The term “scleroderma” encompasses a spectrum of complex and variable conditions primarily characterized by 3 major phenomenon: abnormal, excess formation of connective tissue (fibrosis), vascular alterations, and an autoimmune response (Gabrielli 2009; A.D.A.M. 2013; Derk 2003; Balbir-Gurman 2012; Varga 2008). Scleroderma spectrum disorders share the common feature of hardening or thickening of the skin, but other manifestations can vary greatly between patients. Although the cause of scleroderma is not understood, the hallmark of scleroderma is fibrosis, which is a process similar to the formation of scar tissue; fibrosis is primarily brought about by inflammation. No cure currently exists for scleroderma (Gabrielli 2008; Gomer 2008).
There are 2 primary types of scleroderma:
- The less severe form is called localized scleroderma. It is limited to the skin and underlying muscles, joints, and sometimes bones; it does not affect internal organs. In some cases, localized scleroderma may resolve on its own (Mayo Clinic 2010; NIAMS 2012; Gabrielli 2009; Clemens 2003; Klein-Weigel 2011; Hawk 2001; Takehara 2005). There are about 27 new cases of localized scleroderma per million people per year (Peterson 1997).
- The more widespread and severe form is called systemic scleroderma (also known as systemic sclerosis). This form is progressive and occurs throughout the body, affecting internal organs as well as the skin (Chatterjee 2010). It may affect the connective tissue of the lung, kidney, heart, and other organs, as well as blood vessels, muscles, and joints (Hawk 2001; Heinberg 2007). There are 2 subtypes of systemic sclerosis: limited cutaneous systemic sclerosis (historically known as CREST syndrome) and diffuse cutaneous systemic sclerosis (Jimenez 2012; Gabrielli 2009). Limited cutaneous systemic sclerosis typically affects the skin on limited areas of the body, such as the fingers, hands, or the face and is less likely to involve internal organs early in disease progression. Diffuse cutaneous systemic sclerosis, which may start suddenly and spread over much of the body, is associated with a poorer prognosis (Hughes 2012). There are about 19 new cases of systemic sclerosis per million people per year (Jimenez 2012).
Conventional management strategies for scleroderma are limited to interventions that control specific symptoms; no drug has been developed that successfully treats the underlying cause(s) of scleroderma (Hinchcliff 2008). On the other hand, natural strategies may offer a means of directly targeting fibrosis, a destructive process that involves the formation of tough connective tissue through inflammatory pathways and is a central driving force behind scleroderma progression. Natural constituents like epigallocatechin gallate (EGCG) from green tea, N-acetylcysteine, and vitamin E may help preserve tissue integrity in scleroderma sufferers (Chang 2013; Rosato 2009b; Fiori 2009; de Souza 2009). Moreover, maintaining adequate blood levels of vitamin D may be an important consideration for individuals affected by scleroderma, since studies show it can directly modulate signaling pathways that contribute to fibrosis (Slominski 2013; Arnson 2011).
In this protocol you will learn about the different types of scleroderma and how they are diagnosed and treated. Future research directions will be explored, and a variety of scientifically studied natural interventions will be reviewed. Some important dietary and lifestyle considerations will be presented as well.