Abstract
Abstract
Background
IgG4-related disease (IgG4-RD) is a progressive and sometimes fatal disease that rarely affects pediatric age group. It may affect the orbits, lacrimal and salivary glands, pancreas, kidneys, peritoneum and other organs. Lung and pleura are not commonly reported in IgG4-RD. We here present a rare case of pediatric IgG4-RD with rare involvement of pericardium, pleura and lungs.
Case presentation
A 13-year-old girl presented with intrathoracic IgG4-RD with pleuropericardial involvement. She showed initial improvement on prednisolone. Azathioprine and then mycophenolate failed to control relapses during steroid tapering. Her last relapse was treated by rituximab however, the patient developed acute fatal massive hemoptysis.
Conclusions
Pediatric IgG4-RD is a rare entity with pericardio-pulmonary affection as the rare of the rare. Usual treatment of prednisolone and steroid sparing agents should be used, with rituximab used as a rescue therapy, but fatal complications may occur.
Publisher
Springer Science and Business Media LLC
Subject
Immunology and Allergy,Rheumatology,Pediatrics, Perinatology and Child Health
Reference50 articles.
1. Perugino C, Stone J. IgG4-related disease: an update on pathophysiology and implications for clinical care. Nat Rev Rheumatol. 2020;16(12):702-14. Available from: https://pubmed.ncbi.nlm.nih.gov/32939060/ cited 2021 Oct 9.
2. Umehara H, Okazaki K, Kawa S, Takahashi H, Goto H, Matsui S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Mod Rheumatol. 2021;31(3):529–33. Available from: https://pubmed.ncbi.nlm.nih.gov/33274670/ cited 2021 Oct 9.
3. Yamamoto M, Takahashi H, Shinomura Y. Mechanisms and assessment of IgG4-related disease: lessons for the rheumatologist. Nat Rev Rheumatol. 2014;10(3):148–59. Available from: https://pubmed.ncbi.nlm.nih.gov/24296677/ cited 2021 Oct 9.
4. Lees J, Church N, Langdale-Brown B, Bellamy C, Gibson P, Watson S. IgG4-related disease: a novel, important but easily missed condition. J R Coll Physicians Edinb. 2013;43(2):126–33. Available from: http://www.rcpe.ac.uk/journal/issue/journal_43_2/watson.pdfS.
5. Kaya Akca Ü, Atalay E, KasapCüceoğlu M, Şener S, Balık Z, Başaran Ö, et al. IgG4-related disease in pediatric patients: a single-center experience. Rheumatol Int. 2021;2021(1):1–9. Available from: https://link.springer.com/article/10.1007/s00296-021-04885-5 cited 2021 Oct 9.
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