A case of isolated IgG4-related lung disease in a teenager
IgG4-related disease (IgG4-RD) is a rare immune-mediated fibroinflammatory disease that is characterized by the occurrence of nodules in one or more organs and proceeds in most patients with the elevated levels of IgG4 in serum and/or in the tissues of the affected organs. The majority of patients a...
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| Format: | Article |
| Language: | Russian |
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IMA-PRESS LLC
2020-03-01
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| Series: | Современная ревматология |
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| Online Access: | https://mrj.ima-press.net/mrj/article/view/996 |
| _version_ | 1826556880371056640 |
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| author | E. V. Sokol V. I. Vasilyev N. V. Kokosadze |
| author_facet | E. V. Sokol V. I. Vasilyev N. V. Kokosadze |
| author_sort | E. V. Sokol |
| collection | DOAJ |
| description | IgG4-related disease (IgG4-RD) is a rare immune-mediated fibroinflammatory disease that is characterized by the occurrence of nodules in one or more organs and proceeds in most patients with the elevated levels of IgG4 in serum and/or in the tissues of the affected organs. The majority of patients are elderly men, and the disease in most cases has a slowly progressing systemic course. The cases of isolated IgG4-related injury to the viscera, which are much less common than the systemic type of the disease, are a very difficult differential diagnosis, and biopsy of these organs is associated with technical difficulties and poses a threat to the patient's health. The paper describes just such a case. It is interesting from several points of view: firstly, a rare site (solitary pulmonary nodular lesion); secondly, clinical and laboratory features (childhood onset, no immunological abnormalities – both higher serum IgG4 levels and lower complement components).Clinicians should remember that IgG4-RD does not always have a systemic course and characteristic serological markers. In these cases, histological verification of the diagnosis is of particular importance. If the histological pattern of a fibroinflammatory pseudotumor is identified, especially in the presence of multiple nodules, IgG4-RD should always be included in the differential diagnosis. |
| first_indexed | 2024-04-10T02:05:57Z |
| format | Article |
| id | doaj.art-0a5c2f7bc6cb4b2d945623df29c5e5b2 |
| institution | Directory Open Access Journal |
| issn | 1996-7012 2310-158X |
| language | Russian |
| last_indexed | 2025-03-14T08:19:44Z |
| publishDate | 2020-03-01 |
| publisher | IMA-PRESS LLC |
| record_format | Article |
| series | Современная ревматология |
| spelling | doaj.art-0a5c2f7bc6cb4b2d945623df29c5e5b22025-03-02T13:11:02ZrusIMA-PRESS LLCСовременная ревматология1996-70122310-158X2020-03-01141747710.14412/1996-7012-2020-1-74-772239A case of isolated IgG4-related lung disease in a teenagerE. V. Sokol0V. I. Vasilyev1N. V. Kokosadze2V.A. Nasonova Research Institute of RheumatologyMEDSI Clinical and Diagnostic Center on BelorusskayaN.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of RussiaIgG4-related disease (IgG4-RD) is a rare immune-mediated fibroinflammatory disease that is characterized by the occurrence of nodules in one or more organs and proceeds in most patients with the elevated levels of IgG4 in serum and/or in the tissues of the affected organs. The majority of patients are elderly men, and the disease in most cases has a slowly progressing systemic course. The cases of isolated IgG4-related injury to the viscera, which are much less common than the systemic type of the disease, are a very difficult differential diagnosis, and biopsy of these organs is associated with technical difficulties and poses a threat to the patient's health. The paper describes just such a case. It is interesting from several points of view: firstly, a rare site (solitary pulmonary nodular lesion); secondly, clinical and laboratory features (childhood onset, no immunological abnormalities – both higher serum IgG4 levels and lower complement components).Clinicians should remember that IgG4-RD does not always have a systemic course and characteristic serological markers. In these cases, histological verification of the diagnosis is of particular importance. If the histological pattern of a fibroinflammatory pseudotumor is identified, especially in the presence of multiple nodules, IgG4-RD should always be included in the differential diagnosis.https://mrj.ima-press.net/mrj/article/view/996igg4-related diseaseigg4-related lung diseaseclinical case |
| spellingShingle | E. V. Sokol V. I. Vasilyev N. V. Kokosadze A case of isolated IgG4-related lung disease in a teenager Современная ревматология igg4-related disease igg4-related lung disease clinical case |
| title | A case of isolated IgG4-related lung disease in a teenager |
| title_full | A case of isolated IgG4-related lung disease in a teenager |
| title_fullStr | A case of isolated IgG4-related lung disease in a teenager |
| title_full_unstemmed | A case of isolated IgG4-related lung disease in a teenager |
| title_short | A case of isolated IgG4-related lung disease in a teenager |
| title_sort | case of isolated igg4 related lung disease in a teenager |
| topic | igg4-related disease igg4-related lung disease clinical case |
| url | https://mrj.ima-press.net/mrj/article/view/996 |
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