View clinical trials related to CREST Syndrome.
Filter by:Systemic sclerosis or scleroderma is an autoimmune condition that cause thickening and hardening of the skin, but can also affect internal organs. There are two major subsets of scleroderma: the limited cutaneous systemic sclerosis (lcSSc) that usually affects the skin of the face, neck, lower legs or lower arms, but can also lead to internal organ complications, and the diffuse cutaneous systemic sclerosis (dcSSc) that may affect blood circulation and internal organs, as well as the skin. To date there is no drug that has been definitively proven to cure or modify the course of scleroderma. However, there is emerging evidence that immunosuppression and specifically mycophenolate mofetil (MMF) may be beneficial in lcSSc. The MINIMISE-Pilot trial would be an important first step to evaluate the risk and potential benefit to this disease group. MMF as the intervention of choice is both appropriate and timely, as it has been routinely used in the management of dcSSc. The aim of this pilot trial is to explore whether the immunosuppressive agent MMF can slow down disease progression in patients with lcSSc compared to the current standard of care alone. This pilot trial will also provide critical information for the development of a future large trial that could potentially transform lcSSc patient management.
The principal objective is to measure the degree to which oral microstomia caused by sclerosing skin disease improves after treating patients with local hyaluronidase injections. Investigator will determine improvements in oral aperture by measuring the centimeters of the height of oral opening. The secondary objectives are: Investigator will aim to assess changes in quality of life and functionality, by serial calculations of the Mouth Handicap in Systemic Sclerosis (MHISS). In addition, investigator would like to investigate how many treatments are required prior to treatment efficacy plateauing. Since there is minimal data on the use of hyaluronidase for oral microstomia, it is not yet clear how many treatments are ideally required for maximal effect. Patients will be brought in monthly for photographs, examination, assessment, and treatment. Our hypothesis is that hyaluronidase injections will significantly improve patients' ability to open their mouths and oral functionality. It remains unclear how many treatments will be required for maximal effect.
Background CREST is an acronym for the cardinal clinical features of the syndrome (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia) and part of the heterogeneous group of sclerodermas. Calcinosis is the pathologic calcification of soft tissues. When symptomatic, they can be tender and painful, ulcerate, and drain a white chalky substance. With time, heterotopic bone formation may occur. Inflammatory reactions also intermittently occur at the site of calcinosis. It has been suggested that TGF-beta3 plays a major role in the pathogenesis of calcinosis. A variety of medical therapies have been used to try to alleviate patient symptoms. These include pharmacological approaches (e..g., warfarin), surgical curettage or excision, as well as carbon dioxide laser treatments. No consistently reliable pharmacological treatment seems to be available to prevent or eliminate calcinosis. Curettage and excision and carbon dioxide laser of localized painful large deposits can relieve symptoms but recurrence is common. In addition, aggressive curettage or excision can damage deeper neurovascular structures. While calcinosis is associated with significant morbidity its treatment remains a challenge. Photobiomodulation (PBM) has been shown to promote wound healing, suppress inflammatory reactions and regulate collagen synthesis in a number of in vitro and in vivo studies. Human skin contains photolabile nitric oxide (NO) derivatives which decompose after UVA irradiation and release vasoactive NO. However, aside from blue light, barely nothing has been reported about the effects of red and NIR wavelengths. Method A custom-built air tight sleeve which envelopes the forearm of a subject will be used to measure the NO emanating from the skin under photobiomodulation conditions (red & NIR) and quantified by chemiluminescence detection. Simultaneously, CREST patient's hands exhibiting calcinosis and/or Raynaud phenomenon will be exposed to exogenous gaseous nitric oxide (INOMAX) to determine the vascular impact of this approach. This case series will assess Light Emitting Diode (LED) based PBM therapy as a treatment alternative for cutaneous calcinosis and the effects of gaseous NO on calcinosis and/or Raynaud phenomenon in CREST patients.
Iliac crest bone graft harvest is associated with acute pain that can lead to chronic postoperative pain. Local anesthetic infiltration and regional anesthesia could be of interest to reduce opioid consumption during and after surgery, reduce hyperalgesia and the associated risk of chronic postoperative pain. In this surgery, local anesthetic infiltration of the iliac crest is associated with a pain reduction compare with placebo. Some studies have shown the benefit of a TAP (transversus abdominal block) for postoperative analgesia after iliac crest bone graft harvest. The TFP block (transversalis fascia plane block) has been recently described. Considering the nerves trajectories, the TFP block is probably anatomically the most appropriate block for analgesia after iliac crest bone graft harvest. The aim of this study is therefore to assess the benefit of an ultrasound TFP block for analgesia after iliac crest bone graft harvest compare with local anesthetic infiltration Design is prospective, randomized, double blind, controlled study
The purpose of this study is to investigate whether the use of the study drug (Exparel) is safe and effective for treating circulation problems and pain in the hand or fingers.
Hand or wrist surgery often requires the use of bony material in order to accomplish any associated reconstructive aspects required for the surgery. The iliac crest is often used as the source of bone for such surgery and harvesting from this site is performed simultaneously with the hand/wrist surgery. In terms of anesthesia, patients typically receive a supraclavicular nerve block for the hand/wrist surgery, and a general anesthestic for the bone graft, though spinal block may also be performed. This study will look at the use of a transversalis fascia plane (TFP) nerve block (ie, to numb the nerves going to the iliac crest) in combination with a general anesthetic to see if post-operative pain and opioid consumption is reduced using the TFP block.