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The pathogenesis of PCH associated with CREST syndrome is unclear, and this patient's skin telangiectasia perhaps related to her pulmonary capillary proliferation.
Based on the clinical picture (calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasias) and immunological findings (high levels of anti-centromere and anti-chromatin antibodies), a diagnosis of CREST syndrome was made and cyclosporine treatment was recommended.
He was diagnosed with CREST syndrome - a connective tissue disease that affects the skin, blood vessels, muscles, and internal organs, but is treatable.
"We have attempted to clarify the effectiveness by analyzing the results from 17 patients, each with a firmly established diagnosis of CREST syndrome and painfully disabling Raynaud's phenomenon," said Dr.
There are two types of systemic scleroderma: limited (which includes the CREST syndrome) and diffuse.
The earlier study [9] reported that only 3 of 8 patients with anticentromere antibody had a diagnosis of CREST syndrome, and only 1 of 19 patients with nucleolar antibody had a diagnosis of scleroderma.
(1) SSc is classified according to the extent of skin involvement and includes limited SSc (ISSc), which is also called CREST syndrome, and diffuse SSc (dSSc).