1/2. pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension.OBJECTIVE: pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). We describe a series of 4 patients with systemic sclerosis (SSc), concomitant PH, and biopsy-proven/presumed PVOD. We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/2. Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis.We have encountered a 68-year-old Japanese woman with limited cutaneous systemic sclerosis who developed de novo onset of accelerated hypertension and renal dysfunction; thus we diagnosed scleroderma renal crisis. Anticentromere antibody alone was identified, and not anti-dna topoisomerase I antibody, anti-rna polymerase antibodies, anti-Th/To antibodies, or antiribonucleoprotein antibodies, even with use of immunoprecipitation assay. She was successfully treated with angiotensin-converting enzyme inhibitor. This case, scleroderma renal crisis with detection of anticentromere antibody, is thought to be extremely uncommon.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |