1/11. Global improvement of systemic scleroderma under long-term administration of octreotide.octreotide has proven to be effective for the treatment of intestinal dysmotility in patients with scleroderma in short-term administration. We report a global improvement of scleroderma manifestations under long-term administration of octreotide. A 53-year-old black woman was diagnosed with a four-year history of progressive and severe systemic scleroderma, with diffuse skin sclerosis, myositic involvement, impaired carbon monoxide transfer factor (57% of the predicted normal value and severe digestive involvement with pseudo-obstruction and bacterial overgrowth into the intestinal lumen). After one month of octreotide (75 mug/d), oral feeding was restarted and weight gain of 6.5 kg was achieved. After 8 months of treatment, normal weight was obtained and skin induration was spectacularly reduced and pigmentation returned to a normal state. dyspnea disappeared and physical activity was quite normal. octreotide effects on intestinal transit are unclear and may be secondary to immunomodulation or neurotransmission effects. Extradigestive effects of octreotide in scleroderma have not been studied. This report suggests that long-term administration of octreotide may be beneficial in the treatment of patients with systemic scleroderma. Long-term trials are required to confirm these preliminary results.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/11. skin score decrease in systemic sclerosis patients treated with intravenous immunoglobulin--a preliminary report.The aim of the study was to determine for the first time the response of systemic sclerosis (SSc) patients to treatment with intravenous immunoglobulin (IVIg). Three patients with progressive and rapidly deteriorating disease (mainly affecting the skin) were planned to receive six monthly courses of high-dose IVIg (2 g/kg). All had a thorough physical examination, clinical evaluation by the modified Rodnan total skin thickness score, and measurement of the titres of PM-Scl antibodies before and after the treatment, and before and after each treatment course. Two of the three patients received six IVIg courses as planned and no adverse effects or disease progression occurred during the therapy. The third patient received three courses, after which he developed renal failure and later died of sepsis. All three patients had a large decrease in their skin score after the treatment compared to that before the treatment. No modification of PM-Scl antibody titres was noted in any patient. Intravenous immunoglobulin (IVIg) may have a role in the treatment of SSc patients with rapidly deteriorating skin disease. The specific indications, as well as the safety of this treatment, should be further researched.- - - - - - - - - - ranking = 5.1851609423519keywords = physical examination, physical (Clic here for more details about this article) |
3/11. Physical injury as a provoking factor in three patients with scleroderma.A 51-year-old female developed linear-like scleroderma in the left thigh following a linear wound caused by a car accident. 27 years later she also developed a typical diffuse cutaneous systemic sclerosis with extensive skin involvement and bibasilar pulmonary fibrosis. The second case is a 39-year-old female who had a history of Raynaud's phenomenon since early childhood. She developed a morphea following a burning injury of the left thigh. 17 years later she also developed a typical limited cutaneous systemic sclerosis with sclerodactyly, skin ulcers and subcutaneous calcinosis. The third case is a 43-year-old female who developed a typical morphea of the right elbow around the site of a previous local corticosteroid injection. The two remarkable points of these 3 cases are the possible role of physical injury in the provocation of localized scleroderma and in the first 2 cases the unusual later development of a systemic form of scleroderma.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/11. Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports.We describe two systemic sclerosis (SSc) patients with isolated pulmonary arterial hypertension (PAH) who were given treatment with 50 mg oral sildenafil per day. We evaluated the efficacy of oral sildenafil for isolated PAH in SSc patients by direct assessment with cardiac catheterization before and 6 months after the initiation of sildenafil. Right-heart catheterization demonstrated decreased mean pulmonary artery pressure, decreased pulmonary vascular resistance, and increased cardiac output after treatment with sildenafil. brain natriuretic peptide levels were gradually decreased. The 6-min walking distance was greatly extended. Moreover, the physical conditions of both patients were much improved. We recognized no adverse events. We propose that oral sildenafil may be beneficial as a selective pulmonary vasodilator and as long-term treatment in SSc patients with isolated PAH.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/11. Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis.OBJECTIVE: To evaluate the safety and efficacy of allogeneic hematopoietic cell transplantation (HCT) after myeloablative conditioning in patients with severe systemic sclerosis (SSc). methods: Eligibility criteria for the study included SSc patients with features indicative of a poor prognosis. The myeloablative conditioning regimen included busulfan, cyclophosphamide, and antithymocyte globulin. Prophylaxis for graft-versus-host disease (GVHD) consisted of cyclosporine and methotrexate. bone marrow was transplanted from HLA-identical siblings. RESULTS: Two patients with diffuse cutaneous SSc and lung involvement who were refractory to conventional immunosuppressive treatment were enrolled in the study. In patient 1, there were no complications related to the conditioning regimen, and GVHD did not develop after transplantation. At 5 years after HCT, there was nearly complete resolution of the scleroderma and marked improvement in physical functioning. Internal organ function improved (lung) or remained stable. On examination of serial skin biopsy samples, there was resolution of the dermal fibrosis. Patient 2 experienced skin toxicity from the conditioning regimen and hypertensive crisis that was likely related to high-dose corticosteroids given for treatment of GVHD. Although this patient experienced an improvement in scleroderma and overall functioning, a fatal opportunistic infection developed 17 months after HCT. CONCLUSION: Allogeneic HCT may result in sustained remission of SSc. GVHD and opportunistic infections are the major risks associated with allogeneic HCT for SSc, as for allogeneic HCT in general.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/11. The Shulman syndrome.The Shulman syndrome is a symptom complex recently described in the rheumatology literature that is characterized by eosinophilia, hypergammaglobulinemia, and a diffuse scleroderma-like process of the extremities. The onset of illness has been associated with a period of unusual physical exertion. The condition seems to respond to corticosteroid therapy for systemic effect.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/11. Improvement of lung function in a scleroderma patient treated for 12 months with cyclofenil.A 48 year old male scleroderma patient with typical impairment of respiratory function, including low vital capacity, low static lung compliance and low diffusing capacity for carbon monoxide, was treated with cyclofenil for one year. Assessment of lung function after 3, 6 and 12 months treatment showed marked improvement of vital capacity, increased physical working capacity, less hypoxia at maximum work, increase of static lung compliance and decreases of maximal transpulmonary pressure. Closing capacity decreased, and an increase in difussing capacity for carbon monoxide was seen.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/11. A new case of eosinophilic fasciitis.A new case of Shulman's disease is reported. The patient shows skin induration in both arms and legs, and morphea plaques without visceral involvement. Onset of the disease followed an unusual physical effort. An important blood eosinophilia and a mild hypergammaglobulinemia are present. The histopathological studies show a typical eosinophilic fasciitis. The use of steroids had no effect in the treatment of the disease.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/11. Large vessel occlusive disease associated with crest syndrome and scleroderma.OBJECTIVES--To report the cases of three patients with crest syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors. methods--The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients. RESULTS--These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with crest syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma. CONCLUSIONS--These cases suggest an association of both the crest syndrome and scleroderma with macrovascular disease.- - - - - - - - - - ranking = 5.1851609423519keywords = physical examination, physical (Clic here for more details about this article) |
10/11. Silicone granuloma in acral skin in a patient with silicone-gel breast implants and systemic sclerosis.A 68-year-old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu-like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone-gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured. physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal. According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X-ray energy dispersive analysis by scanning electron-microscopy confirmed the presence of elemental silicon in the small refractile particles. The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow-up.- - - - - - - - - - ranking = 5.1851609423519keywords = physical examination, physical (Clic here for more details about this article) |
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