For Patients

If you have scleroderma, you are not alone. The scleroderma community is made up of tens of thousands of patients and their loved ones worldwide. The SRF is here to help.

The first time many people hear about scleroderma is when they, a family member or friend are diagnosed with the disease. Scleroderma is a complex and surprisingly widespread illness, affecting as many people as more commonly recognized diseases such as multiple sclerosis and muscular dystrophy.

In addition to funding the most promising research aimed at improved therapies and a cure, the Scleroderma Research Foundation provides information that may help scleroderma patients better understand their disorder and more effectively manage its symptoms.

To learn more about the various forms and subtypes of scleroderma, please click here. This section of the Scleroderma Research Foundation’s Web site provides information for patients to educate themselves, as well as their caretakers and loved ones, about this serious disease.

Please remember, information provided on this Website and others is intended as a guide. Specific medical advice can only be provided by your health care professional.

 
 

Research News

Adipocyte–myofibroblast transition: linking intradermal fat loss to skin fibrosis in SSc

Author: Sarah Onuora
Date Published: January-2015
Source: Nature Reviews Rheumatology

Myofibroblasts are considered the primary fibrogenic effector cells in systemic sclerosis (SSc), but the origin of these cells within fibrotic lesions is a matter of debate. A new study published in Arthritis & Rheumatology shows that the majority of dermal myofibroblasts in fibrotic skin arise from adiponectin-positive progenitors resident in the…

Review: Cancer-Induced Autoimmunity in the Rheumatic Diseases

Author: Ami A. Shah, Livia Casciola-Rosen and Antony Rosen
Date Published: January-2015
Source: Arthritis & Rheumatology

Tantalizing connections between autoimmune rheumatic diseases and cancer have become increasingly evident over the past several decades. These connections are complex, with different relationships in frequency, timing, and types of cancers observed in different diseases or disease subgroups. Several recent advances from disparate fields have begun to illuminate the dynamic and bidirectional interactions occurring at the cancer–immune system interface which may be relevant to understanding the origins of autoimmunity ([1]). These interactions include the existence of potent anticancer immune responses that limit tumor growth, as well as multiple immune and inflammatory pathways that can contribute to tumor growth and robustness. The striking ability of immune checkpoint inhibitors to reveal powerful anticancer immune responses in patients with cancer highlights the fact that natural immune responses to cancers occur, and may regulate the emergence of cancer ([2]).

Autologous Fat Grafting in the Treatment of Fibrotic Perioral Changes in Patients With Systemic Sclerosis

Author: Del Papa, Nicoletta; Caviggioli, Fabio; Sambataro, Domenico; et al
Date Published: January-2015
Source: Cell Transplantation

Autologous fat tissue grafting (AFTG) has been successfully used in the treatment of different sclerotic conditions, including localized scleroderma. Patients with advanced systemic sclerosis (SSc)-related perioral thickening and mouth opening limitation are candidates for this therapeutic approach. AFTG of the lips was performed to improve mouth opening in patients with SSc. We enrolled in the study 20 female patients with diffuse SSc (median age 35 ± 15 years and 11 ± 10 years of disease duration). Two-milliliter fractions of autologous fat drawn from trochanteric or periumbilical areas were injected in eight different sites around the mouth.

Impaired BMPRII Signalling in a TGFβ Dependent Mouse Model of Pulmonary Hypertension and in Systemic Sclerosis.

Author: A. Gilbane, E. Derrett-Smith, S. Trinder, R. Good, A. Pearce , C. Denton , and A. Holmes
Date Published: January-2015
Source: American Journal of Respiratory and Critical Care Medicine

Rationale: Up to 10 percent of systemic sclerosis (SSc) patients develop pulmonary arterial hypertension (PAH). This risk persists throughout the disease and is time-dependent, suggesting that SSc is a susceptibility factor. Outcome for SSc-PAH is poor compared with heritable (hPAH) or idiopathic (iPAH) forms, despite clinical and pathological similarities. Whereas susceptibility in hPAH and iPAH is strongly associated with gene mutations leading to reduced expression of bone morphogenetic protein type II receptor (BMPRII), these mutations have not been observed in SSc-PAH.

Clinical Trial for MEDI-551 in Scleroderma Completed

Author: Mahoney JM, Taroni J, Martyanov V, Wood TA, Greene CS, Pioli PA, Hinchcliff ME, Whitfield ML
Date Published: January-2015
Source: PLoS One

Systemic sclerosis (SSc) is a rare systemic autoimmune disease characterized by skin and organ fibrosis. The pathogenesis of SSc and its progression are poorly understood. The SSc intrinsic gene expression subsets (inflammatory, fibroproliferative, normal-like, and limited) are observed in multiple clinical cohorts of patients with SSc. Analysis of longitudinal skin biopsies suggests that a patient's subset assignment is stable over 6-12 months. Genetically, SSc is multi-factorial with many genetic risk loci for SSc generally and for specific clinical manifestations.

News for Patients

7 Things You Should Know About Autoimmune Diseases

Author: Sarah Klein
Date Published: February-2015
Source: Huffington Post

As if living with chronic illness wasn't challenging enough, living with an autoimmune disease can be even more difficult. Still highly misunderstood by medical professionals and the public alike, autoimmune diseases are characterized by nebulous symptoms that can make diagnoses difficult to come by. Treatments vary, and in some cases rely entirely on behavior changes. In an effort to making living with -- or loving someone with -- an autoimmune disease just a little bit easier, here are seven important things to know about these health conditions.

Autoantibodies May Explain Link Between Cancer and Scleroderma Onset

Author: Patricia Silva
Date Published: February-2015
Source: Scleroderma News

Researchers at Johns Hopkins University School of Medicine in Baltimore investigated the association between autoantibodies, the risk of developing cancer and the possible link between cancer and scleroderma. The study is entitled “Examination of autoantibody status and clinical features that associate with cancer risk and cancer-associated scleroderma,” and is published in the journal Arthritis & Rheumatology.

FDA Approves Expansion of Pivotal Scleroderma STAR Trial to 20 Clinical Sites

Author: Cytori
Date Published: February-2015
Source: Cytori

Cytori Therapeutics, Inc. (NASDAQ: CYTX) announced today that it received approval from FDA to expand the number of Scleroderma clinical trial sites from 12 to 20 centers in the United States. The STAR study is an 80 patient pivotal clinical trial approved by FDA in January 2015 to study the effects of Cytori’s lead drug ECCS-50 for treatment of patients with hand manifestations of Scleroderma.

Researchers Report Audiovestibular Dysfunction in Systemic Sclerosis Patients

Author: Patricia Silva
Date Published: February-2015
Source: Scleroderma News

Researchers at Cairo University in Egypt recently published in The Egyptian Rheumatologist journal their findings on auditory dysfunction observed in systemic sclerosis patients. The study is entitled “Otolith function assessment in patients with systemic sclerosis.” Systemic sclerosis, also known as scleroderma, is a rare, chronic autoimmune disease in which the body’s own immune system attacks healthy tissues resulting in a hardening and tightening of the skin and connective tissues. Scleroderma usually affects the skin, but it can also affect internal organs such as the lungs, blood vessels and the digestive tract, being more common among women than men.

New Study Summarizes Scientific Findings Concerning Scleroderma Renal Crisis

Author: Patricia Inacio
Date Published: February-2015
Source: Scleroderma News

In a recent study entitled “Scleroderma renal crisis,” the authors summarize the most recent findings related to scleroderma renal crisis, a serious but treatable complication of systemic sclerosis. The study was published in the journal Seminars in Arthritis and Rheumatism. Scleroderma (or systemic sclerosis) is an autoimmune disease characterized by skin thickening, a process known as fibrosis. Several complications associated with scleroderma are due to the fibrosis process that is actually not restricted to the skin, reaching in severe cases, internal organs such as kidneys.

Ways to Give

There are many ways that you can support the work of the Scleroderma Research Foundation. We are grateful for your commitment to helping the SRF fund research that will result in improved therapies and, ultimately, a cure.

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