Breaking News
February 20, 2019 - CVD Does Not Modify Depression-Mortality Link in Elderly
February 20, 2019 - Electrical activity early in fruit flies’ brain development could shed light on how neurons wire the brain
February 20, 2019 - Self-reported sleep duration is a useful tool to measure sleep in children, study suggests
February 20, 2019 - T-cells play key role in how the body fights follicular lymphoma
February 20, 2019 - Study shows how 3D organization of genetic material helps perpetuate the species
February 20, 2019 - Researchers engineer stem cell with ‘suicide genes’ to induce cell death in all but beta cells
February 20, 2019 - Study reveals major sex differences in management of cardiovascular risk factors among U.S. adults
February 20, 2019 - Health Tip: Get Your Child to School on Time
February 20, 2019 - Shortcut strategy for screening compounds with clinical potentials for drug development
February 20, 2019 - Common acid reflux drugs tied to elevated risk for kidney disease
February 20, 2019 - Microbiome could be culprit when good drugs do harm
February 20, 2019 - Prenatal exposure to forest fires causes stunted growth in children
February 20, 2019 - Gene therapy restores hearing in mice with congenital genetic deafness
February 20, 2019 - First molecular test predicts treatment response for kidney cancer
February 20, 2019 - New method for improved visualization of single-cell RNA- sequencing data
February 20, 2019 - Researchers capture altered brain activity patterns of Parkinson’s in mice
February 20, 2019 - A possible blood test for detecting Alzheimer’s disease before symptoms show
February 20, 2019 - Primary care physicians associated with longevity, new research finds
February 19, 2019 - New study identifies many key lessons to establish sanctioned safe consumption sites
February 19, 2019 - Single CRISPR treatment can safely and stably correct genetic disease
February 19, 2019 - Multinational initiative to study familial primary distal renal tubular acidosis
February 19, 2019 - Breakthrough study highlights the promise of cell therapies for muscular dystrophy
February 19, 2019 - Subsymptom Threshold Exercise Speeds Concussion Recovery
February 19, 2019 - Midline venous catheters – infants: MedlinePlus Medical Encyclopedia
February 19, 2019 - Searching for side effects
February 19, 2019 - Humanity is all right, probably, although human extinction remains quite possible, researcher says
February 19, 2019 - Having Anesthesia Once as a Baby Does Not Cause Learning Disabilities, New Research Shows
February 19, 2019 - Anti-cancer immunotherapy could be used to fight HIV
February 19, 2019 - Customized Micropatterning for Improved Physiological Relevance
February 19, 2019 - Unique gene therapy approach paves new way to tackle rare, inherited diseases
February 19, 2019 - Activating gene that helps excite neurons reverses depression in male mice
February 19, 2019 - Science Puzzling Out Differences in Gut Bacteria Around the World
February 19, 2019 - Cells that destroy the intestine
February 19, 2019 - On recovery, vulnerability and ritual: An exhibit in white
February 19, 2019 - Scientific Duo Gets Back To Basics To Make Childbirth Safer
February 19, 2019 - COPD patients need more support when understanding new chest symptoms
February 19, 2019 - Using light-based method for production of pharmaceutical molecules
February 19, 2019 - Scientists find link between inflammation and cancer
February 19, 2019 - The High Cost Of Sex: Insurers Often Don’t Pay For Drugs To Treat Problems
February 19, 2019 - Hearing impairment associated with accelerated cognitive decline with age
February 19, 2019 - Researchers identify multiple genetic variants associated with body fat distribution
February 19, 2019 - Influenza and common cold are completely different diseases, study shows
February 19, 2019 - Scientists untangle how microbes manufacture key antibiotic compound
February 19, 2019 - Greater primary care physician supply associated with longer life spans
February 19, 2019 - HIV-1 protein suppresses immune response more broadly than thought
February 19, 2019 - Brain imaging indicates potential success of drug therapy in depressive patients
February 19, 2019 - For 2020 Dem Hopefuls, ‘Medicare-For-All’ Is A Defining Issue, However They Define It
February 19, 2019 - Specialized lung cells appear in the developing fetus much earlier than previously thought
February 19, 2019 - KU professor discusses promise of brain-computer interface to aid, restore communication
February 19, 2019 - Highly effective solution for detecting onset of aggregation in nanoparticles
February 19, 2019 - Early marker of cardiac damage triggered by cancer treatment identified
February 19, 2019 - Antidepressant drug could save people from deadly sepsis, research suggests
February 19, 2019 - CRISPR technology creates pluripotent stem cells that are ‘invisible’ to the immune system
February 19, 2019 - New study establishes how stress favors breast cancer growth and spread
February 19, 2019 - Midlife Systemic Inflammation Linked to Later Cognitive Decline
February 19, 2019 - Therapy derived from parasitic worms downregulates proinflammatory pathways
February 19, 2019 - Antimicrobial reusable coffee cups are less likely to become contaminated with bacteria, study shows
February 19, 2019 - Harnessing the evolutionary games played by cancer cells to advance therapies
February 19, 2019 - AHA News: Heart Transplant Survivor Gets Wedding Proposal at Finish Line
February 19, 2019 - HIV hidden in patients’ cells can now be accurately measured
February 19, 2019 - Research finds reasons for sudden cardiac death in patients with stable ischemic disease
February 19, 2019 - New protocol could help physicians to rule out bacterial infections in infants
February 19, 2019 - Women experiencing miscarriage should be offered treatment choices
February 19, 2019 - New protocol can help identify febrile infants at low risk for serious bacterial infections
February 19, 2019 - Innovative way to block HIV runs into a roadblock
February 19, 2019 - Springer Nature with BCRF conduct pilot project to make their research datasets more accessible
February 19, 2019 - Study finds neuromelanin-sensitive MRI as potential biomarker for psychosis
February 19, 2019 - Improvements in cardiovascular care for elderly save billions in health care costs
February 19, 2019 - Chilean food regulations are changing food perceptions and purchasing habits, study suggests
February 19, 2019 - Index endoscopy results are crucial for assessment of Barrett’s patients
February 18, 2019 - Breast cancer screening age should be lowered to 35
February 18, 2019 - Brain synchronization depends on the language of communication
February 18, 2019 - Drug Company Payments Over Time May Influence Rx Practices
February 18, 2019 - Despite socioeconomic gains, black-white ‘health gap’ remains
February 18, 2019 - Researchers report progress in the treatment of aggressive brain tumors
February 18, 2019 - Scientists discover trigger that turns strep infections into devastating disease
February 18, 2019 - Scanning children’s teeth may predict future mental health issues
February 18, 2019 - Health Highlights: Feb. 14, 2019
February 18, 2019 - New knowledge could help predict and prevent depression
February 18, 2019 - More primary care physicians leads to longer life spans | News Center
Seeking solutions to treat scleroderma

Seeking solutions to treat scleroderma

image_pdfDownload PDFimage_print

It begins with cold hands.

Not just put-on-some-gloves cold. Instead, the fingers overreact to cold by turning white or blue and may become numb or tingly.

This hypersensitivity to cold, called Raynaud’s phenomenon, may occur alone, or it may be the first symptom of systemic scleroderma. A rare, autoimmune disease, scleroderma sometimes is known as the “disease that turns people to stone.”

Months or even two or three years after the onset of Raynaud’s phenomenon, people with the systemic form of scleroderma usually notice swelling of the hands with an uncomfortable tight sensation. That’s followed by thickening and hardening of the skin that usually starts in the fingers and may spread to the hands, forearms, body, legs and feet.

The word “scleroderma” comes from the Greek “sclero,” meaning hard, and the Latin “derma,” meaning skin. The localized form of scleroderma -; more correctly called morphea -; is primarily a skin disease and does not affect the internal organs. However, the systemic form of scleroderma (systemic sclerosis or SSc) is much more complicated and serious.

“SSc is far more than a skin disease,” says Maureen Mayes, M.D., professor in the Division of Rheumatology and Clinical Immunogenetics and the Elizabeth Bidgood Chair in Rheumatology at McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth). “In most people SSc is a multi-organ system disease.”

With scleroderma, the immune system becomes activated, damages the small blood vessels and may cause excessive buildup of fibrous connective tissue, or scar tissue, in the lungs, heart, gastrointestinal tract, kidneys, muscles and joints, as well as the skin. It may cause difficulty breathing, trouble swallowing, joint pain or other complications.

Estimated to affect 300,000 people in the United States (200,000 with morphea and 100,000 with systemic disease), scleroderma affects primarily women who are 25 to 55 years old at onset. Although there is a genetic component, the exact cause of this noncontagious disease is not yet known.

“We know there are about 30 genes thus far -; and there are probably more of them -; that will increase a person’s susceptibility to SSc,” Mayes says.

“It’s rare, but occasionally we see that more than one family member will have scleroderma. Then we also see in other families that one member has lupus, someone else has scleroderma, and someone else has rheumatoid arthritis, so those families have susceptibility to multiple autoimmune diseases.”

There’s no explanation for occurrence of the disease in individuals who have none of the known susceptibility genes.

“Just having the genes is not adequate to cause the disease,” Mayes says. “People have to be exposed to something that triggers the disease, and that might not be a single thing. It could be a viral infection or a bacterial infection or some sort of environmental exposure. We’ve certainly looked into a lot for potential environmental exposures but have not yet found much that seems to be specific for people with scleroderma versus those without scleroderma.

“We don’t know what triggers the immune system to respond in this way. Partly because of that, although we can treat it, we can’t prevent or cure it,” says Mayes, who is a past president and a current director of the National Medical and Scientific Board of the United Scleroderma Foundation.

People with scleroderma typically see a rheumatologist first, then other specialists, depending on what organ systems are involved. The UT Physicians Scleroderma Clinic, which is directed by Mayes, coordinates all the subspecialists.

“We try to have a more holistic approach toward the diagnosis and management of scleroderma so we know what’s going on in the lungs, the heart, the kidneys and the GI tract and can help to coordinate therapy,” she says.

Patients generally take multiple medications, some just to treat symptoms. For milder cases, for example, there are medications for Raynaud’s phenomenon and for heartburn and reflux.

“One of the important things that we do in the scleroderma clinic is to monitor people with annual or biannual testing. Just because a patient doesn’t have lung disease today doesn’t mean that they might not get lung disease a year from now. So we get pulmonary function tests and sometimes chest CAT (computerized axial tomography) scans if it looks like the pulmonary function tests are abnormal. We have some treatments that, although not curative, can slow or stop the progression of the disease,” Mayes says.

“Our treatments have potential adverse effects, so you don’t want to give them to someone who’s stable and not worsening. But you want to be able to identify someone who is developing internal organ involvement. It’s better to get treatments started before a lot of damage has been done,” she adds.

Immunosuppressants are the major category of medications used against the overactive immune system. Unfortunately, in decreasing the activity of the immune system, the medications also can make people more susceptible to infections. To guard against serious side effects of some of the medications, the UT Physicians Scleroderma Clinic tests for hepatitis and TB before beginning treatment with immunosuppressants and treats those diseases first if they are present.

Most of Mayes’ patients who participated in the study did very well. Although some features of scleroderma remain -; such as Raynaud’s, vascular damage and scar tissue in the lungs -; those problems tend to stop progressing, and skin manifestations tend to improve significantly. Because there was a 3 percent mortality rate from destruction of the immune system, this treatment is appropriate only for people with severe disease and poor prognosis.

Mayes is optimistic about development of future treatments. She encourages people with scleroderma to participate in a clinical trial if they have the opportunity.

“I’ve been involved in almost every single treatment trial that has been conducted in scleroderma over the past 25 years. At first, there would be one trial and then two or three years later a second trial. Now we have six ongoing trials simultaneously, which is, I think, very hopeful that at least one of these medications is going to be effective,” she says.

Source:

https://www.uthealthleader.org/story/scleroderma-seeking-solutions-to-a-difficult-puzzle

Tagged with:

About author

Related Articles