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  • Title: [Severe isolated pulmonary hypertension as main manifestation of Crest syndrome].
    Author: Arenas Gracia M, Ruiz Riquelme J, Tovar Martínez A, Martínez López V, Calduch Broseta JV.
    Journal: An Med Interna; 2000 Jul; 17(7):369-71. PubMed ID: 10981335.
    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.
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