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Painsi, C.
Dermatologische Merkmale der systemischen Sklerodermie bei pulmonaler Hypertonie
[ Diplomarbeit/Master Thesis ] Graz Medical University ; 2009. pp.43. [OPEN ACCESS]
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Authors Med Uni Graz:: Painsi Clemens
Advisor:: Aberer Elisabeth; Olschewski Horst
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Abstract:: Background: Pulmonary hypertension is one of the major causes which shorten survival in patients affected by systemic sclerosis. However, pulmonary involvement may commonly be silent, whereas skin fibrosis is usually the clinical feature drawing most attention. We investigated the relationship between cutaneous involvement and elevated pulmonary arterial pressure in SSc. Methods: We studied the nailfold capillaries (using capillary microscopy), the distribution-pattern of teleangiectasia, morphologic aspects of hands and skin sclerosis (using modified Rodnan Skin Score) in 45 patients affected by scleroderma and compared SSc patients with EPAP ((n=15) using right-heart catheterization) and SSc patients with NPAP ((n=7) using stressechocardiography) Results: Our data suggest that there is no difference between the EPAP and NPAP group with respect to capillary abnormalities. The distribution-pattern of teleangiectasias showed that 71.4% of the patients with NPAP had teleangiectasias on the palms and on the v area as opposed to the NPAP group where only 46.7% showed this pattern. The morphologic investigation of the hands in SSc showed that 42.9% of NPAP patients had a Madonna like configuration whereas in the EPAP group we did not observe this configuration. In addition we found an increased number of ulcerations, a higher score in skin sclerosis and some patients also showed blue hands in the EPAP group. Conclusion: Some morphologic aspects in scleroderma could refer to EPAP. Especially ulcerations, a higher Rodnan skin score and a blue aspect of the hands. Patients with a Madonna like configuration, calcinosis and teleangiectasias on the palms and on the v area could have a lower risk for EPAP.