11/907. Marked and sustained improvement two years after autologous stem cell transplantation in a girl with systemic sclerosis. Autologous transplantation of hematopoietic stem cells has recently been proposed as a possible treatment for autoimmune diseases that are associated with a very severe prognosis. A 12-year-old girl who, since 4 years of age, had systemic sclerosis with progressive pulmonary involvement underwent autologous peripheral blood-derived stem cell transplantation (aPBSCT) using CD34 selection, cyclophosphamide, and the infusion of the monoclonal antibody CAMPATH-1G. Following transplantation, in the absence of any treatment other than symptomatic therapy, the patient's exertional dyspnea and alveolitis disappeared and she experienced a marked improvement in skin score, height velocity, and general well-being that has persisted 2 years after the transplantation procedure. Autologous PBSCT associated with the infusion of the monoclonal antibody CAMPATH-1G appears to be a useful therapy for otherwise intractable forms of progressive systemic sclerosis. ( info) |
12/907. diagnosis and physiopathology of laryngeal deposits in autoimmune disease. We report the clinical features and pathology of a previously unreported form of vocal fold disease seen in 4 patients, 3 of whom were diagnosed as autoimmune disease. The characteristic features of these lesions were found as bilateral transverse lesions in the mid portion of the vocal folds. The patients had dysphonia and diplophonia. Stroboscopic examinations showed 180-degree phase shifts between the anterior and posterior portion of the vocal folds. Case 1 had systemic lupus erythematosus, case 2 had Hashimoto's thyroiditis, and case 3 had progressive systemic sclerosis. Prior to the onset of hoarseness, autoimmune antibody titers were increased. These cases need differential diagnosis from vocal fold nodules and cysts. Two cases were recurrent after endoscopic surgery, 1 recurring 3 times. Glucocorticoid was effective in preventing the recurrence in the early phase. recurrence may have occurred because the surgical strategy was the same as that used for vocal fold nodules or cysts. ( info) |
13/907. Watermelon-stomach as a cause of chronic iron deficiency anemia in a patient with systemic sclerosis. Watermelon-stomach is a rare cause of gastrointestinal bleeding. There has been an increasing number of reports on the association of this lesion with diseases of the scleroderma group, causing chronic, sometimes severe gastrointestinal blood loss. The present report presents the case of a 75-year-old female with limited cutaneous systemic sclerosis and watermelon-stomach, which was the cause of her long-standing sideropenic anemia. ( info) |
14/907. Atypical esophageal diverticula associated with progressive systemic sclerosis. Five cases of unusual esophageal diverticula associated with progressive systemic sclerosis (scleroderma) or collagen vascular disease are presented. These wide-mouthed saccular diverticula were infrequently seen in a group of PSS patients with the typical motility disturbance of esophageal involvement and are reminiscent of the diverticula of the colon involved by PSS. ( info) |
| Chronic intestinal pseudoobstruction (CIPO) is a rare syndrome that may occur in association with connective tissue diseases (CTD). Effective management is a major challenge. We report 3 cases in which subcutaneous octreotide was efficacious in the treatment of digestive symptoms in CIPO. In 2 of the 3 cases, previous treatment with domperidone, cisapride, or erythromycin had been unsuccessful. All 3 patients underwent a regimen of oral antibiotics along with octreotide to stimulate small bowel motility. The effects of octreotide were evident within 48 hours after the first injection in all patients. In 2, the efficacy seemed to decrease after 1 week and 6 months respectively, but increasing the dosage led to another remission. CIPO in CTD is a severe condition that can evolve regardless of the underlying disease activity. octreotide appears to be efficacious in improving both clinical symptoms and manometric patterns. When its therapeutic effect diminishes, increasing the dosage can be useful. ( info) |
16/907. Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis. Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon. ( info) |
17/907. Takayasu's arteritis overlapping with systemic sclerosis. We describe an unusual case of overlap between Takayasu's arteritis (TA) and systemic sclerosis (SSc). TA has been found in association with several diseases, but not with SSc. To our knowledge this is the first case report of TA associated with SSc in the literature. It is possible that the expression of the two diseases in our patient was influenced by the presence of genetic factors predisposing to both TA and SSc. ( info) |
| scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis. The patient's serum contained Scl 70 antibodies, characteristic of scleroderma. electromyography showed signs of acute myositis and the creatine phosphokinase (CPK) level was elevated. Multiply passaged fibroblasts from the patient's skin lesions showed altered growth response in vitro. The patient was treated with cyclosporin (4 mg/kg/day) with improvement. ( info) |
19/907. Spinal anaesthesia for caesarean section in a patient with systemic sclerosis. We describe the management of a diabetic primigravid woman with systemic sclerosis and thrombocytopaenia who required Caesarean section for pre-eclampsia. This was performed successfully under spinal anaesthesia. ( info) |
20/907. Acute effects of nebulised epoprostenol in pulmonary hypertension due to systemic sclerosis. Pulmonary hypertension often has a lethal outcome in systemic sclerosis and the treatment is challenging. epoprostenol is a potent pulmonary vasodilator and its efficacy has been demonstrated when delivered by the intravenous and aerosolized routes. We report the haemodynamic and functional benefits of epoprostenol administered by inhalation to a spontaneously breathing patient with partially reversible pulmonary hypertension due to systemic sclerosis. Aerosolized epoprostenol, equivalent to the maximum tolerated intravenous dose (31.2 micrograms), produced a 58% fall in pulmonary vascular resistance, increased the cardiac output by 42% and improved functional performance by one MET (3.5 ml kg-1 min-1 of oxygen uptake) without any significant side-effects. Selective distribution of epoprostenol by the inhaled route may offer a new strategy for treatment of pulmonary hypertension. ( info) |