Undifferentiated Tissue Disease
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Posted by Elaine Moore Feb 25, 2007 |
Undifferentiated connective tissue disease (UCTD) is a term first coined in 1980, which is used to describe conditions characterized by signs and symptoms that suggest a systemic autoimmune disease but that don’t satisfy the diagnostic criteria for specific connective tissue diseases, such as systemic lupus erythematosus (SLE) and related disorders, rheumatoid arthritis, and others.
Who is Affected?
Undifferentiated connective tissue disease is seen worldwide and affects people of all races although studies suggest that whites are more likely to be affected. People of all ages may be affected although the peak incidence occurs in middle age. Women are affected about ten times as frequently as men.
Disease Course, Signs, and Symptoms
About 25 percent of patients diagnosed with undifferentiated connective disease will develop a specific autoimmune connective tissue disorder such as SLE within the first year from diagnosis. In one five-year study of patients newly diagnosed with SLE, most patients whose UCTD differentiated into a specific disorder developed Sjogren’s syndrome, followed by SLE, mixed connective tissue disease, systemic vasculitis, progressive systemic sclerosis, and poymyositis/dermatomyositis. Overall, most patients with UCTD who develop a specific connective tissue disease do so within the first two years following their initial diagnosis.
However, in this and other studies, about 10 percent of newly diagnosed patients experience complete remission within one year. Within the first five years following diagnosis, about 65 percent of patients experience a reduction in symptoms, develop new symptoms, or continue to manifest the same signs and symptoms of UCTD indefinitely. A stable disease course is common in patients whose symptoms appear unchanged. Overall, the prognosis in UCTD is considered to be good.
The most common signs and symptoms of undifferentiated connective tissue disease include arthritis, arthralgias, Raynaud’s phenomenon, eye dryness (sicca syndrome), pleuritis/pericarditis, peripheral neuropathy, vasculitis, fever, weight loss, fatigue, mucocutaneous manifestations, cutaneous (skin) involvement including malar rash, hives, skin tightening and alopecia, respiratory symptoms, urethral discharge, muscle weakness, myositis, rash, and leukopenia (low white blood cell count) in the absence of neurological symptoms or kidney involvement. About 80 percent of patients with undifferentiated connective tissue disease have only one autoantibody, usually anti-Ro or anti-RNP.
In some cases, patients who are initially diagnosed with Raynaud’s phenomenon alone go on to develop undifferentiated connective tissue disease.
Diagnosis
UCTD is diagnosed in patients with symptoms of a connective tissue disease who fail to meet classic diagnostic criteria for a specific disorder. Patients with UCTD are diagnosed with blood and imaging tests, which are used to measure pulmonary capacity.
Proposed preliminary diagnostic criteria for UCTD include
- Signs and symptoms suggestive of a connective tissue disease
- Positive ANA test results
- Disease course lasting at least three years
Suspected environmental triggers include ultraviolet light and infection.
Treatment
Patients with UCTD are treated variably depending on predominant symptoms and the severity of the disease course. Common treatments include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, immunosuppressive agents, calcium channel blockers, and cyclooxygenase-2 inhibitors.
Resources
Bodolay E, Csiki Z, Szekanecz Z, Ben T, Kiss E, et al, Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD), Clin Exp Rheumatol, 2003 May-Jun; 21(3): 313-320.
Duffy, Benjamin, Undifferentiated Connective-Tissue Disease, eMedicine from Web MD, April 25, 2006, assessed Feb 14, 2007.
Mosca M, Tani C, Neri C, Baldini C, Bomardieri S, Undifferentiated connective tissue diseases (UCTD), Autoimmune Rev, 2006 Nov;6(1): 1-4.