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Unusual presentation of systemic lupus erythematosus in a male child: a case presentation

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Title: Unusual presentation of systemic lupus erythematosus in a male child: a case presentation
Authors: Khyati Gupta, Vishal Dnyaneshwar Sawant, Sushma Save
Source: Egyptian Pediatric Association Gazette, Vol 71, Iss 1, Pp 1-5 (2023)
Publisher Information: SpringerOpen, 2023.
Publication Year: 2023
Collection: LCC:Pediatrics
Subject Terms: Systemic lupus erythematosus, Nephrotic syndrome, Steroid resistance, Membranous glomerulonephritis, Focal segmental glomerulosclerosis, Antinuclear antibody, Pediatrics, RJ1-570
More Details: Abstract Background Systemic lupus erythematosus is an autoimmune connective tissue disorder that is common among women of age group 15–40 years. The novelty in our case is owed to the deceptive demographic characteristics, not only the sex (F > > M) but also of the age profile (common in 15–44 years age group) along with deviation from typical disease presentation of skin rash/arthritis/nephritic syndrome. We aim to emphasize the importance of having a high index of suspicion in any child presenting with nephrotic range proteinuria in order to prevent delay in diagnosis. Case presentation Eight-year-old boy presented with generalized swelling, proteinuria, hypoalbuminemia and hypertriglyceridemia and was found to be unresponsive to systemic steroid therapy. Further testing revealed low complement levels(C3/C4) along with ANA positive, homogenous pattern (titre ≥ 1:80) and anti-dsDNA positive (titre 229:24) pointing towards the diagnosis of childhood SLE, which was made based on EULAR/ACR criteria. Subsequent renal biopsy was done in order to stage the disease and for initiation of appropriate treatment protocol. Conclusions SLE is a highly heterogenous disorder in terms of clinical presentation. All patients with steroid resistant nephrotic syndrome should undergo renal biopsy as a part of their workup. This case is a learning opportunity which demonstrates that even in absence of typical disease manifestations and demographic profile, a high index of suspicion will help in rapid diagnosis and prevention of complications. Knowledge about the varied presentations of renal lupus is of utmost importance for the same.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 2090-9942
Relation: https://doaj.org/toc/2090-9942
DOI: 10.1186/s43054-023-00205-7
Access URL: https://doaj.org/article/62db645e847440d2b319649f7b356efd
Accession Number: edsdoj.62db645e847440d2b319649f7b356efd
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  Data: Unusual presentation of systemic lupus erythematosus in a male child: a case presentation
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  Data: Egyptian Pediatric Association Gazette, Vol 71, Iss 1, Pp 1-5 (2023)
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  Data: Abstract Background Systemic lupus erythematosus is an autoimmune connective tissue disorder that is common among women of age group 15–40 years. The novelty in our case is owed to the deceptive demographic characteristics, not only the sex (F > > M) but also of the age profile (common in 15–44 years age group) along with deviation from typical disease presentation of skin rash/arthritis/nephritic syndrome. We aim to emphasize the importance of having a high index of suspicion in any child presenting with nephrotic range proteinuria in order to prevent delay in diagnosis. Case presentation Eight-year-old boy presented with generalized swelling, proteinuria, hypoalbuminemia and hypertriglyceridemia and was found to be unresponsive to systemic steroid therapy. Further testing revealed low complement levels(C3/C4) along with ANA positive, homogenous pattern (titre ≥ 1:80) and anti-dsDNA positive (titre 229:24) pointing towards the diagnosis of childhood SLE, which was made based on EULAR/ACR criteria. Subsequent renal biopsy was done in order to stage the disease and for initiation of appropriate treatment protocol. Conclusions SLE is a highly heterogenous disorder in terms of clinical presentation. All patients with steroid resistant nephrotic syndrome should undergo renal biopsy as a part of their workup. This case is a learning opportunity which demonstrates that even in absence of typical disease manifestations and demographic profile, a high index of suspicion will help in rapid diagnosis and prevention of complications. Knowledge about the varied presentations of renal lupus is of utmost importance for the same.
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