Breaking News
September 20, 2018 - Investigators find that bile acids reduce cocaine reward
September 20, 2018 - Cannabinoid drugs reduce perceived unpleasantness of painful stimuli and increase tolerance
September 20, 2018 - Geroscience takes center stage in Journal of the American Medical Association
September 20, 2018 - Ambient Particulate Matter Linked to Emergency Asthma Care
September 20, 2018 - Patient satisfaction with plastic surgery—it’s the surgeon, not the practice
September 20, 2018 - Medicine is a team sport – and that’s exactly how it should be
September 20, 2018 - Logos Biosystems releases new electrophoretic tissue clearing system with twice the features in half the space
September 20, 2018 - Novel micro-platform reveals never-before-seen behaviors of cancer cells
September 20, 2018 - PAREXEL partners with Datavant to enhance clinical study design and generate real-world evidence
September 20, 2018 - Robert Koch Institute publishes new data on allergies, mental health problems, and accident injuries
September 20, 2018 - Study finds higher readmission rates in for-profit hospitals
September 20, 2018 - Encouraging youth to do strength-based exercises could help tackle child obesity
September 20, 2018 - Sleep apnea, congenital heart disease in hospitalized infants strongly associated with death
September 20, 2018 - Researchers find way to map mysterious content of non-coding RNA
September 20, 2018 - Air Pollutants Reach Placenta, Might Harm Fetus: Study
September 20, 2018 - Sleep apnea, congenital heart disease may be deadly mix for hospitalized infants
September 20, 2018 - My relative has cancer, should I worry? Encouraging cascade genetic testing
September 20, 2018 - Investigators determine specific treatable traits that can predict future asthma attacks
September 20, 2018 - More doctor visits can lower risk of suicide attempts in fibromyalgia patients
September 20, 2018 - Computer avatars play role in diagnosis of dementia
September 20, 2018 - Addition of CTLA4 targeted therapy to PD-1 targeted therapy may benefit patients with ovarian cancer
September 20, 2018 - ASPREE trial explores whether low dose aspirin can prolong good health in elderly people
September 20, 2018 - ATS publishes new guideline focused on weight loss strategies for sleep apnea patients
September 20, 2018 - The Current issue of “The view from here” is concerned with Drug Delivery
September 19, 2018 - Sleep apnea could favour tumor growth at young ages
September 19, 2018 - Stealth vaping fad hidden from parents, teachers
September 19, 2018 - Witnessing school violence linked to later risk of psycho-social and academic impairment
September 19, 2018 - Common household cleaners could make children overweight by changing gut microbiota
September 19, 2018 - Salk research in yeast leads to serendipitous finding about hypomyelinating leukodystrophy
September 19, 2018 - Study: Overweight or obese women may have increased risk of urinary incontinence
September 19, 2018 - Study shows how cellular waste disposal processes also promote inflammation
September 19, 2018 - New multidisciplinary microsurgery microscope, PROVIDO, introduced by Leica
September 19, 2018 - Phase 2b STORM Data Evaluating Selinexor in Patients with Penta-Refractory Multiple Myeloma Presented at the Society of Hematologic Oncology 2018 Annual Meeting
September 19, 2018 - Decisions recruiting gut feelings seen as reflection of true self, more assuredly held, study says
September 19, 2018 - How AI can improve end-of-life care
September 19, 2018 - UNIST and Ulsan initiate research collaboration to develop human organs-on-chips
September 19, 2018 - Study highlights key role of migrating shoals of fish in sustaining deep-ocean microorganisms
September 19, 2018 - Disagreeable individuals can benefit most from behaving more compassionately, finds study
September 19, 2018 - Janssen Submits New Drug Application to U.S. FDA Seeking Approval of Erdafitinib for the Treatment of Metastatic Urothelial Cancer
September 19, 2018 - Neuroplasticity is increased but dysregulated in the aging brain, study finds
September 19, 2018 - Suicide: A public health crisis
September 19, 2018 - Infants using popular anti-reflux medicines are not at increased risk of lung infections
September 19, 2018 - Stanford team will participate in NIH-funded study of tobacco policies | News Center
September 19, 2018 - Women with high levels of anti-Müllerian hormone more likely to develop breast cancer
September 19, 2018 - Researchers use larval zebrafish model to reveal role of locus coeruleus in anesthesia
September 19, 2018 - Effects of prematurity found to be more severe for the brains of males than females
September 19, 2018 - NIH funds CWRU to investigate new imaging approach for diagnosing peripheral arterial disease
September 19, 2018 - NUS researchers develop new device for quick and accurate screening of diseases
September 19, 2018 - Leading nutritionist explains about different types of iron in oral supplements
September 19, 2018 - New Crest Gum & Enamel Repair toothpaste may boost enamel repair and reverse gingivitis
September 19, 2018 - Analysis does not find any safety concerns of bivalent HPV vaccine
September 19, 2018 - Many Older Adults Transition to Long-Term Benzodiazepine Use
September 19, 2018 - Caregiving – resources – older adults: MedlinePlus Medical Encyclopedia
September 19, 2018 - Research shows that cystic fibrosis impacts growth in the womb
September 19, 2018 - Study shows how head, neck positioning affects concussion risk | News Center
September 19, 2018 - Antioxidant in green tea helps sneak therapeutic RNAs into cells
September 19, 2018 - Excess pregnancy weight gain affects cardiometabolic risk in offspring
September 19, 2018 - Penn researchers find common thread linking almost all TNR expansion diseases
September 19, 2018 - Ipsen receives approval from Health Canada for CABOMETYX tablets for treating renal cell carcinoma
September 19, 2018 - Researchers use CRISPR screen to reveal new targets in squamous cell carcinomas
September 19, 2018 - UGR to coordinate the European H2020 project focused on Smart Personalized Nutrition
September 19, 2018 - Mumps Spread Quickly at Texas Cheerleading Meets: Study
September 19, 2018 - Addressing child stunting in Pakistan is critical
September 19, 2018 - Innovative system makes hospitals, clinics accessible for people with visual impairments
September 19, 2018 - CXCL14 protein is secreted by brown adipose tissue and has beneficial effects in metabolic diseases
September 19, 2018 - Air pollution increases risk of dementia
September 19, 2018 - Inequality issues persist even under new U.S. kidney transplant allocation system
September 19, 2018 - New study reveals mechanisms that lead to cognitive decline in Type 2 diabetes
September 19, 2018 - FDA launches new comprehensive effort to educate kids about dangers of e-cigarettes
September 19, 2018 - Study reveals mechanism underlying plants’ ability to signal defense
September 19, 2018 - Researchers harness Zika virus vaccine under development to target glioblastoma
September 19, 2018 - Novel deep learning drug discovery platform gets £1 million innovation boost
September 19, 2018 - Sensor array may detect de novo Parkinson’s disease in breath
September 19, 2018 - A roadmap for the future of electronic health records
September 19, 2018 - Surprising research showing peptide adaptability may pave way to develop immunotherapies
September 19, 2018 - Amyloid β protein makes comeback as therapeutic target for Alzheimer’s disease
September 19, 2018 - Alcon expands its global support of eye care professionals through Alcon Experience Academy
September 19, 2018 - Study gives new insights into how cells leverage GPCRs to control inflammation
September 19, 2018 - Automatic relevance detection in ophthalmic surgery videos
September 19, 2018 - UNIST to accelerate discovery, development of new medicines for incurable diseases
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