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Year : 2019  |  Volume : 22  |  Issue : 12  |  Page : 1781-1784

Coexistence of unusual and distinctive initial manifestations of severe systemic lupus erythematosus: A child's case, presenting as adrenal insufficiency associated with autoimmune polyglandular syndrome

Department of Pediatric Endocrinology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey

Correspondence Address:
Dr. M Karaoglan
Division of Pediatric Endocrinology of Medical Faculty of Gaziantep, Sahinbey, Gaziantep
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njcp.njcp_667_18

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The involvement of multiple endocrine organs is rarely identified as initial manifestations of systemic lupus erythematosus (SLE) and autoimmune polyglandular syndrome (APS) in infancy. Childhood SLE tends to present with more severe clinical symptoms at an early age and with a set of unexpected findings. In the literature, the case reports of children presenting with SLE and APS components at the same time are extremely rare. Adrenal insufficiency may be overlooked due to mild clinical findings in later periods, except for neonates characterized by marked salt depletion and ambiguous genitalia signs. Moreover, adrenal insufficiency as an initial symptom in childhood is quite unusual as a component of SLE-associated APS. This report describes the overlap of unexpected, unusual, and severe clinical findings in an infant with APS with a comorbidity of SLE, where the involvement of multiple endocrine organs coexists with multiple serious clinical manifestations from the beginning.

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