losartan-potassium and Scleroderma--Systemic

Systemic sclerosis (SSc) is often complicated by severe skin ulcers that are unresponsive to traditional treatments. Vascular alterations are responsible for the ischaemic features of the disease in both the skin and visceral organs. Defective neoangiogenesis correlates with an abnormally reduced quantity of circulating endothelial progenitor cells (EPCs) caused by impaired maturation potential and proliferative capacity of bonemarrow endothelial stem cells. We report a patient with nonhealing cutaneous ulcers successfully treated with recombinant human erythropoietin (rHuEPO). The possible biological effects of this drug were also investigated. Before rHuEPO treatment, the bone-marrow sample contained reduced numbers of EPCs, which were functionally impaired. After a 6-month rHuEPO cycle, a marked increase in endothelial progenitor markers was seen, along with a significant reduction in their apoptotic rates. The clinical and laboratory data variations before and after rHuEPO treatment give new insights into the pathogenetic role of impaired endothelial stem-cell maturation and defective neoangiogenesis in patients with SSc.

Topics: Aged; Apoptosis; Bone Marrow; Endothelial Cells; Erythropoietin; Humans; Male; Neovascularization, Physiologic; Recombinant Proteins; Scleroderma, Systemic; Skin Ulcer; Wound Healing

We first describe the onset of acute myositis in a patient suffering from systemic sclerosis after the administration of darbepoetin alpha for renal failure-related anemia. Therapeutic implications and risks are discussed.

Topics: Acute Disease; Adrenal Cortex Hormones; Anti-Inflammatory Agents; Darbepoetin alfa; Dose-Response Relationship, Drug; Erythropoietin; Female; Hematinics; Humans; Injections, Subcutaneous; Methylprednisolone; Middle Aged; Myositis; Prednisone; Scleroderma, Systemic; Severity of Illness Index; Treatment Outcome

Systemic sclerosis (SSc) is frequently complicated by skin ulcers, often unresponsive to traditional treatments. A preliminary evaluation of the effects of recombinant human erythropoietin (rHuEPO) was carried out in 14 patients with SSc with nonhealing, severe cutaneous ulcers. Patients received rHuEPO subcutaneously at a dosage of 150 IU/kg 3 times weekly for 2 weeks, twice weekly for the next 2 weeks, and then once weekly for 1 month. At follow-up 3-6 months from the beginning of the treatment, six patients showed complete resolution of the skin ulcers, while a significant reduction (> 60%) in lesional areas was obtained in the other eight patients (mean +/- SD ulcer area reduced from 27.6 +/- 28 to 5.3 +/- 7.8 cm(2); P<0.005). Moreover, patients' quality of life significantly improved (pain, as measured on visual analogue scale reduced from 96 +/- 5 to 46 +/- 17 points; P=0.0001; disability as measured by the Health Assessment Questionnaire-Disability Index reduced from 1.6 +/- 0.5 to 0.9 +/- 0.4 points; P=0.0001). The rHuEPO may represent a novel treatment of nonhealing scleroderma skin ulcers, suggesting some important aetiopathological implications.

Topics: Adult; Erythropoietin; Female; Humans; Male; Middle Aged; Pain Measurement; Quality of Life; Recombinant Proteins; Scleroderma, Systemic; Skin Ulcer

A 40-year-old black male with scleroderma lung disease presented with blurry vision and headache. His presenting hemoglobin was 22.3 g/dL and his serum erythropoietin level was surprisingly low. Although nocturnal hypoxemia was evident, his daytime resting arterial oxygen saturation was normal. The patient's symptoms of hyperviscosity improved after phlebotomy, as his hemoglobin gradually decreased to 18.3 g/dL. Repeat serum erythropoietin levels were in normal and high ranges. Patients with chronic interstitial lung disease and erythrocytosis could have normoxemia at rest and a normal or low serum erythropoietin level at the peak of erythrocytosis. A repeat sampling of serum erythropoietin and monitoring of oxygen saturation during sleep and exertion may help in diagnosis. Physicians should prescribe continuous oxygen therapy for patients with chronic interstitial lung disease and erythrocytosis, even if diurnal resting hypoxemia is absent.

Topics: Adult; Chronic Disease; Erythropoietin; Headache; Humans; Lung Diseases, Interstitial; Male; Oxygen Inhalation Therapy; Polycythemia; Scleroderma, Systemic; Vision, Low