title: Connective tissue disease presenting with signs and symptoms of pulmonary hypertension in children.
abstract: Our case series describes three children who were initially diagnosed as having severe pulmonary arterial hypertension (PAH) and subsequently found to be positive for specific autoantibodies suggestive of an underlying autoimmune process. The signs and symptoms of PAH are subtle and may be part of the initial presentation of childhood connective tissue disease (CTD). Evaluation for connective tissue disease in the newly diagnosed pulmonary hypertension (PH) patient is important because early diagnosis of PH as well as CTD is crucial in the successful management of these complex patients. Ongoing monitoring for CTD in patients with severe PAH is warranted.
authors: Department of Pediatrics, The Children's Hospital, University of Colorado Denver School of Medicine, 3123 East 16th Avenue, B100, Aurora, CO 80045, USA. tekeshin0621@msn.com
authors: Shinichi
authors: S
authors: Takatsuki
authors: Jennifer B
authors: JB
authors: Soep
authors: Michelle
authors: M
authors: Calderbank
authors: David Dunbar
authors: DD
authors: Ivy
mesh_terms: D000293:Adolescent; D002648:Child; D003240:Connective Tissue Diseases / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D003937:Diagnosis, Differential; D015150:Echocardiography, Doppler; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D011669:Pulmonary Wedge Pressure; D014057:Tomography, X-Ray Computed