Long-term Evaluation of Penicillamine or Cyclofenil in Systemic Sclerosis:Results from a Two-year Randomized Study

Abstract

In a two-year prospective therapeutic trial 13 patients with systemic sclerosis (SSc) were treated with penicillamine, 9 with cyclofenil, and 7 with neither. At entry skin involvement and esophageal, lung, heart, and kidney function did not differ significantly between the groups. Reevaluation after one and two years did not show any significant changes in skin, esophageal, heart, and kidney manifestations, while lung function had slightly improved in both drug-treatment groups. This study thus shows little overall effect of penicillamine and cyclofenil, although both drugs may arrest worsening of pulmonary dysfunction.

Esophageal Disease in Scleroderma

“…92-95 D-penicillamine and cyclofenil improved lung functions slightly but had no effects on esophageal disease. 96,97 Surgery yielded good results in 95% of patients at 2 y, 82% at 3 y, 71% at 5 y, and 0% at 12 y using various operations. [98][99][100] Surgery is generally considered only in severe esophageal disease resistant to medical therapy.…”

Sex hormones and sex hormone-targeting therapies in systemic sclerosis: A systematic literature review

“…For the second research question À (2) À we found 9 articles assessing sex hormone levels in patients with SSc [9,25,29,30,33,41,49,51,54], 6 case reports [36,42,44,47,48,50] about the association of male hypogonadism and SSc, and 2 retrospective cohort studies evaluating the effect of menopause on SSc [34,35]. Finally, for the third research question À (3) À we included 7 studies on sex hormone-targeting therapies and their potential effect on SSc [26,28,38,40,43,45,46].…”

Lung Disease in Systemic Sclerosis (Scleroderma)

“…Low-dose corticosteroids have been used in combination with cyclophosphamide with beneficial effects on symptoms and lung volumes. 33,41,42 Azathioprine, studied in IPF, has not undergone study in SSc patients.…”

“…Other studies of D-Pen have shown an improvement in pulmonary function, particularly in those patients with a mildly reduced D LCO . 41,45 However, other investigators have noted that an initially decreased D LCO may revert to normal without therapeutic intervention, 19 thus making the significance of a D-Pen effect on the D LCO less certain.…”

“…63 Echocardiography with Doppler is one of the most reliable noninvasive means of assessing pulmonary hypertension, and in a study by Denton and colleagues, 64 it had a sensitivity of 90% and specificity of 75% when compared with right heart catheterization in 33 SSc patients with clinically suspected pulmonary hypertension. The incorrect classification of patients with echocardiographic evidence of pulmonary hypertension but normal cardiac catheterization pressures always occurred in the subgroup of patients with upper limits of normal values or mildly elevated pulmonary pres- sures (27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41)(42). Exercise pulse oximetry can be used to assess the severity of pulmonary vascular disease by detecting exercise-induced oxygen desaturation.…”