
It is presumed that many of the same immunologic mechanisms that play a role in defined connective tissue diseases like lupus or rheumatoid arthritis may be involved. This then leads the immune system to incorrectly target the body itself as if it is a “foreign invader” (as it is designed to target harmful bacteria in the context of infection). One theory is that some people have a genetic predisposition or risk of developing connective tissue disease and then experience an environmental trigger, such as an infection. UCTD is difficult to study because it affects people with so many different symptoms and blood markers. The actual pathogenesis, or cause, of UCTD, like that of many rheumatic diseases, is not well understood.

What are the causes of undifferentiated connective tissue disease? In general, these two terms are used when patients have clear features of at least two defined connective tissue diseases, and thus may have more than one diagnosis at the same time. Rheumatologists and scientists do not all agree on a precise definition for overlap syndromes. UCTD is different from another group of vague-sounding disorders called “overlap syndromes,” and from “mixed connective tissue disease” (MCTD), which is a term used to describe a more specific pattern of overlapping symptoms. It does not mean that a doctor does not know what to call the condition, it just means that some or all of the clinical features traditionally seen in defined connective tissue disease are not present. In fact, as many as a quarter of all patients seen by rheumatologists have UCTD. 3,4Īlthough the word "undifferentiated" sounds vague, it describes a real problem and one that is quite common 5. Other names used early on to describe some of these patients included "latent lupus" and "incomplete lupus erythematosus.” 2 While many patients with UCTD have symptoms that are also seen in lupus, others have symptoms more characteristic of a different defined connective tissue disease, which is why we prefer to use the term “UCTD.” There is also more recent evidence to suggest that stable UCTD may in fact be a distinct disease process, as opposed to an early phase of a defined connective tissue disease. 1 At that time, it was noted that a substantial proportion of these patients either remained “undifferentiated” or experienced a disease remission and never developed a defined connective tissue disease. The term "undifferentiated connective tissue disease" was first used in the 1980s to identify people who were recognized as being in the early stages of, but who did not yet meet the standard criteria for, a defined connective tissue disease. The term "undifferentiated connective tissue disease" (UCTD) is used to describe a condition in people who have symptoms and lab test results which suggest a a systemic autoimmune disorder or connective tissue disease but which are not extensive or specific enough to meet usual criteria for a diagnosis of a defined connective tissue disease such as systemic lupus erythematosus (lupus), rheumatoid arthritis, systemic sclerosis ( scleroderma), Sjogren’s syndrome, or myositis. What is undifferentiated connective tissue disease?

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