Системная склеродермия.Схемы лечения
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- Хасанбек Окунчаев
Киста вилочковой железы
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- Igor V
Видела выше посты о пенициллинах - возможно, имелся в виду D-penicillamine ?
- Виктору Тутунову
Скопирую оригинальный текст, он на английском D-penicillamine is a drug capable of affecting both collagen biosynthesis and the immune system. One possible mechanism is that autoantibodies in SSc are formed against autoantigens that have been fragmented via reactive oxygen species and specific metals such as copper or iron [34]. Penicillamine, a chelator of metals, may therefore minimize the formation of these autoantigens (see Pathogenesis of systemic sclerosis (scleroderma) , section on 'Autoantibodies'.The precise role of penicillamine in the treatment of SSc has yet to be determined. Estimates of the efficacy of penicillamine and the effect upon survival can be found in uncontrolled studies published by several groups with extensive experience with this drug. The possible benefit of penicillamine can be illustrated by the following observations:
- Виктор Тутунов
A retrospective analysis from Pittsburgh compared 73 patients with SSc who had received penicillamine to 45 patients who had not [35]. The mean daily dose of penicillamine varied from 500 to 1500 mg and therapy was given for a mean period of 24 months; the mean follow-up was at least three years. Patients treated with penicillamine showed an improved cumulative five year survival (80 percent) and a lower rate of new visceral involvement compared to patients not exposed to penicillamine. Except for skin softening, other intergroup differences did not reach statistical significance [35].Another group reported their 15 year, uncontrolled experience with penicillamine in SSc [36]. This study consisted of 69 selected patients with diffuse disease of less than 18 months' duration in whom a 30 percent increase in skin involvement had occurred in the preceding six months. Treatment with penicillamine (750 mg/day) for at least six months was associated with a 75 percent improvement in skin sclerosis (from 65 to 16 percent of total body surface), and an 80 percent five-year cumulative survival rate. Renal disease was uncommon and pulmonary disease was not progressive in patients treated for more than six months.These and other studies have noted that skin sclerosis worsened during the first four to six months despite therapy. This observation emphasizes the necessity for the development of a rapidly acting antifibrotic agent.To assess the effects of high dose penicillamine on disease activity, 134 patients with early dcSSc were enrolled in a multicenter double-blind randomized study comparing high-dose (750 to 1000 mg/day) with low-dose (125 mg every other day) therapy [37]. Sixty-eight patients completed 24 months of therapy. No significant differences were observed between the two treatment groups with respect to the skin thickness scores and the incidence of renal crisis and mortality. However, 16 of the 20 adverse event-related withdrawals occurred in the high-dose treatment group. Although this study could not answer the question of whether low dose penicillamine is effective, there appeared to be no advantage in using high dose penicillamine. Based upon the results of this key trial we do not initiate penicillamine therapy for SSc. However patients who are stable on therapy may be continued, with dose reduction towards the low dose used in the controlled trial (125 mg every other day).
- Igor V
Есть определенные бенефиты от использвания интерферонов, но существуют и многие но! - это весьма серьезные лекарства с вероятностью значит побочных действийThe interferons have been reported to be beneficial in the therapy of various diseases, including SSc. In particular, an antifibrotic effect has been reported in vitro. However recent data have implicated interferons in the pathogenesis of some autoimmune diseases. Interferon gamma (IFN-gamma) is a more potent inhibitor of collagen synthesis in vitro than IFN-alpha. It has other effects, however, which might contribute to aberrant cellular activation in SSc. (See Pathogenesis of systemic sclerosis (scleroderma) .) As examples, in vitro IFN gamma can lead to:Macrophage activationClass II antigen expression (DR, DP, DQ) on endothelial and fibroblasts cellsIncreased interleukin-2 receptor expressionIncreased intracellular adhesion molecule-1 expression on endothelial cellsIgG Fc receptor expressionThese concerns have been apparently confirmed in uncontrolled studies in which IFN-gamma was associated with considerable vascular side effects, including renal hypertensive crisis [38]. As a result, no controlled studies are currently planned.IFN-alpha has received less attention than IFN-gamma. A pilot study of 14 patients with early diffuse disease of less than three years in duration found a significant measurable reduction in type-1 collagen synthesis from fibroblasts cultured from uninvolved skin, stabilization or improvement in the skin test in 10 of the patients [39].However, a multicenter double-blind study of 35 patients found that IFN-alpha provides no increased benefit compared with placebo and may be deleterious [40]. In this report, patients with early SSc were randomized to interferon-alpha (13.5 million units per week given subcutaneously for 12 months) or placebo. Compared with the placebo group, intent-to-treat analysis found that the group treated with IFN-alfa had more substantial skin involvement (as assessed by the skin score), and a significantly greater deterioration in lung function (as assessed by the forced vital capacity [p = 0.01] or the diffusing capacity for carbon monoxide [p = 0.002])
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