Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case Report
The B lymphocyte developmental blocks agammaglobulinemia, leading to peripheral B cell depletion and plasma immunoglobulin reduction. Agammaglobulinemia is a rare yet severe disease since it is presented with recurrent sinopulmonary and skin, central nervous system, bone, and joint infections. The o...
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| Format: | Article |
| Language: | English |
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Mashhad University of Medical Sciences
2020-06-01
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| Series: | Reviews in Clinical Medicine |
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| Online Access: | https://rcm.mums.ac.ir/article_16296_ac20e5875b782cb5d6e34fdbfa7dc1b8.pdf |
| _version_ | 1797976064961544192 |
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| author | Azadeh Zare Feizabadi Abdolreza Malek Hamid Ahanchian |
| author_facet | Azadeh Zare Feizabadi Abdolreza Malek Hamid Ahanchian |
| author_sort | Azadeh Zare Feizabadi |
| collection | DOAJ |
| description | The B lymphocyte developmental blocks agammaglobulinemia, leading to peripheral B cell depletion and plasma immunoglobulin reduction. Agammaglobulinemia is a rare yet severe disease since it is presented with recurrent sinopulmonary and skin, central nervous system, bone, and joint infections. The onset of the disease is reported to be at the age of six months. Associations have been reported between arthritis and immunodeficiency disorders, such as agammaglobulinemia and common variable immunodeficiency (CVID). This study aimed to present the case of a 3.5-year-old female with a three-month history of the swelling of the left knee, mimicking oligoarticular juvenile idiopathic arthritis. After the initiation of immunosuppressive treatment, the patient developed large, tender, erythematous lesions on the inguinal region bilaterally, which developed to ecthyma gangrenosum due to Pseudomonas. The patient’s mother also reported recurrent episodes of infections since the patient was a one-year-old infant. Subsequent to the immunological examinations and laboratory tests, the patient was diagnosed with autosomal recessive agammaglobulinemia due to low serum immunoglobulin concentration and the absence of peripheral B cells. Primary immunodeficiency conditions (particularly agammaglobulinemia) and CVID should be considered in children with arthritis and recurrent infections. Moreover, immunological analysis should be performed prior to treatment in these children |
| first_indexed | 2024-04-11T04:45:24Z |
| format | Article |
| id | doaj.art-3abc3c5849b141259d12e46039148d86 |
| institution | Directory Open Access Journal |
| issn | 2345-6256 2345-6892 |
| language | English |
| last_indexed | 2024-04-11T04:45:24Z |
| publishDate | 2020-06-01 |
| publisher | Mashhad University of Medical Sciences |
| record_format | Article |
| series | Reviews in Clinical Medicine |
| spelling | doaj.art-3abc3c5849b141259d12e46039148d862022-12-27T13:03:33ZengMashhad University of Medical SciencesReviews in Clinical Medicine2345-62562345-68922020-06-0172757710.22038/rcm.2020.43271.128916296Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case ReportAzadeh Zare Feizabadi0Abdolreza Malek1Hamid Ahanchian2Student Research Committee, Mashhad University of Medical Sciences, Mashhad, IranDepartment of Pediatrics, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IranAllergy Research Center, Mashhad University of Medical Sciences, Mashhad, IranThe B lymphocyte developmental blocks agammaglobulinemia, leading to peripheral B cell depletion and plasma immunoglobulin reduction. Agammaglobulinemia is a rare yet severe disease since it is presented with recurrent sinopulmonary and skin, central nervous system, bone, and joint infections. The onset of the disease is reported to be at the age of six months. Associations have been reported between arthritis and immunodeficiency disorders, such as agammaglobulinemia and common variable immunodeficiency (CVID). This study aimed to present the case of a 3.5-year-old female with a three-month history of the swelling of the left knee, mimicking oligoarticular juvenile idiopathic arthritis. After the initiation of immunosuppressive treatment, the patient developed large, tender, erythematous lesions on the inguinal region bilaterally, which developed to ecthyma gangrenosum due to Pseudomonas. The patient’s mother also reported recurrent episodes of infections since the patient was a one-year-old infant. Subsequent to the immunological examinations and laboratory tests, the patient was diagnosed with autosomal recessive agammaglobulinemia due to low serum immunoglobulin concentration and the absence of peripheral B cells. Primary immunodeficiency conditions (particularly agammaglobulinemia) and CVID should be considered in children with arthritis and recurrent infections. Moreover, immunological analysis should be performed prior to treatment in these childrenhttps://rcm.mums.ac.ir/article_16296_ac20e5875b782cb5d6e34fdbfa7dc1b8.pdfagammaglobulinemiaimmunoglobulinsjuvenile idiopathic arthritis |
| spellingShingle | Azadeh Zare Feizabadi Abdolreza Malek Hamid Ahanchian Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case Report Reviews in Clinical Medicine agammaglobulinemia immunoglobulins juvenile idiopathic arthritis |
| title | Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case Report |
| title_full | Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case Report |
| title_fullStr | Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case Report |
| title_full_unstemmed | Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case Report |
| title_short | Autosomal Recessive Agammaglobulinemia in Juvenile Idiopathic Arthritis: A Case Report |
| title_sort | autosomal recessive agammaglobulinemia in juvenile idiopathic arthritis a case report |
| topic | agammaglobulinemia immunoglobulins juvenile idiopathic arthritis |
| url | https://rcm.mums.ac.ir/article_16296_ac20e5875b782cb5d6e34fdbfa7dc1b8.pdf |
| work_keys_str_mv | AT azadehzarefeizabadi autosomalrecessiveagammaglobulinemiainjuvenileidiopathicarthritisacasereport AT abdolrezamalek autosomalrecessiveagammaglobulinemiainjuvenileidiopathicarthritisacasereport AT hamidahanchian autosomalrecessiveagammaglobulinemiainjuvenileidiopathicarthritisacasereport |