Childhood-Onset Sjögren Syndrome Presenting as Pulmonary Hemorrhage.
Pediatrics
; 148(2)2021 08.
Article
in En
| MEDLINE
| ID: mdl-34321337
ABSTRACT
Primary Sjögren syndrome is an autoimmune disease characterized by inflammation of the salivary and lacrimal exocrine glands but can also present with systemic extraglandular manifestations, including pulmonary disease. Commonly described pulmonary manifestations of Sjögren syndrome include airway disease, interstitial lung disease, pulmonary arterial hypertension, and lymphoproliferative disorders. However, diffuse alveolar hemorrhage as a sequela of Sjögren syndrome has rarely been described in the adult literature and has never been described in a child. Here we report the case of an 11-year-old girl who presented with diffuse alveolar hemorrhage and was diagnosed with childhood-onset Sjögren syndrome who otherwise lacked typical clinical features, such as sicca symptoms, at the time of presentation. She was successfully treated with corticosteroids and rituximab, with sustained pulmonary remission 1 year post diagnosis. Our case highlights the heterogenous presentation of Sjögren syndrome in the pediatric population and the need for increased awareness among pediatric providers to recognize potential systemic manifestations of this disease to avoid delayed diagnosis.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Sjogren's Syndrome
/
Hemorrhage
/
Lung Diseases
Type of study:
Diagnostic_studies
Limits:
Child
/
Female
/
Humans
Language:
En
Journal:
Pediatrics
Year:
2021
Document type:
Article