},{"key":"R8-14-20210128","doi-asserted-by":"crossref","first-page":"1587","DOI":"10.1099\/jmm.0.46722-0","article-title":"Intractable colitis associated with chronic granulomatous disease","volume":"55","author":"Arimura","year":"2006","journal-title":"J Med Microbiol"},{"key":"R9-14-20210128","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1097\/01.bor.0000231907.50290.6f","article-title":"Omenn syndrome: a lack of tolerance on the background of deficient lymphocyte development and maturation","volume":"18","author":"Honig","year":"2006","journal-title":"Curr Opin Rheumatol"},{"key":"R10-14-20210128","doi-asserted-by":"crossref","first-page":"F9","DOI":"10.1084\/jem.192.4.F9","article-title":"Homeostatic T cell proliferation: how far can T cells be activated to self-ligands?","volume":"192","author":"Surh","year":"2000","journal-title":"J Exp Med"},{"key":"R11-14-20210128","doi-asserted-by":"crossref","first-page":"6795","DOI":"10.4049\/jimmunol.169.12.6795","article-title":"Naive B lymphocytes undergo homeostatic proliferation in response to B cell deficit","volume":"169","author":"Cabatingan","year":"2002","journal-title":"J Immunol"},{"key":"R12-14-20210128","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/S1074-7613(02)00508-3","article-title":"Neonates support lymphopenia-induced proliferation","volume":"18","author":"Min","year":"2003","journal-title":"Immunity"},{"key":"R13-14-20210128","doi-asserted-by":"crossref","first-page":"1515","DOI":"10.1084\/jem.20020033","article-title":"Cytokine requirements for acute and basal homeostatic proliferation of naive and memory CD8+ T cells","volume":"195","author":"Goldrath","year":"2002","journal-title":"J Exp Med"},{"key":"R14-14-20210128","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1016\/j.it.2004.11.006","article-title":"The new paradigm of T-cell homeostatic proliferation-induced autoimmunity","volume":"26","author":"Baccala","year":"2005","journal-title":"Trends Immunol"},{"key":"R15-14-20210128","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/S0092-8674(04)00335-6","article-title":"Homeostatic expansion of T cells during immune insufficiency generates autoimmunity","volume":"117","author":"King","year":"2004","journal-title":"Cell"},{"key":"R16-14-20210128","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1172\/JCI30513","article-title":"Homeostatically proliferating CD4 T cells are involved in the pathogenesis of an Omenn syndrome murine model","volume":"117","author":"Khiong","year":"2007","journal-title":"J Clin Invest"},{"key":"R17-14-20210128","doi-asserted-by":"crossref","first-page":"1260","DOI":"10.1172\/JCI30928","article-title":"A hypomorphic R229Q Rag2 mouse mutant recapitulates human Omenn syndrome","volume":"117","author":"Marrella","year":"2007","journal-title":"J Clin Invest"},{"key":"R18-14-20210128","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.1056\/NEJM199006283222601","article-title":"Clinical variation of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) in a series of 68 patients","volume":"322","author":"Ahonen","year":"1990","journal-title":"N Engl J Med"},{"key":"R19-14-20210128","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1038\/ni906","article-title":"Aire regulates negative selection of organ-specific T cells","volume":"4","author":"Liston","year":"2003","journal-title":"Nat Immunol"},{"key":"R20-14-20210128","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/hmg\/11.4.397","article-title":"Aire deficient mice develop multiple features of APECED phenotype and show altered immune response","volume":"11","author":"Ramsey","year":"2002","journal-title":"Hum Mol Genet"},{"key":"R21-14-20210128","doi-asserted-by":"crossref","first-page":"1395","DOI":"10.1126\/science.1075958","article-title":"Projection of an immunological self shadow within the thymus by the aire protein","volume":"298","author":"Anderson","year":"2002","journal-title":"Science"},{"key":"R22-14-20210128","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1172\/JCI200523087","article-title":"AIRE deficiency in thymus of 2 patients with Omenn syndrome","volume":"115","author":"Cavadini","year":"2005","journal-title":"J Clin Invest"},{"issue":"Suppl 1","key":"R23-14-20210128","doi-asserted-by":"crossref","first-page":"S11","DOI":"10.1007\/s10875-008-9176-5","article-title":"Primary immune deficiency disorders presenting as autoimmune diseases: IPEX and APECED","volume":"28","author":"Moraes-Vasconcelos","year":"2008","journal-title":"J Clin Immunol"},{"key":"R24-14-20210128","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1097\/00002281-200307000-00010","article-title":"Immune dysregulation, polyendocrinopathy, enteropathy, and X-linked inheritance (IPEX), a syndrome of systemic autoimmunity caused by mutations of FOXP3, a critical regulator of T-cell homeostasis","volume":"15","author":"Gambineri","year":"2003","journal-title":"Curr Opin Rheumatol"},{"key":"R25-14-20210128","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1136\/jmg.39.8.537","article-title":"Clinical and molecular features of the immunodysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome","volume":"39","author":"Wildin","year":"2002","journal-title":"J Med Genet"},{"key":"R26-14-20210128","doi-asserted-by":"crossref","first-page":"2399","DOI":"10.4049\/jimmunol.181.4.2399","article-title":"Homeostatic proliferation in the mice with germline FoxP3 mutation and its contribution to fatal autoimmunity","volume":"181","author":"Chang","year":"2008","journal-title":"J Immunol"},{"key":"R27-14-20210128","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1093\/rheumatology\/39.2.142","article-title":"Antibodies to CD4 in primary Sjogren's syndrome","volume":"39","author":"Henriksson","year":"2000","journal-title":"Rheumatology (Oxford)"},{"key":"R28-14-20210128","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1002\/art.1780280305","article-title":"Subset specificity of antilymphocyte antibodies in systemic lupus erythematosus: preferential reactivity with cells bearing the T4 and autologous erythrocyte receptor phenotypes","volume":"28","author":"Yamada","year":"1985","journal-title":"Arthritis Rheum"},{"key":"R29-14-20210128","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1172\/JCI111261","article-title":"Relationship between systemic lupus erythematosus T cell subsets, anti-T cell antibodies, and T cell functions","volume":"73","author":"Morimoto","year":"1984","journal-title":"J Clin Invest"},{"key":"R30-14-20210128","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/S0022-3476(97)70123-9","article-title":"Clinical spectrum of X-linked hyper-IgM syndrome","volume":"131","author":"Levy","year":"1997","journal-title":"J Pediatr"},{"key":"R31-14-20210128","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1097\/01.md.0000100046.06009.b0","article-title":"The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients","volume":"82","author":"Winkelstein","year":"2003","journal-title":"Medicine (Baltimore)"},{"key":"R32-14-20210128","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1097\/MOH.0b013e3282f97f88","article-title":"When T cells and macrophages do not talk: the hemophagocytic syndromes","volume":"15","author":"Arceci","year":"2008","journal-title":"Curr Opin Hematol"},{"key":"R33-14-20210128","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1111\/j.0105-2896.2005.00230.x","article-title":"Molecular and cellular pathogenesis of X-linked lymphoproliferative disease","volume":"203","author":"Nichols","year":"2005","journal-title":"Immunol Rev"},{"key":"R34-14-20210128","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1038\/2424","article-title":"Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene","volume":"20","author":"Coffey","year":"1998","journal-title":"Nat Genet"},{"key":"R35-14-20210128","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1038\/ng1601","article-title":"TACI is mutant in common variable immunodeficiency and IgA deficiency","volume":"37","author":"Castigli","year":"2005","journal-title":"Nat Genet"},{"key":"R36-14-20210128","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1038\/ng1600","article-title":"Mutations in TNFRSF13B encoding TACI are associated with common variable immunodeficiency in humans","volume":"37","author":"Salzer","year":"2005","journal-title":"Nat Genet"},{"key":"R37-14-20210128","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1006\/clim.1999.4725","article-title":"Common variable immunodeficiency: clinical and immunological features of 248 patients","volume":"92","author":"Cunningham-Rundles","year":"1999","journal-title":"Clin Immunol"},{"key":"R38-14-20210128","first-page":"1205","article-title":"Juvenile rheumatoid arthritis and common variable hypogammaglobulinemia","volume":"25","author":"Uluhan","year":"1998","journal-title":"J Rheumatol"},{"key":"R39-14-20210128","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1084\/jem.20032000","article-title":"TACI and BAFF-R mediate isotype switching in B cells","volume":"201","author":"Castigli","year":"2005","journal-title":"J Exp Med"},{"key":"R40-14-20210128","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1038\/89790","article-title":"Activation and accumulation of B cells in TACI-deficient mice","volume":"2","author":"Yan","year":"2001","journal-title":"Nat Immunol"},{"key":"R41-14-20210128","doi-asserted-by":"crossref","first-page":"876","DOI":"10.1016\/S0022-3476(05)82002-5","article-title":"A multiinstitutional survey of the Wiskott\u2013Aldrich syndrome","volume":"125","author":"Sullivan","year":"1994","journal-title":"J Pediatr"},{"key":"R42-14-20210128","doi-asserted-by":"crossref","first-page":"e622","DOI":"10.1542\/peds.111.5.e622","article-title":"Autoimmunity in Wiskott\u2013Aldrich syndrome: risk factors, clinical features, and outcome in a single-center cohort of 55 patients","volume":"111","author":"Dupuis-Girod","year":"2003","journal-title":"Pediatrics"},{"key":"R43-14-20210128","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1084\/jem.190.9.1329","article-title":"Antigen receptor-induced activation and cytoskeletal rearrangement are impaired in Wiskott-Aldrich syndrome protein-deficient lymphocytes","volume":"190","author":"Zhang","year":"1999","journal-title":"J Exp Med"},{"key":"R44-14-20210128","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1189\/jlb.0804444","article-title":"WASP deficiency leads to global defects of directed leukocyte migration in vitro and in vivo","volume":"77","author":"Snapper","year":"2005","journal-title":"J Leukoc Biol"},{"key":"R45-14-20210128","doi-asserted-by":"crossref","first-page":"11351","DOI":"10.1073\/pnas.162376099","article-title":"Wiskott-Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses","volume":"99","author":"Orange","year":"2002","journal-title":"Proc Natl Acad Sci U S A"},{"key":"R46-14-20210128","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.1093\/intimm\/dxg148","article-title":"Impaired signaling via the high-affinity IgE receptor in Wiskott-Aldrich syndrome protein-deficient mast cells","volume":"15","author":"Pivniouk","year":"2003","journal-title":"Int Immunol"},{"key":"R47-14-20210128","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/S1074-7613(00)80590-7","article-title":"Wiskott-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation","volume":"9","author":"Snapper","year":"1998","journal-title":"Immunity"},{"key":"R48-14-20210128","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1172\/JCI29539","article-title":"Wiskott-Aldrich syndrome protein is required for regulatory T cell homeostasis","volume":"117","author":"Humblet-Baron","year":"2007","journal-title":"J Clin Invest"},{"key":"R49-14-20210128","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.clim.2007.02.001","article-title":"Impaired in vitro regulatory T cell function associated with Wiskott-Aldrich syndrome","volume":"124","author":"Adriani","year":"2007","journal-title":"Clin Immunol"},{"key":"R50-14-20210128","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1084\/jem.20061338","article-title":"The Wiskott-Aldrich syndrome protein is required for the function of CD4(+)CD25(+)Foxp3(+) regulatory T cells","volume":"204","author":"Maillard","year":"2007","journal-title":"J Exp Med"},{"key":"R51-14-20210128","doi-asserted-by":"crossref","first-page":"2943","DOI":"10.1182\/blood.V95.9.2943.009k17_2943_2946","article-title":"Wiskott-Aldrich syndrome protein is necessary for efficient IgG-mediated phagocytosis","volume":"95","author":"Lorenzi","year":"2000","journal-title":"Blood"},{"key":"R52-14-20210128","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1038\/322032a0","article-title":"Cloning the gene for an inherited human disorder\u2013chronic granulomatous disease-on the basis of its chromosomal location","volume":"322","author":"Royer-Pokora","year":"1986","journal-title":"Nature"},{"key":"R53-14-20210128","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1097\/00005792-200005000-00003","article-title":"Chronic granulomatous disease. Report on a national registry of 368 patients","volume":"79","author":"Winkelstein","year":"2000","journal-title":"Medicine (Baltimore)"},{"key":"R54-14-20210128","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1038\/nri1312","article-title":"NOX enzymes and the biology of reactive oxygen","volume":"4","author":"Lambeth","year":"2004","journal-title":"Nat Rev Immunol"},{"key":"R55-14-20210128","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1016\/S0952-7915(03)00109-2","article-title":"Chronic granulomatous disease","volume":"15","author":"Heyworth","year":"2003","journal-title":"Curr Opin Immunol"},{"key":"R56-14-20210128","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1038\/ng0295-202","article-title":"Mouse model of X-linked chronic granulomatous disease, an inherited defect in phagocyte superoxide production","volume":"9","author":"Pollock","year":"1995","journal-title":"Nat Genet"},{"key":"R57-14-20210128","doi-asserted-by":"crossref","first-page":"636","DOI":"10.4049\/jimmunol.172.1.636","article-title":"Gene expression profiling provides insight into the pathophysiology of chronic granulomatous disease","volume":"172","author":"Kobayashi","year":"2004","journal-title":"J Immunol"}],"container-title":["Current Opinion in Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.lww.com\/10.1097\/BOR.0b013e32831cb939","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,13]],"date-time":"2021-05-13T22:57:49Z","timestamp":1620946669000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/00002281-200901000-00014"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,1]]},"references-count":56,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2009]]}},"URL":"https:\/\/doi.org\/10.1097\/bor.0b013e32831cb939","relation":{},"ISSN":["1040-8711"],"issn-type":[{"value":"1040-8711","type":"print"}],"subject":[],"published":{"date-parts":[[2009,1]]}}}