Effects of N-acetylcysteine on Pulmonary Functions in Patients with Systemic Sclerosis: A Randomized Double Blind, Placebo Controlled Study

Background: Systemic sclerosis (SSc) is a systematic and rare autoimmune disease that affects many organs. N-acetylcysteine (NAC), thiol-containing compound, can act both as the precursor of reduced glutathione and direct scavenger of reactive oxygen species. Objective: We assessed the clinical effect of NAC on the pulmonary function test of patients with diffuse scleroderma. Methods: This study is a randomized double-blind clinical trial that was done on 25 patients with diffuse SSc without lung involvement on primary chest high-resolution computed tomography. Placebo was administered for 13 patients and 1200 milligram NAC for 12 patients. Body plethysmography parameters were assessed at the beginning of the study and after 24 weeks. Results: Patients in the two groups were matched in the basic demographic data like age, duration of disease, and modified Rodnan skin score. The analysis showed no significant differences in parameters of plethysmography between the two groups. After importing the data of 2 patients in the placebo-treated group, who developed interstitial lung disease, DLCO in the placebo-treated group was 90.69 ± 21.29 milliliter at the end of the study, which significantly decreased compared with the beginning of the study (102.30 ± 13.83 ml). Also, changes of DLCO between the two groups were significantly different. Conclusions: In this trial, the sensitivity of DLCO as the first marker in the evaluation of pulmonary function in patients with SSc was confirmed. On the other hand, NAC had no effect versus placebo in a period of 24 weeks.

Avocado–Soybean Unsaponifiables: A Panoply of Potentialities to Be Exploited

Avocado and soybean unsaponifiables (ASU) constitute vegetable extracts made from fruits and seeds of avocado and soybean oil. Characterized by its potent anti-inflammatory effects, this ASU mixture is recommended to act as an adjuvant treatment for osteoarthritic pain and slow-acting symptomatic treatment of hip and knee osteoarthritis; autoimmune diseases; diffuse scleroderma and scleroderma-like states (e.g., morphea, sclerodactyly, scleroderma in bands). Besides, it was reported that it can improve the mood and quality of life of postmenopausal women in reducing menopause-related symptoms. This article aims to summarize the studies on biological effects of the avocado–soybean unsaponifiable, its chemical composition, pharmacotherapy as well as applications in autoimmune, osteoarticular and menopausal disorders. Finally, we will also discuss on its safety, toxicological and regulatory practices.

A clinical case of a patient with scleroderma-like syndrome in chronic graft-versus host disease

Chronic graft versus host disease is observed within the fi rst 100 days following allogeneic haematopoietic stem cell transplantation and can affect all tissues and organs (in 80% of the cases, it affects the skin). There are some clinical correspondences between chronic graft versus host disease and certain autoimmune diseases, such as systemic scleroderma, Sjogren‘s syndrome, autoimmune hepatitis. We present a case of a 54-year-old man with manifested diffuse, scleroderma-like skin changes, which occurred about a year and a half after allogenic bone marrow transplantation from an unrelated donor due to a blast transformation of chronic myelomonocytic leucosis type 2. The patient was treated in a haematology clinic with corticosteroids, 10 photophoresis sessions, Ciclosporin, Tacrolimus, Mycophenolate mofetil, Imatinib. It has been assumed that this is a case of chronic graft versus host disease resistant to corticosteroids, and Methotrexate 25 mg/weekly every other month was prescribed with no significant clinical improvement. In terms of the differential diagnosis, the question remains whether or not this is a case of paraneoplastic systemic scleroderma – autoimmune phenomena accompanying malignancy and often preceding it for months.

Cardiac transplantation in a 20-year-old woman with scleroderma

Purpose: To describe cardiac transplantation in a young woman with juvenile onset diffuse scleroderma and cardiac involvement. Methods: Case report. Results: A young White girl developed anti-topoisomerase-1 positive diffuse scleroderma aged 14 years with myositis. Pulmonary function tests were normal. Skin disease was treated with mycophenolate mofetil 1 g twice daily, methotrexate 7.5 mg weekly and periodic intravenous prostacyclin. When aged 17 years, she developed raised troponin T of 0.207 mcg/L (normal range <0.03) and NTproBNP (155 pmol/L); 6-min walking distance was 341 m, and she had episodes of presyncope with effort. The next year she developed symptomatic ventricular tachycardias and dual-chamber implantable cardioverter-defibrillator was inserted, with further episodes of ventricular tachycardia and one shock delivered. By age 19 years, 6-min walking distance was 125 m. Echocardiography showed ejection fraction of 15%–20% with dilated left ventricle and pericardial effusion. She was treated with intravenous Rituximab. She became breathless while dressing and managed only 118 m in 6 min. She experienced increasing orthopnoea and peripheral oedema and was found to be in a low cardiac output state, requiring treatment with intravenous milrinone to maintain renal function. She underwent orthotopic cardiac transplantation, making an excellent post-operative recovery, and was discharged 16 days later with tacrolimus, mycophenolate mofetil and prednisolone. After 1 year, she was in New York Heart Association functional class I and with normal cardiac function on echocardiography. Conclusion: This case illustrates the severe cardiac involvement that can occur in juvenile onset diffuse cutaneous systemic sclerosis, in which cardiac involvement is the leading cause of death.