title: [Severe isolated pulmonary hypertension as main manifestation of Crest syndrome].
abstract: Interstitial pulmonary fibrosis is the leading cause of secondary pulmonary hypertension in systemic sclerosis, and it occurs in either limited or diffuse cutaneous scleroderma subset. Isolated pulmonary hypertension, without pulmonary disease, occurs primarily in patients with limited cutaneous scleroderma (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia [CREST] variant) although it is an unusual feature in this subset, with a worse prognosis in the short term. We present a previously undiagnosed patient with the CREST syndrome, with severe isolated pulmonary hypertension and secondary respiratory failure as major feature of its connective tissue disease. Clinical, prognostic and therapeutical aspects are commented.
authors.affiliation: Servicio de Medicina Interna, Hospital General de Elda, Alicante.
authors.forename: M
authors.initials: M
authors.lastname: Arenas Gracia
authors.forename: J
authors.initials: J
authors.lastname: Ruiz Riquelme
authors.forename: A
authors.initials: A
authors.lastname: Tovar Martínez
authors.forename: V
authors.initials: V
authors.lastname: Martínez López
authors.forename: J V
authors.initials: JV
authors.lastname: Calduch Broseta
mesh_terms: D000368:Aged; D017675:CREST Syndrome / Q000150:complications* / Q000175:diagnosis*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D012720:Severity of Illness Index