Systemic Sclerosis with Focus on Scleroderma Renal Crisis.

Known as systemic sclerosis (SSc), this autoimmune rheumatic disease has vast pathogenesis on many organs, including kidneys. It can lead to the point where the patient's survival relies entirely on dialysis. This report has basically focused on scleroderma renal crisis (SRC), which is the most serious renal manifestation of SSc, characterized by renal failure and sudden onset of hypertension. A 44-year-old man was hospitalized with hypertension, headache, vertigo, nausea, rhinorrhea, reflux, dysphagia, dyspnea (Fc II), visual impairment, mechanical arthralgia, and edema (+3) accompanied by a rare skin lesion. Raynaud's phenomenon was also remarkable in fingers and toes. According to signs and symptoms, SSc diagnosed and the proper treatment was applied. It is of great importance that in the case of malignant hypertension in patients with scleroderma, renal crisis always be kept in mind.

The effect of treatment with N-acetylcysteine on the serum levels of C-reactive protein and interleukin-6 in patients on hemodialysis.

Patients with end-stage renal disease (ESRD) are at an increased risk of cardiovascular disease due to many factors including inflammation and oxidative stress. N-acetylcysteine (NAC) is a thiol-containing anti-oxidant with anti-inflammatory properties. We aimed to assess the effect of three months treatment with oral NAC on the plasma levels of inflammatory mediators like interleukin-6 (IL-6) and C-reactive protein (hs-CRP) in patients on hemodialysis (HD). Twenty-four patients (nine males and 15 females) on maintenance HD were recruited in the study. Their mean age was 55.3 years. All the patients received oral NAC (600 mg twice a day) for a period of three months. The serum levels of biomedical parameters and IL-6 and hs-CRP were measured at baseline and three months after initiation of treatment. A significant decrease in serum levels of hs-CRP (22.4 vs. 5.2), IL-6 (8.1 vs. 3.6), parathyroid hormone (iPTH) (257.2 vs. 158.8), ferritin (632.0 vs. 515.1) and erythrocyte sedimentation rate (ESR) (54.2 vs. 38.3) was observed following NAC treatment. Female subjects presented with a significantly higher change in serum levels of hs-CRP compared with males (23 vs. 5.4). In three subjects who were less than 40 years old, the hs-CRP and IL-6 levels showed an increase following NAC treatment. Our study found that short-term oral NAC treatment might result in the reduction of IL-6 and hs-CRP in patients who are on regular HD. This suggests that patients with ESRD may benefit from the anti-inflammatory effects of NAC.

Long-term follow-up of children treated with daclizumab for steroid-refractory gastrointestinal GvHD in a prospective study.

Daclizumab, a humanized MoAB to IL-2Ra, has been found to be safe and effective in adults with refractory GvHD; however, data in children are limited. The aim of this prospective study was to evaluate the long-term safety and efficacy of daclizumab in children with steroid-refractory GI aGvHD. This study included 13 children who developed steroid-refractory GI GvHD between 2007 and 2009. When first-line treatment failed, daclizumab was given in a regimen of 1 mg/kg intravenously and then repeated on a 10- to 14-day interval for maximum five doses if necessary. Daclizumab was well tolerated, but infections were common. Ten patients responded to daclizumab completely, one patient responded partially, and two patients failed to respond. With a median follow-up of 630 days, 10 patients were alive and free of severe infections, but among them, four patients were suffering from cGvHD. Of the three remaining patients, one died because of bacterial meningitis, and the other two patients died because of severe refractory GI GvHD. This long-term evaluation showed that daclizumab could be an effective and relatively safe treatment in most of the pediatric patients with severe steroid-refractory GI GvHD.