A case of isolated IgG4-related lung disease in a teenager
https://doi.org/10.14412/1996-7012-2020-1-74-77
Abstract
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.
About the Authors
E. V. Sokol
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
34A, Kashirskoe Shosse, Moscow 115522
Russian Federation
34A, Kashirskoe Shosse, Moscow 115522
V. I. Vasilyev
MEDSI Clinical and Diagnostic Center on Belorusskaya
Russian Federation
3A, Gruzinsky Lane, Moscow 123056
Russian Federation
3A, Gruzinsky Lane, Moscow 123056
N. V. Kokosadze
N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia
Russian Federation
23, Kashirskoe Shosse, Moscow 115478
Russian Federation
23, Kashirskoe Shosse, Moscow 115478
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Review
For citations:
Sokol EV, Vasilyev VI, Kokosadze NV. A case of isolated IgG4-related lung disease in a teenager. Sovremennaya Revmatologiya=Modern Rheumatology Journal. 2020;14(1):74-77. https://doi.org/10.14412/1996-7012-2020-1-74-77
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